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Contributors
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Reviewers
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Preface
General and Oral Pathology for Dental Hygiene Practice the underlying disease mechanism may be sparse or
is an introductory textbook written for dental hygiene missing. Additionally, etiologies and risk factors for
students studying general and oral pathology, and as diseases are not consistently emphasized. To address
a resource for dental educators and clinicians. Our this issue, we begin each chapter with concise foun-
career journeys have included training and clinical dational and basic principles of pathology related to
practice in dental assisting, dental hygiene, dentistry, disorders or lesions presented in the chapter. The ef-
and oral and maxillofacial pathology. We have more fects of disease processes on the oral cavity and human
than 76 years combined experience in dentistry, oral body are emphasized as they relate to clinical practice.
and maxillofacial pathology, and dental education. The text is organized into nine chapters around seven
Throughout the years we have received numerous core topics in pathology: 1) reactive/inflammatory
requests to write a pathology textbook targeting dental disorders, 2) infectious diseases, 3) immune-based dis-
hygiene that contains current material contributed orders, 4) developmental disorders, 5) genetic disor-
from experts in their respective fields. This inspired us ders, 6) neoplasms, and 7) oral manifestations of
to write such a text that is also student-oriented, in- systemic disorders.
structor-friendly, and includes online supplementary Third, we have updated the material to incorporate
materials. General and Oral Pathology for Dental Hygiene the most recent changes in pathology nomenclature
Practice is a direct result of our combined passion for and classification. For example, we have included
dental hygiene education, dentistry, and oral pathol- human papilloma virus-related pathologies in the
ogy. Our goal is to use educational tools that promote chapter on infectious diseases rather than in the chap-
critical thinking and transfer learning outcomes to ter on neoplasia, where they have traditionally been
clinical dental hygiene practice. located. This emphasizes that infectious agents can in-
duce neoplastic changes. We believe this approach en-
Approach hances retention of knowledge and promotes active
First, we understand that not everyone who teaches learning by minimizing repetition of disease topics in
pathology in the dental hygiene curriculum has ad- multiple chapter locations, which can be confusing for
vanced training in general and oral pathology. Our new learners. The result is enhanced subject integra-
textbook seeks to train the trainer by use of supple- tion rather than duplication within the text and other
mentary materials. These instructor resources not only courses in the curriculum.
enhance student learning but also promote instructor One of our goals in writing this text is to under-
confidence in the material. score the central and critical role pathology plays in
Second, our colleagues in dental hygiene education patient care. To this end, we have consistently endeav-
report that currently available texts often do not pro- ored to point out where, how, and why particular
vide a sufficient depth of background knowledge im- pathological findings are important to both clinicians
portant for understanding and assessing pathology. and patients. The specific terminology of pathology
For instance, oral lesions associated with systemic is featured and promoted to facilitate communication
conditions are described, but essential discussion of between all members of the health-care team. A suite
xi
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xii Preface
Acknowledgments
The first edition of any textbook is a daunting task and MS; Susan Hadler MD; James Hughes DDS, MS;
this is certainly true for General and Oral Pathology for Michael A. Kahn DDS; Shanti Kaimal BDS, MDS, MS;
Dental Hygiene Practice. We would like to acknowledge John R. Kalmar DMD, PhD; Robert D. Kelsch DMD;
the many individuals who have contributed to this Ioannis Koutlas DDS, MS; Walter Kucaba DDS,
book and guided us through our first adventure in MS; John Ludlow DDS, MS; Alan Lurie DDS,
publication. Without their help, this would not have PhD; Denis P. Lynch DDS, PhD; Richard Madden
been possible. DDS, MS; Beth McKinney RDH, MS; Kristin K. Mc-
Our current and former editors at F. A. Davis Namara DDS, MS; Nicole Miller DDS; Glenn Minsley
deserve a tremendous amount of gratitude. They in- DMD; Madhu Mohan DMD; Danny Mora DMD;
clude: Quincy McDonald, Senior Acquisitions Editor; Leslie Orzech DMD; Ricardo Padilla DDS; Ali Pourian
Jennifer Ajello, Development Editor; Liz Schaeffer, DDS, MS; Pavithra Pugalagiri BDS, MS; Yeshwant
Developmental Editor/Electronic Products Coordi- Rawal BDS, MDS, MS; Renee Reich DDS; Jonathan
nator; Mackenzie Lawrence, Freelance Editor/Project Reside DDS, MS; Michael Rohrer DDS, MS; James
Manager; and Jackie Lux, Marketing/Convention Rokos DDS, MS; Vladimir Leon-Salazar DDS, MS;
Coordinator. We appreciate their expertise and their Shelly Stecker DDS, MS; Lan Su DMD, PhD; Kurt F.
infinite patience as we navigated this new world. Summersgill DDS, PhD; Jeffrey Thomas DDS; Brian
We acknowledge our chapter contributors who Vandersea DDS; J. Craig Whitt DDS, MS; Dag
took time from their hectic lives to help draft interest- Zapatero DDS; and Theodore Zislis DDS.
ing and challenging material: Mansur Ahmad BDS, We also thank the University of Minnesota School
PhD; Walter Bowles DDS, MS, PhD; Catherine of Dentistry for allowing us access to images from the
Champagne PhD; Raj Gopalakrishnan BDS, PhD; archives of the late Dr. Robert J. Gorlin. We also need
Nelson Rhodus DMD, MPH; Molly Rosebush DDS, to credit Northwest Dentistry for use of several pho-
MS; and Chelsia Sim BDS, MS. tographs from an article Sandra Myers wrote.
An oral pathology textbook is nothing without good We would like to acknowledge the reviewers whose
photographs from which students learn to recognize valuable and insightful comments and suggestions
disease in their patients. Our colleagues who generously greatly enhanced the final product. Their time and
stepped forward to share images from their personal expertise are greatly appreciated.
archives cannot be thanked enough: Abdel-Ghany Special thanks to Dr. Christine Harrington, Ohio
Al-Saidi DDS; Carl Allen DDS, MSD; Mark Anderson State University; Alexandra Komichek RDH, MS,
DDS; Bruce F. Barker DDS; Soraya Beiraghi DDS, University of North Carolina; Patricia Lenton GDH,
MSD, MS, MSD; Indraneel Bhattacharyya DDS, MA, University of Minnesota; and Hanae Tsujimoto
MSD; James Castle DDS, CAPT, DC, USN; Risa RDH, MS, University of Minnesota for their contri-
Chaisuparat DDS; Kitrina G. Cordell DDS, MS; butions to our ancillaries.
Darren P. Cox DDS, MBA; Andre Paes Batista DeSilva Our goal with this book is to spread the passion
DDS; Kerry Dove DMD; John E. Fantasia DDS; for oral pathology to the next generation of dental
George Gallagher DMD, DMSc; Thalji Ghadeer DDS, hygienists. We would be remiss if we did not
xiii
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xiv Acknowledgments
acknowledge our own mentors who over many Norman H. Rickles, Robert J. Bruckner, Nelson
years inspired us to follow our dreams and become Rhodus, and Robert J. Gorlin; Suzanne Box RDH,
dental assistants, dental hygienists, dentists, oral MS; Drs. Samir K. ElMofty, Douglas Damm, Dean
pathologists, and finally, educators. They include: White, Jim Drummond, Charles Waldron, Peter A.
Drs. Harold C. Reid, Wei-Yung Yih, LeGrand H. Pullon, and Ronnie Weathers.
Woolley, Murray H. Bartley Jr., Charles C. Thompson,
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Contents
xv
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Clinical Features of Immune Disorders 130 Developmental Anomalies That Mimic Cysts of the
Allergic Disorders 130 Jaws (Pseudocysts) 187
Ulcerative Conditions 133
Lichen Planus 139 Chapter 6
Lichenoid Mucositis 143 Disorders of Genetics and Inheritance 193
Desquamative Gingivitis Associated With Basic Principles of Genetic and Inherited
Autoimmune Vesiculobullous or Vesiculoerosive Disorders 194
Disorders 143 Basics of Genetics 194
Orofacial Manifestations of Systemic Modes and Patterns of Gene Transmission 195
Immunodysregulation 147 Syndromes 199
Genetic and Inherited Disorders of the Head
Chapter 5
and Neck 199
Developmental Disorders of the Orofacial Disorders of the Periodontium/Gingiva 199
Complex 163 Disorders of the Jaws and Facial Structures 202
Fundamentals of Oral-Facial Embryology Disorders of the Oral Soft Tissues 214
and Development 164 Inherited Disorders Limited to the Dentition 216
Facial Development 164 Disorders of Enamel: Types of Amelogenesis
Development of Neural Crest Cells 164 Imperfecta 216
Pharyngeal or Branchial Arches 165 Hereditary Disorders of Dentin Formation 218
Development of the Nose, Lips, and Palate 165 Disorders of Cementum: Odontohypophosphatasia 220
Development of the Tongue and Thyroid Gland 166
Abnormalities of the Face and Oral Cavity 167 Chapter 7
Cleft Lip and Cleft Palate 167 Neoplasms of the Oral Soft Tissues
Bid Uvula and Bid Tongue 168 and Facial Skin 227
Lip Pits 168 Basic Principles of Neoplasia 228
Double Lip 168 Tumor Nomenclature 230
Aglossia and Ankyloglossia 168 Benign Versus Malignant 230
Macroglossia and Microglossia 169
Oral Squamous Cell Carcinoma (Oral Cancer) 232
Fissured Tongue 169
Epidemiology and Risk Factors of Oral Cancer 232
Lingual Thyroid (Ectopic Thyroid) 170
Clinical Features of Oral Cancer 234
Lingual Varicosities 170
Progression to Oral Cancer 235
Hemifacial Microsomia 171
High-Risk Locations for Oral Cancer 237
Fundamentals of Tooth Development 171 Verrucous Carcinoma 240
Enamel Organ 171 Staging of Oral Cancer 242
Rests of Serres 171 Oral Cancer Treatment and Side Eects 243
Reduced Enamel Epithelium 171
Epithelial Neoplasms of the Oral Mucous
Dental Papilla and Dental Sac 172 Membranes and Facial Skin 247
Rests of Malassez 172 Benign Tumors 247
Developmental Abnormalities of the Epithelial Disorders With Malignant Potential 248
Dentition 172 Malignant Epithelial Tumors 249
Congenitally Missing Teeth 173
Salivary Gland Neoplasia 252
Hyperdontia (Supernumerary Teeth) 173
Benign Salivary Gland Tumors 252
Fusion and Gemination 174
Malignant Salivary Gland Tumors 253
Concrescence 174
Soft Tissue Neoplasia 255
Abnormalities of Tooth Shape 174
Adipose Tissue 255
Abnormalities of Tooth Color 177
Connective Tissue Proper 255
Enamel Hypoplasia 178
Nerve and Nerve Sheath Tumors 255
Developmental Cysts of the Head and Neck 179
Neoplasms of Blood and Lymphatic Vessels 257
Odontogenic Cysts 181
Tumors of Muscle 259
Nonodontogenic Cysts 184
Metastases to Oral Soft Tissues 260
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Chapter 9
Systemic Pathology and Oral Manifestations
of Systemic Diseases 287
Oral Manifestations of Systemic Disease 288
Overview of the Endocrine System and
Endocrinopathies 288
Pituitary Gland 288
Thyroid Gland 291
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2577_Ch01_001-026 11/07/14 11:06 AM Page 1
C h a p t e r
1
Introduction to Oral and Maxillofacial
Pathology and the Study of Disease
Overview of Oral and Maxillofacial Pathology Head, Neck, and Intraoral Examinations
The Practice of Oral and Maxillofacial Pathology Terminology Used in Describing Oral Pathologic Lesions
Assessment of Oral Pathologic Lesions Supplemental Diagnostic Aides
Patient Assessment and History Dierential Diagnosis
Signs and Symptoms Variants of Normal
Learning Outcomes
At the end of this chapter, the student will be able to:
1.1. Define and describe all key terms 1.5. Explain why using descriptive termi-
included in the chapter. nology for pathological conditions is
1.2. Explain the ways general and oral important in dental hygiene practice.
maxillofacial pathology impacts 1.6. Demonstrate key elements of a
dental hygiene practice. proper head, neck, and intraoral
1.3. Describe characteristics included in examination.
physical assessment of the patient and 1.7. Explain the role of supplemental diag-
explain why they are important. nostic aides in establishing a diagnosis.
1.4. Compare and contrast characteristics 1.8. Recognize common variants of
of signs and symptoms. normal oral structure.
OVERVIEW OF ORAL AND main focus of dental and dental hygiene practice.
MAXILLOFACIAL PATHOLOGY However, many other diseases affect the oral and
Pathology (Greek. pathos suffering + logia study) is maxillofacial structures. Some are unique to the oral
defined as the study or science of disease and in- cavity, whereas others are oral manifestations of dis-
cludes its causes, development, course, and effects. eases elsewhere in the body. Although diagnosis
The most common diseases affecting the oral cavity is generally not within the scope of dental hygiene
are dental caries and periodontal disease. Prevention practice, disease recognition is of great concern to
and treatment of these two diseases comprise the the dental hygienist who is in an optimal position to
1
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2 Chapter 1 Introduction to Oral and Maxillofacial Pathology and the Study of Disease
examine the entire oral cavity, discuss signs and Assessment of Oral Pathologic Lesions
symptoms with patients, and provide patient educa- The first step in the assessment of oral pathologic con-
tion. Therefore, a basic knowledge of disease is es- ditions is recognition of a lesion, a general term de-
sential for successful dental hygiene practice. fined as a pathological change in an organ or tissue
The study of oral pathology encompasses general that alters normal form or function. Identification
pathology, the study of basic disease processes such as and assessment of oral lesions is best performed using
infection, autoimmunity, and neoplasia. The study of a methodical approach that includes a complete patient
systemic pathology includes diseases that affect spe- history; assessment of signs and symptoms; a complete
cific organ systems, such as cardiovascular, respiratory, head, neck, and intraoral exam; and use of clinical and
gastrointestinal, or endocrine. Knowledge of both laboratory diagnostic procedures.
general and systemic pathology is important for the
dental hygienist in treating patients with both localized Patient Assessment and History
and systemic pathology. Patient assessment is the cornerstone of dental hygiene
Oral and maxillofacial pathology is the study of dis- practice. It is fundamental in the early detection of
eases that affect the oral cavity and facial structures. pathological conditions and systemic problems that
Oral pathologists are dentists with advanced training may pose a serious threat to the patient and or dental
in oral pathology devoted to the diagnosis of oral dis- team. Determining health status in patients is also ex-
ease. This chapter provides an overview of oral and tremely important for two reasons. First, it may lead
maxillofacial pathology. It provides important infor- to the identification of undiagnosed medical condi-
mation on the recognition, assessment and documen- tions. Second, it enables ongoing evaluation of known
tation of disease, as well as a basic foundation for conditions for progression and changes that may im-
effective communication between clinicians and pa- pact dental treatment. Deferral of dental treatment
tients. Additionally, examples of variations of common may be required and the patient may need referral to
oral findings that are considered within the range of an appropriate health-care provider if new conditions
normal but often mistaken for pathology are reviewed. or changes in existing conditions are detected.
Patient assessment includes obtaining complete
The Practice of Oral and Maxillofacial medical, dental, and social histories, physical evalua-
Pathology tion of the patient, and performing head and neck and
intraoral examinations. Ultimately, patient assessment
Oral and maxillofacial pathology is the dental specialty
determines decisions about clinical treatment and di-
that identifies and manages diseases affecting the oral
rectly impacts quality of patient care.
and maxillofacial regions and investigates the causes,
Patient assessment begins the first moment a pa-
processes, and effects of these diseases. The practice of
tient is observed in the waiting room or dental opera-
oral and maxillofacial pathology involves the diagnosis
tory. Physical evaluation and the process of updating
of disease through the use of clinical, radiographic,
or taking a comprehensive medical and dental history
microscopic, and/or biochemical examinations. Oral
are critical before undertaking head and neck and in-
pathologists collaborate with many other dental and
traoral examinations. This is important because some
medical professionals to advance oral health care needs
medical problems have oral manifestations that are
of their patients by providing consultation and diag-
easily observed, such as spontaneous gingival bleeding
nostic services.
in hemophilia; while other medical problems, such as
Forensic odontology is a branch of forensic medi-
hypertension, have no oral manifestations, but their
cine that deals with matching characteristics of the teeth
medications may produce visible oral side effects.
or oral cavity with existing dental records. Forensic
odontologists identify victims of accidents or contribute
expertise to other tragedies with legal ramifications. Al- Clinical Implications
though oral pathologists may serve as forensic patholo-
Many patients do not consider the herbal supple-
gists, one does not have to be an oral pathologist to
ments and vitamins they take to be medications.
participate on a forensic team. In fact, many dental hy-
Herbal supplements may produce important side
gienists use their superior knowledge of the oral cavity
eects, and many people are allergic or otherwise
to serve on forensic teams that include physicians, den-
sensitive to these often powerful agents. Be sure to
tists, crime scene investigators, funeral directors, and
include these items in your medical history review.
other experts.
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Chapter 1 Introduction to Oral and Maxillofacial Pathology and the Study of Disease 3
The social history includes important information Head and neck examinations are accomplished
on drug, alcohol, and tobacco consumption. Alcohol through procedures known as inspection, palpation,
along with tobacco contributes to the development of auscultation, and percussion. Inspection is the critical
pathologic lesions found in the oral cavity, including appraisal involving examination, measurement, and
oral cancer. Questions about other habits, including comparison with normal. Palpation occurs when the
type of oral health care products and use of alternative examiner feels the size, shape, or firmness of the tis-
therapies, may also contribute important information sues. One or several fingers are used to feel below the
for evaluation of risk factors for pathology in patients. surface skin for thickenings and masses not visible on
Diagnosis is the identification of a disease from its the mucosal surface. Auscultation is the act of listen-
signs and symptoms. Although diagnosis is not within ing to body sounds to evaluate if a structure is normal
the scope of dental hygiene practice, recognition of ab- or abnormal. This can be performed by listening with
normalities and their proper documentation, as well as the unaided ear or with a stethoscope. Auscultation is
patient education, are. To properly document abnor- used in evaluation of the temporomandibular joint
malities, it is important to become familiar with the ter- (TMJ) to detect crepitus (grating or crackling) or pop-
minology of pathology. ping. Percussion is tapping on a surface to determine
The etiology of a disease refers to its cause. If a dis- the condition of the underlying structure. It can be
ease has more than one etiology, these are referred to performed with the fingers, hand, or an instrument.
as etiologic factors or agents. Examples of etiologic This is helpful in determining both the sensitivity and
factors include trauma, genetic abnormality, and infec- relative density of the structure (i.e., whether it is solid
tion. Iatrogenic etiology means the lesion occurred as or hollow). Percussion is used in the evaluation of
a result of treatment. A facticial lesion is an injury that muscles and bones, and is also applied to the teeth to
is self-induced, either internationally or unintention- determine sensitivity.
ally. If the cause of a disease is unknown, the term Table 1-1 reviews the basic steps in the extraoral
idiopathic is used. head and neck examination and illustrates the type and
variety of lesions that may be discovered at each step.
Signs and Symptoms Table 1-2 reviews the basic steps of the intraoral
Signs are objective findings observed by the clinician examination and illustrates intraoral lesions that may
that the patient may or may not be aware of. An ex- be discovered at each step.
ample is a painless white patch on the lateral border of
the tongue. Symptoms are subjective findings per- Terminology Used in Describing Oral
ceived by the patient but not observable by the clini- Pathologic Lesions
cian. Examples are fatigue, headache, anxiety, and Significant clinical findings must be clearly described
sensations such as tingling, itching, burning, bad taste, and documented in the patient record using proper de-
soreness, or numbness. scriptive terminology. Consistent descriptive terminol-
Symptomatic means the lesion is causing pain or ogy is essential for:
other symptoms perceived by the patient. Asymptomatic
1. Accurate record-keeping
means the lesion is not causing pain or symptoms.
2. Comparison of lesions between visits
Patients are often unaware of a lesion until a clinician
3. Clear communication among colleagues
brings it to their attention.
4. Development of a differential diagnosis based on
clinical appearance
Head, Neck, and Intraoral Neck
Examinations When describing a lesion, the anatomic location,
color, shape, borders, and consistency all need to be
In order for a disease or lesion to be diagnosed, it must
recorded. See Table 1-3 for descriptive terminology
first be recognized. The patient may bring it to the at-
and examples of lesions that illustrate each term. For
tention of the clinician, or it may be discovered during
obvious reasons, consistency cannot be illustrated here.
routine examination. Structures in the head and neck
In addition to basic descriptive terminology, the fol-
to be examined include the muscles of the face and
lowing specific terminology is generally used for some
neck, salivary glands, lymph nodes, oral mucosa, and
of the most commonly encountered lesions.
skin. Careful examination will reveal normal anatom-
ical structures, variations from normal, or pathologic Vesicle: a small blister (< 5 mm) located below the
changes. epithelium that is filled with clear fluid. These
(Text continued on page 15)
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4 Chapter 1 Introduction to Oral and Maxillofacial Pathology and the Study of Disease
Table 1.1 Steps for the Extraoral Head and Neck Examination and Examples of Lesions That
May Be Encountered
Steps for Head and Neck Examination Significant Finding and Chapter Number
Chapter 1 Introduction to Oral and Maxillofacial Pathology and the Study of Disease 5
Table 1.1 Steps for the Extraoral Head and Neck Examination and Examples of Lesions That
May Be Encounteredcontd
Steps for Head and Neck Examination Significant Finding and Chapter Number
6 Chapter 1 Introduction to Oral and Maxillofacial Pathology and the Study of Disease
Table 1.1 Steps for the Extraoral Head and Neck Examination and Examples of Lesions That
May Be Encounteredcontd
Steps for Head and Neck Examination Significant Finding and Chapter Number
Table 1.2 Steps for Intraoral Examination and Lesions That May Be Encountered
Steps for Intraoral Examination Significant Finding and Chapter Number
Chapter 1 Introduction to Oral and Maxillofacial Pathology and the Study of Disease 7
Table 1.2 Steps for Intraoral Examination and Lesions That May Be Encounteredcontd
Steps for Intraoral Examination Significant Finding and Chapter Number
Leukoplakia (2)
Continued
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8 Chapter 1 Introduction to Oral and Maxillofacial Pathology and the Study of Disease
Table 1.2 Steps for Intraoral Examination and Lesions That May Be Encounteredcontd
Steps for Intraoral Examination Significant Finding and Chapter Number
Leukoplakia (2)
Palpate the oor of the mouth bimanually. Use index
nger of one hand to press the oor of the mouth
down against ngers of the opposite hand, placed
externally under the chin and mandible.
Chapter 1 Introduction to Oral and Maxillofacial Pathology and the Study of Disease 9
Table 1.2 Steps for Intraoral Examination and Lesions That May Be Encounteredcontd
Steps for Intraoral Examination Significant Finding and Chapter Number
Continued
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10 Chapter 1 Introduction to Oral and Maxillofacial Pathology and the Study of Disease
Chapter 1 Introduction to Oral and Maxillofacial Pathology and the Study of Disease 11
Continued
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12 Chapter 1 Introduction to Oral and Maxillofacial Pathology and the Study of Disease
Chapter 1 Introduction to Oral and Maxillofacial Pathology and the Study of Disease 13
Borders
Well-Circumscribed
Diuse
Regular
Irregular
Continued
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14 Chapter 1 Introduction to Oral and Maxillofacial Pathology and the Study of Disease
Elevated
Flat
Surface Texture
Smooth
Chapter 1 Introduction to Oral and Maxillofacial Pathology and the Study of Disease 15
Consistency
Soft
Spongy
Compressible
Fluctuant: uid lled
Firm
Indurated: extremely
rm
Bony hard
are seen in herpes simplex and herpes zoster Macule: a small (< 1 cm), well-defined, nonelevated
infections. (See Chapter 3.) area of discoloration on the skin or mucosa.
Bulla: a blister > 5 mm (bullae pl.). These larger Freckles and oral melanotic macules on the lip
fluid-filled lesions are observed in blistering con- are examples. (See Chapter 2.)
ditions such as pemphigus and pemphigoid. (See Papule: small (< 1 cm), solid elevations that are not
Chapter 4.) fluid-filled or pus-filled. Papules may be seen in
Pustule: a small (< 1 cm), creamy-white, inflamed, lichen planus of the skin. (See Chapter 4.)
blister-like elevation containing pus. These are Nodule: a solid, well-circumscribed, palpable mass
seen in acne and chickenpox. (See Chapter 3.) 1 cm that arises from deeper tissues. Fibromas
Cyst: an epithelial-lined cavity below the body sur- present as palpable nodules. (See Chapter 2.)
face containing liquid or semi-solid material. Tumor: by definition refers to any swelling or collec-
Apical (radicular) cysts occur along the root or tion of cells appearing as a mass or lump, com-
at the apex of nonvital teeth. (See Chapter 5.) monly used to refer to a neoplasm. Neoplasms
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16 Chapter 1 Introduction to Oral and Maxillofacial Pathology and the Study of Disease
may be benign or malignant. Lipomas and carci- Fissure: linear clefts or cracks occurring in soft tissues.
nomas are examples. (See Chapter 7.) An example is fissured tongue. (See Chapter 5.)
Erosion: refers to a condition in which the superfi- Sinus tract: a channel from deep tissues that com-
cial epithelial surface of skin or mucosa is miss- municates between an area of suppuration (pus)
ing. It may occur as a result of trauma or disease, and surface epithelium or mucosa. A sinus tract
such as when vesicles and bullae break. (See may develop between an apical abscess and the
Chapters 2 and 3.) oral mucosa. (See Chapter 2.)
Ulcer: refers to an area of missing surface epithe-
lium that extends down into the deeper tissues. Supplemental Diagnostic Aides
Ulcers may result from trauma, infections, or
In many instances, a definitive diagnosis cannot be de-
neoplasms such as squamous cell carcinoma.
termined based only on the clinical examination. Ad-
(See Chapter 7.)
ditional diagnostic procedures may be required to
Scar: tissue defects comprised mainly of collagen,
obtain further information. Examples include:
left over after a wound has healed. They are most
commonly seen on the skin. (See Chapter 2.) Imaging: including periapical, bitewing, and panoramic
Plaque: describes a flat, slightly raised patch or a radiographs, cone beam (CT) scans, and magnetic
layer of material deposited on a tissue surface. resonance imaging (MRI). Table 1-4 describes
Reticular lichen planus in the oral cavity is an terms used when interpreting radiographic images
example. (See Chapter 4.) and examples of lesions that illustrate them.
Unilocular apical radiolucency Single compartment around Apical cyst or apical periodontitis (2)
apex of tooth.
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Chapter 1 Introduction to Oral and Maxillofacial Pathology and the Study of Disease 17
Continued
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18 Chapter 1 Introduction to Oral and Maxillofacial Pathology and the Study of Disease
Radiopacity (poorly dened) Cortical bone fades into Osseous dysplasia, endstage (2)
surrounding tissue
Chapter 1 Introduction to Oral and Maxillofacial Pathology and the Study of Disease 19
Clinical Implications
Patients often fear undergoing a biopsy because
they mistakenly equate it with the diagnosis of can-
cer. Dental hygienists are in a position to educate
patients that biopsy does not automatically imply
cancer, but rather is a procedure used to assure
proper diagnosis for a wide spectrum of diseases. Figure 11 Formalin. Ten percent buffered formalin is
the ideal tissue preservative.
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20 Chapter 1 Introduction to Oral and Maxillofacial Pathology and the Study of Disease
Dierential Diagnosis
Exfoliative cytology, also called cytological smear,
Those features that are specifically characteristic of a
involves scraping surface cells from the skin or mucous
particular disease or lesion are referred to as pathog-
membranes. The cells are applied thinly to a glass slide
nomonic. Blistering skin lesions distributed along the
for preservation and staining, and then examined
skin or oral mucosa served by specific nerve pathways
under the microscope. Oral cytological smears are
or dermatomes are pathognomonic of herpes zoster
helpful in the diagnosis of candidiasis (Chapter 3).
(shingles). The appearance of a small white cauliflower-
Brush biopsies or brush tests are used to collect
like lesion, sometimes with finger-like projections, is
cells for cytological evaluation. Because the cells are
pathognomonic of oral papilloma (see Chapter 3).
disassociated from each other during the process, con-
For many lesions, pathognomonic features are not
clusions about the diagnosis may be limited depending
always present, so a strategy must be used to arrive at
on the pathologic condition evaluated.
the final diagnosis. A differential diagnosis is a list of
diseases that share signs and symptoms. Diseases on
this list are then investigated with specific diagnostic
Clinical Implications
tools to help separate and rule them in or out. The
Oral cytological smears are not invasive proce- goal is to narrow the list down to a final specific or de-
dures and require no anesthesia. Therefore, in finitive diagnosis. See Table 1-5 for an example of a dif-
many states, performing cytological smears is ferential diagnosis.
within the scope of dental hygiene practice. Once a diagnosis is established by the sequence
of medical, dental, and social history review, assess-
ment of signs and symptoms, and collection of cells
Laboratory tests may be required to help the pathol- or tissues, the disease may be treated or managed.
ogist reach a definitive diagnosis. These include: Options for treatment and management depend on the
1. serological (blood) tests, such as a complete blood diagnosis. These may include medications, complete
count (CBC), to determine if there is a higher than surgical excision of a remaining lesion, referral to
2577_Ch01_001-026 11/07/14 11:07 AM Page 21
Chapter 1 Introduction to Oral and Maxillofacial Pathology and the Study of Disease 21
Lesion Chapter
Irritation broma 2
Neuroma 7
Lipoma 7
Minor salivary gland tumor 7 Figure 14 Linea alba. Thick white line along the buccal
mucosa where teeth routinely occlude.
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22 Chapter 1 Introduction to Oral and Maxillofacial Pathology and the Study of Disease
Chapter 1 Introduction to Oral and Maxillofacial Pathology and the Study of Disease 23
24 Chapter 1 Introduction to Oral and Maxillofacial Pathology and the Study of Disease
Review Questions
1. Pathology is the study of 7. Which one of the following statements is
A. disease processes. true?
B. infectious diseases. A. Signs are subjective and perceived only by
C. neoplasia. the patient.
D. All of the above B. Signs are objective and observed by the
clinician.
2. A lesion is a C. Examples of signs are fatigue, anxiety, and
A. symptom of cancer within the tissues. sensations.
B. defined area of pathologic tissue alteration. D. Symptoms are observed by both clinicians
C. cytologic smear. and patients.
D. tissue biopsy.
8. Inspection is the critical appraisal of a patient
3. A biopsy is for pathology and includes
A. a surgical procedure. A. examination.
B. performed when the condition cannot be B. measurement.
diagnosed with clinical procedures alone. C. comparison with normal.
C. obtained in order to determine the defini- D. All of the above
tive diagnosis.
D. All of the above 9. Auscultation is
A. tapping on a surface to evaluate the under-
4. Cytology lying tissue structure.
A. involves removal of deep tissue cells for B. the act of listening to body sounds.
microscopic examination. C. used to determine hypersensitivity of teeth.
B. studies individual cells that are within biop- D. performed using the fingers and/or hands.
sied tissue.
C. involves removal of superficial tissue cells 10. Laboratory tests to help reach a definitive di-
for microscopic examination. agnosis include all of the following EXCEPT:
D. is contraindicated in cases of candidiasis. A. urinalysis.
B. microbiologic cultures.
5. The first step in the assessment of oral pathol- C. radiographs.
ogy is D. complete blood count.
A. recognition.
B. biopsy. 11. An excisional biopsy of an oral lesion is
C. cytology. performed
D. laboratory testing. A. when a lesion is large.
B. to cure the patient.
6. Patient assessment C. when a lesion is small and can be totally
A. includes obtaining medical, dental, and removed.
social histories. D. when a lesion has gone unnoticed for a
B. impacts proposed dental care. prolonged period of time.
C. includes physical evaluation of the patient.
D. All of the above
2577_Ch01_001-026 11/07/14 11:07 AM Page 25
Chapter 1 Introduction to Oral and Maxillofacial Pathology and the Study of Disease 25
12. Oral cytologic smears are helpful in 18. Which one of the following statements is false
A. diagnosing candidiasis. about tori?
B. evaluating allergic reactions. A. They represent excessive growths of normal
C. identifying normal antibody titers. bone.
D. identifying abnormal antibody titers. B. They are exostoses.
C. They may occur in the palate or bilaterally
13. Pathognomonic features along the lingual aspect of the mandible.
A. do not include signs. D. They most commonly occur in the pres-
B. do not include symptoms. ence of other disease processes.
C. are virtually characteristic of a particular
disease. 19. Which one of the following statements is
D. are only suggestive of a particular disease. false?
A. Lingual tonsils are found immediately be-
14. A list of diseases that share signs and symp- neath the foliate papillae.
toms is known as B. Hairy tongue indicates a serious disease
A. a treatment plan process.
B. the differential diagnosis. C. In the skin, fordyce granules are oil glands.
C. the definitive diagnosis. D. Leukoedema is most common in dark-
D. abnormal variation. skinned individuals.
C h a p t e r
2
Orofacial Injury and Reactive Disorders
Cell and Tissue Response to Injury Clinical Features of Orofacial Soft Tissue Injury
Oral Pathologic Lesions Due to Injury Injuries to the Mucous Membranes
Reversible Cell Injury Proliferative Repair Responses
Irreversible Cell Injury Injuries to the Gingiva
The Inflammatory Response Traumatic Salivary Gland Disorders
Inammation and the Inammatory Response Denture and Prosthesis-Related Injury
Three Basic Types of Inammation Pulpal Injury and Its Sequelae
Cardinal Signs and Symptoms of Inammation Radiolucent Periapical Pathology
Chemical Mediators of Inammation Radiopaque Periapical Pathology
Cessation of Inammation Clinical and Radiographic Features of Oral Hard
Cellular Interactions in the Inammatory Process Tissue Injury
Systemic Eects of Inammation Bone Injury
Postinammatory Tissue Healing External Injuries to Teeth
Learning Outcomes
At the end of this chapter, the student will be able to:
2.1. Explain the difference between re- 2.7. Differentiate among the clinical fea-
versible and irreversible cell injury. tures of frictional keratosis, pigmented
2.2. Differentiate among the reactive tattoos, hypermelanosis, traumatic
processes of hyperplasia, hypertro- ulcerations, burns, nicotine stomatitis,
phy, atrophy, metaplasia, dysplasia, geographic tongue, hematoma, foreign
necrosis, and ischemia. body reaction, and keloid.
2.3. Discuss the role of free radicals in 2.8. Differentiate among the clinical
irreversible cell injury. features of the proliferative repair
2.4. Discuss the three basic types of inflam- responses pyogenic granuloma, trau-
mation and give an example of each. matic fibroma, traumatic neuroma,
and verruciform xanthoma.
2.5. Explain the cardinal signs of
inflammation. 2.9. Discuss the etiology and clinical
identifying features of injuries to the
2.6. Explain the differences among heal-
salivary glands, gingiva, dental pulp,
ing by primary intention, secondary
bones of the jaws, and the teeth.
intention, and tertiary intention.
27
2577_Ch02_027-076 11/07/14 11:08 AM Page 28
CELL AND TISSUE RESPONSE TO INJURY Table 2.1 Types of Cell Injury
All disease originates at the cellular level. When cells
are stressed or injured, they undergo a variety of Reversible Irreversible
adaptive changes depending on the particular stressor Hyperplasia Necrosis
and the ability of the cell to respond. Basically, cells
Hypertrophy Ischemia
recover, adapt, or die. This chapter presents general
principles of how the body responds to injury and how Atrophy Apoptosis
it restores form and function following injury. Com- Metaplasia Free radical damage
mon oral injuries and their effects on the oral cavity Dysplasia Pathologic calcication
are presented.
Intracellular pigments Cell aging
Hyperplasia Atrophy
Hyperplasia is an increase in the size of an organ or tis- Atrophy refers to decrease in size of cells, organs, tissues,
sue due to an increase in the number of cells. Hyperpla- or body parts because of disease, hormonal alteration,
sia can be physiologic or pathologic. Physiologic injury, or lack of use. Atrophy may be physiologic or
hyperplasia is a response to a specific stimulus in which pathologic. Physiologic atrophy is generally not re-
the cells remain subject to normal regulatory control versible. For example, once a female reaches menopause,
mechanisms. It may be caused by increased demand, the ovaries begin to decrease in size and atrophy. An
such as that seen in a remaining kidney when one has example of reversible physiologic atrophy is muscle
been removed for donation (Fig. 2-1A). Physiologic hy- weakness/wasting (atrophy) that occurs when a broken
perplasia also occurs when hormone changes result in arm is kept in a cast and not used (disuse atrophy)
enlargement of the breasts during pregnancy and lacta- (Fig. 2-1E). An example of pathologic atrophy is loss of
tion. On the other hand, pathologic hyperplasia can be filiform papillae on the tongue following long-standing
seen in the reaction of the gingival tissues (Fig. 2-1B) candidiasis (Fig. 2-1F). Once the fungal infection resolves
2577_Ch02_027-076 11/07/14 11:08 AM Page 29
Physiologic Pathologic
Normal cells
Hyperplasia A B
Hypertrophy
C D
Atrophy
E F
Figure 21 Physiologic versus pathologic changes. A. Kidney shows physiologic
hyperplasia. B. Gingival hyperplasia is pathologic. C. Physiologic hypertrophy can be
seen in pregnancy. D. Bruxism can produce muscle hypertrophy. E. Physiologic atrophy
can occur with disuse, as in this example of a broken arm in a cast. F. An example of
pathologic atrophy is atrophic glossitis.
(injurious agent is removed), the tongue tissue regains for developing squamous cell carcinoma of the lung.
some or all of its former level of functioning. With timely elimination of an irritant, such as tobacco
smoke, cells may revert to their normal state.
Metaplasia Another example of metaplasia occurs in the sali-
Metaplasia is caused by a stimulus that changes one vary glands. Normal epithelial cells of salivary gland
cell type into another. An example can be seen in the ducts and acini undergo metaplasia from simple
epithelial lining of the respiratory tract. When the cuboidal epithelium to larger cells called oncocytes.
normal pseudostratified ciliated columnar epithelium The resulting nodular oncocytic metaplasia may create
of the respiratory tract comes into contact with to- enlargement of the tissues and give the erroneous im-
bacco smoke, it may transform to stratified squamous pression that a tumor is present. This type of metapla-
epithelium, which is thicker and more protective. But sia occurs most often in the parotid gland and appears
stratified squamous epithelium has no cilia or mucous to be a degenerative phenomenon related to aging.
to protect the respiratory tract from common irri-
tants. Therefore, inflammatory pulmonary disorders Dysplasia
may develop. Also, because the most common form Dysplasia is defined as lack of proper maturation of
of lung cancer is squamous cell carcinoma, the new a tissue. When cells are unable to mature, the tissue
stratified squamous epithelium presents a higher risk cannot properly develop. The classic example is the
2577_Ch02_027-076 11/07/14 11:08 AM Page 30
Ischemia
Ischemia is a restriction in blood supply generally due
to damaged blood vessels. The result is damage, dys-
function, or death of tissue supplied by that blood vessel,
called infarction. For example, blockage of coronary
arteries causes ischemia of the cardiac muscle, referred
to as myocardial infarction. Blockage of cerebral arteries
leads to a cerebrovascular accident (CVA), commonly
called a stroke. Although some forms of ischemia are
transient and do not result in permanent damage,
ischemia typically causes permanent irreversible injury A
to tissues.
Necrosis
Necrosis is the death of cells and tissues that fail to
adapt to changes in their environment. Pathologic
necrosis occurs when normal cell functions cannot be
sustained due to infection, toxins, trauma, or lack of
oxygen. For example, infarction of the cardiac muscle
(blockage of blood flow to the heart) causes necrosis
of myocardial cells due to lack of oxygen. Once cells
and tissue become necrotic, they cannot be repaired.
Several types of necrosis can develop, depending on
the type of injury. B
Coagulative necrosis results from lack of oxygen to Figure 24 Coagulative necrosis. A. Normal kidney
the cell (hypoxia). As the cells die, the general cell B. Kidney necrosis due to lack of oxygen; cell death with
architecture of the tissue is preserved and can be preservation of tissue form.
2577_Ch02_027-076 11/07/14 11:08 AM Page 32
Pathologic Calcification
Pathologic calcification is defined as abnormal dep-
osition of calcium and may be dystrophic or metasta-
tic. Dystrophic calcification occurs in areas of necrosis
when blood calcium is at normal levels. It indicates
that the ability of cells to properly metabolize calcium
has been altered or destroyed. This can be seen in ar-
teries in advanced cases of atherosclerosis and in heart
Figure 25 Liquefactive necrosis. Enzymatic breakdown
valves that have been damaged previously by rheu-
of cells resulting in a liquid mass.
matic heart disease. Metastatic calcification occurs when
blood calcium levels are elevated, a condition called
injury. Apoptosis occurs when a specific gene turns hypercalcemia. This occurs in hyperparathyroidism,
on and turns off during embryogenesis, allowing bone disease, vitamin D disorders, and renal failure.
tissues and organs to assume their proper form. In- Metastatic calcification most frequently affects the
appropriate or excessive apoptosis causes atrophy; gastrointestinal tract, kidneys, and lungs, causing dis-
lack of proper apoptosis results in uncontrolled cell ruptions in normal function.
proliferation. Pathologic apoptosis can be seen in
some tumors and inflammatory disorders.
Clinical Implication
Free Radicals Areas of dystrophic calcication can be observed
Free radicals are molecules responsible for aging, tis- in both vital and necrotic pulp tissues. Proposed
sue damage, and a number of diseases. The molecules causes of pulpal calcications include irritation,
are unstable because they are incomplete, lacking an injury, and reaction to the caries process. In
even number of electrons. Instead, they have a single some cases of pulp injury, calcium is deposited
unpaired electron in their outer shell, making them into the inamed pulp. The resulting dystrophic
highly reactive. Free radicals seek to bond with other calcications can be seen histologically and on
molecules, capturing electrons to become complete or occasion radiographically.
stable. The body creates free radicals for many impor- Secondary dentin formation and/or dystrophic
tant functions. One example is the neutralizing or calcication may result in total obliteration of
killing of bacteria and viruses. In the process they may pulp chambers and canals (Fig. 2-7A and B).
produce collateral cell damage, causing cells to mal-
function, become cancerous, or die. Free radicals
may also form after exposure to chemicals, environ- Cell Aging
mental toxins, radiation, or tobacco smoke, to name a Cell aging is related to changes in genetic factors,
few examples. diet, social conditions, and ongoing illness. Only a
Free radicals inflict damage when they react with fixed number of divisions occur in normal cells before
cell membranes or cellular DNA. Damage to cell mem- they die, a process called senescence. Senescent cells lose
branes is via peroxidation of membrane lipids, a process their ability to self-repair chromosomal damage and
that has been implicated in some diseases as well as in take up nutrients. This slowing down and loss of func-
aging. Antioxidants are defined as molecules that in- tion manifests as the aging process.
hibit or counteract oxidation. They prevent or repair
damage to cells by free radicals. Problems result if the
number of free radicals increases beyond the bodys THE INFLAMMATORY RESPONSE
ability to neutralize or destroy them. The major dam- Inflammation is not a disease but an attempt by the
age is most often to DNA or cell membranes, leading body to counteract injurious stimuli and to repair
2577_Ch02_027-076 11/07/14 11:08 AM Page 33
Ultraviolet rays
Stress
Cytoplasm
Cell
membrane
Poor nutrition DNA being destroyed
by free radicals
Electron
the damage they create. It is referred to as a response from a variety of injuries. Keep in mind that inflam-
because of the bodys innate ability to defend itself mation also occurs as a result of exposure to infectious
against injury. microorganisms (see Chapter 3) allergens or with
autoimmune diseases, and autoimmune diseases,
Inammation and the Inammatory such as rheumatoid arthritis (see Chapter 4).
Response The duration and extent of the inflammatory re-
Inflammation is considered an essential defense sponse depends on the injury. It may be local and lim-
mechanism necessary for health. When a tissue is in- ited, or it may become systemic and widespread if the
volved in the inflammatory response, it is referred to injury is extensive. It is a dynamic process involving
as inflamed. The suffix -itis is used to indicate the complex cellular and chemical reactions. Redundant
specific inflammatory pathology (e.g., gingivitis or systems within the body allow for variations in the in-
tonsillitis). Inflammation is essential in recovering flammatory response and provide backup for deficient
2577_Ch02_027-076 11/07/14 11:09 AM Page 34
Acute Inflammation
Acute is a general term that means abrupt onset and
short duration. Acute inflammation occurs immedi-
ately after an injury and is characterized by exudative
processes in which fluid, plasma proteins, and cells
leave the bloodstream and enter the tissues. Polymor-
phonuclear leukocytes (PMNs, or neutrophils) are the
predominant cells involved in the acute inflammatory
response.
Chronic Inflammation
Chronic is a general term that means gradual onset
over time and of longer duration. Chronic inflammation
often follows acute inflammation, but may occur with-
out a pre-existing acute inflammatory response. An ex-
ample is deep caries affecting the dental pulp over time.
Microscopically, chronic inflammation differs from
A acute inflammation in that lymphocytes, plasma cells,
and macrophages are the predominant cells. Chronic
inflammation can be described as a proliferation of
cells, neovascularization (new blood vessel formation),
and formation of new connective tissue.
Chronic inflammation may develop in two ways,
depending on the nature of the inflammatory stimulus
involved. It may (1) arise following an acute inflamma-
tory response that has not resolved or (2) develop in
the absence of a preceding acute response, especially
in infections and autoimmune diseases, which are dis-
cussed in Chapters 3 and 4.
Granulomatous Inflammation
This is a special subtype of chronic inflammation. It
occurs when cells of the chronic inflammatory response
attempt to wall off substances that are perceived as for-
eign but that cannot be eliminated by the acute and
chronic inflammatory processes alone. These walled-
off zones of tissue are referred to as granulomas and
B
can sometimes be detected clinically as nodules. Many
Figure 27 Pulp calcication. A. Pulp chamber and pathologic nodules are referred to as granulomas;
canals have calcified in response to injury. B. Calcification however, a nodule cannot be called a true granuloma
of pulp in lower anterior teeth.
without specific microscopic criteria. Some specific
infectious microorganisms (see Chapter 3) elicit gran-
or deactivated constituents of this response. Unfortu- ulomatous inflammation, as do some foreign objects.
nately, the inflammatory response can cause pain, dis- Granulomas are persistent and only heal after the
ability, and collateral tissue damage. etiologic agent is eliminated.
These signs are clearly observed with injury to the skin accumulation within tissue. The process by which fluid
and underlying tissues. and plasma proteins leave the blood vessels in inflam-
matory conditions and enter the tissues is called exu-
Vascular Events dation. Exudates can range from serous (clear fluid with
The bodys initial response to physical injury, such as few cells) to purulent (fluid with many dead cells).
a paper cut or skin scrape, is vasoconstriction. Tran- Edema refers to an abnormally large amount of leakage
sient pallor, or paleness of the skin, may occur as vessels of fluid from the bloodstream into the surrounding
constrict, followed immediately by vasodilatation. This tissues, causing tissue swelling or distortion. This
is clearly demonstrated in paper cuts, where bleeding swelling can put pressure on nerve endings, resulting
is initially stopped via vasoconstriction, then followed in pain. Although swelling from edema may appear
immediately by bleeding resulting from vasodilation. harmful, it has several positive aspects, as indicated in
Fluid movement and cell migration from blood vessels Table 2-4.
to the site of injury also results in redness and swelling.
Increased blood viscosity and slower blood flow occur Chemical Mediators of Inammation
as fluid is lost from local blood vessels. This allows Inflammation is controlled by substances known as
leukocytes (white blood cells) to attach to vessel walls chemical mediators, each of which has a specific role at
and further increase resistance to blood flow. a specific stage of the inflammatory response. Three
major groups of mediators include vasoactive amines,
Transudation and Edema
plasma proteases, and the fibrinolytic system.
Migration of white blood cells (leukocytes) out of the
blood vessels helps promote fluid and plasma protein Vasoactive Amines: Histamine and Serotonin
Histamine increases vascular permeability and causes
the contraction of smooth muscle. Serotonin is a chem-
Table 2.3 Cardinal Signs and Symptoms ical messenger that can produce blood vessel narrow-
of Inflammation ing and transmit signals between nerve cells.
Pain (Dolor) Fluid pressure and pain- Provides nutrients for inammatory cells
producing agents such Contains protective antibodies
as kinins Contains brinogen, which removes collagen to facilitate
Loss of function Swelling and/or pain movement of inammatory cells into the wound
2577_Ch02_027-076 11/07/14 11:09 AM Page 36
Platelets (Fig 2-8C) make an important contribution increase blood glucose, suppress the immune system,
in injury, inflammation, and repair by playing a role in and aid in fat, protein, and carbohydrate metabolism.
activating complement, binding immune complexes, Serum cortisol levels increase soon after the onset of
enhancing vascular permeability, and participating in inflammation stresses the body. Corticosteroids coun-
the coagulation cascade. They are not cells but rather teract the effects of the inflammatory response.
small fragments of cytoplasm derived from larger pre-
cursor cells called megakaryocytes. As they circulate in Leukocytosis
the blood, their primary function is hemostasis Leukocytosis is an increase in the circulating leuko-
through adherence to the site of vascular injury, where cyte count in the blood due to the additional release
they form a hemostatic plug. Platelets have a lifespan of neutrophils from the bone marrow. The white cell
of 5 to 9 days, during which time they release multiple count typically reaches 15,000 or 20,000 per mm3 but
growth factors. may reach levels of 40,000 to 100,000 per mm3.
Megakaryocyte
Granulation Tissue
Granulation tissue forms at the site of an injury as the
body starts to rebuild. Whenever there is an injury, the
natural response is for granulation tissue to form (Fig.
2-10A). Granulation tissue acts as a scaffold upon which
new tissue will develop. Granulation tissue, no matter A
where it is found, is composed mainly of chronic inflam-
Fibroblast Angioblast
matory cells, fibroblasts, and angioblasts (Fig. 2-10B).
Fibroblasts produce an extracellular matrix that serves
as a foundation upon which new tissue will develop to
repair the wound. Angioblasts form tiny new vascular
channels to aid in reconstruction, a process called neo-
vascularization. If the source of the injury is not removed
or if the injured site becomes secondarily traumatized,
there may be an excess buildup of granulation tissue in
response to the continuous damage. The body will con-
tinually attempt to complete the repair, but it is impeded
by continued assault via the injurious agent. If inflam-
mation is resolved quickly, little additional tissue injury
results; however, if inflammation persists, excessive tis- B
sue injury can occur. The resultant chronic inflamma-
Chronic inflammatory cell
tion can produce additional problems, such as joint
destruction, as seen in rheumatoid arthritis, or bone loss Figure 210 Granulation tissue. A. Exuberant granulation
tissue response to injury of buccal mucosa. B. Chronic
around teeth, as seen in periodontitis. inflammatory cells, fibroblasts, and angioblasts make up
granulation tissue.
Clinical Implications
Healing
A common example of inability of the body to
Healing associated with physical tissue trauma is
proceed with completion of its repair function is
classified as healing by primary, secondary, or tertiary
the so-called pyogenic granuloma, incorrectly
intention.
named because it is neither pyogenic (pus-
producing) nor granulomatous (see Figs. 2-10A Primary Intention
and B). A pyogenic granuloma is simply a local-
Healing by primary intention occurs when wound edges
ized exuberant attempt at repair. Pyogenic gran-
can touch each other or can be held together by surgical
ulomas are overgrowths of granulation tissue in
staples, sutures, or tape. Examples are surgical incisions
response to a local injury that is not resolved.
and injuries with minimal tissue loss, such as paper cuts.
Although pyogenic granulomas can occur any-
Complete regeneration is possible, and formation of
where on any skin or mucosal surface, they are
lasting scar tissue is absent or minimal.
common in the oral cavity. The most common lo-
cation in the oral cavity is the gingiva, where the Secondary Intention
injurious agent is typically calculus that acts as a
Wounds in which the edges cannot be closely opposed
persistent irritant. Scaling can help resolve these
heal by secondary intention. Missing tissue limits the
lesions, but occasionally the granulation tissue is
bodys ability to restore pre-wound conditions. Surgical
so bulky that it must be removed surgically.
and trauma cases in which there is removal of significant
2577_Ch02_027-076 11/07/14 11:09 AM Page 40
Excess keratin
Orthokeratinized
stratified
squamous
epithelium
Amalgam, Graphite, and Ethnic Tattoos deposition of larger particles (Fig. 2-16B). This
Amalgam tattoos are the result of accidental implantation means negative radiographic findings do not defini-
of dental amalgam within the oral tissues (Fig. 2-16A). tively rule out amalgam tattoos. A similar phenome-
Injury to oral mucosa may permit amalgam debris to non can be seen in patients, especially children, who
be introduced into tissue. Amalgam can be introduced accidentally implant graphite from a lead pencil into
into oral mucosa in several ways. It can occur during the gingival tissues. Biopsy must be considered for
placement or removal of amalgam restorations or dur- any pigmented area in the oral cavity that cannot be
ing crown preparation, when a slurry containing amal- confirmed radiographically to be an amalgam or
gam particles is produced within the gingival sulcus. graphite tattoo.
Fracture of dental amalgams via trauma or tooth
extraction may also leave residual amalgam within soft
tissues. In addition, endodontic treatment that involved Clinical Implications
placing amalgam at the apex of the tooth can result in Occasionally, a patient with no amalgam
an amalgam tattoo. restorations will present with what appears to
Amalgam tattoos are often confirmed radiograph- be an amalgam tattoo in the premolar area.
ically by detecting the presence of radiopaque amal- This generally represents an amalgam tattoo
gam fragments; however, not all amalgam tattoos initiated at the time of extraction of a decidu-
produce radiographic findings. Amalgam tattoos ous tooth containing an amalgam restoration
may be produced by leaching of tiny silver particles (Fig. 2-16C). Another form of exogenous pig-
into the underlying connective tissue rather than by mentation referred to as ethnic tattooing is
seen in cultures that use this as a decoration or
status symbol (Fig. 2-17).
Hypermelanosis
Hypermelanosis is the result of excess melanin pro-
duced by melanocytes found in the basal layer of strat-
ified squamous epithelium. Melanin is a protective
pigment that can be produced in response to any inju-
rious stimulus. It may be excessive in the oral cavity
under several circumstances described below.
Post-inflammatory hypermelanosis or postinflammatory
hyperpigmentation occurs after the skin or mucous
membranes experience inflammatory events such as
Figure 212 Frictional keratosis. Thickening of surface infection, allergic reaction, trauma, or other inflam-
in edentulous space due to rough foods. matory diseases. Melanin is overproduced in an
2577_Ch02_027-076 11/07/14 11:09 AM Page 42
Linea alba Normal occlusal contact Thin white line on buccal mucosa along line of occlusion
Frictional keratosis Chronic rubbing Thick white plaque that does not wipe o and contacts
rough restoration or prosthesis; also from oral habits
such as chewing on coarse foods
Morsicatio (buccarum, Habitual chewing or nib- Thick white plaque that does not wipe o with a shred-
linguorum, labiorum) bling of buccal mucosa, ded surface texture
tongue, or labial mucosa
Snuff dippers keratosis Direct contact of smokeless Wrinkled, corrugated, or folded pink, white to red; adja-
tobacco product cent gingiva may show recession from teeth
attempt to protect the skin or mucous membranes melanophages, in the connective tissue, causing local-
from injury. This results from the release of ized or diffuse black to brown pigmentation. Postin-
prostaglandins and other inflammatory products stim- flammatory hyperpigmentation is more common in
ulating melanocytes to increase synthesis of melanin. individuals with darker skin.
Melanin becomes trapped by macrophages, called Oral postinflammatory pigmentation is associated
with chronic inflammatory disorders such as oral
lichen planus or pemphigoid (see Chapter 4) and can
Lesion suspected be seen following oral surgery. Lesions may be brief,
to be reactive disappearing shortly after the inflammatory process re-
solves. In other cases, the hypermelanosis may be pres-
ent for many years (Fig. 2-18). These lesions cause
Look for source concern because they may resemble early melanoma.
of irritation In many cases, a biopsy and histological evaluation is
the only way to differentiate between the two. Once
confirmed, postinflammatory hyperpigmentation re-
quires no treatment.
Oral melanotic macule most likely represents a form
Suspected source No source identified of post-inflammatory hyperpigmentation, although
identified and or suspected:
removed or source cannot be
the precise etiology is unknown. It most often presents
relieved removed or relieved as a focal flat (macular) brown discoloration of the oral
mucosa or the vermilion border of the lower lip, both
sites that are readily traumatized. The buccal mucosa,
Patient returns in
23 weeks
Patient returns in
another 2-3 weeks:
lesion still present
Figure 213 Management of a lesion suspected to be Figure 214 Lip chewing. Shredded appearance to labial
reactive/traumatic. mucosa results.
2577_Ch02_027-076 11/07/14 11:09 AM Page 43
A A
B B
Figure 215 Snu dippers keratosis. A. Corrugated
tissue at site of tobacco placement. B. Thick red and white
folded tissue at site of tobacco placement.
Figure 217 Ethnic tattoo. Young woman from Africa Figure 220 Melanoacanthosis. Flat, dark brown-to-
with tattoo placed as a status symbol. black, well-defined area that arises suddenly following a
traumatic event.
Clinical Implications
Oral melanoma is rare. There are several non-
neoplastic disorders of pigmentation that can
mimic oral melanoma. The only way to achieve
certainty about the diagnosis of unidentied pig-
mented lesions is to perform a biopsy. To pre-
vent overlooking a developing melanoma, a good
rule of thumb for pigmented lesions of the oral
cavity is: If a pigmented lesion cannot be identi-
ed and/or does not appear radiographically as
Figure 219 Oral melanotic macule. Oval brown an amalgam tattoo, biopsy it.
discoloration of the lower lip of unknown etiology.
2577_Ch02_027-076 11/07/14 11:09 AM Page 45
Traumatic ulcer Physical injury such as bit- Usually single, well-dened area of erythema surrounding
ing the tongue or contact yellow brinopurulent membrane; slightly raised border;
with sharp object tender/painful; resolves in 7 to 10 days with removal of
etiology
Traumatic ulcerative Traumatic ulcer that re- Long duration; raised rolled border; crater with yellow b-
granuloma ceives persistent mild rinopurulent membrane; nonpainful or mildly tender;
chronic trauma slow to resolve with removal of etiology; often requires
surgical excision and healing by primary intention
Denture ulcer New denture with ill- Ovoid erythematous area with yellow necrotic center;
adapted ange; prolonged contacts irregular area of denture; resolves with denture
denture wearing adjustment
Anesthetic necrosis Ischemia from epinephrine Usually on hard palate at injection site; painful well-
in local anesthetic or dened intense red area with central necrosis; heals
trauma during injection without treatment in 10 to 14 days
Thermal burn Contact with hot foods such Painful yellow to white zone of necrosis of surface mucosa
as pizza of palate or buccal mucosa; tissue sloughs; patient
reports etiologic event; resolve with no treatment
Electrical burn Contact with live electrical Yellow to black painless area that gradually becomes ede-
cord or extension cord matous; sloughs and bleeds; usually on lips of children
Chemical burn Contact with caustic med- Supercial white corrugated or cracked appearance;
ications, dental materials, epithelium sloughs leaving red painful surface; resolves
improper use of anal- without treatment in 10 to 14 days
gesics, mouth rinses
Chemical burns can result when caustic agents come aspirin tablets or powders that contain acetylsalicylic
in contact with the oral mucosa. Chemical burns may acid (Fig. 2-25). Aspirin may cause a significant burn
appear as thick, rough hyperkeratotic plaques with a if placed adjacent to or over a painful tooth, rather
corrugated or cratered surface and/or areas of ulcera- than being swallowing. Patients may use strong agents,
tion. Patients may misuse acidic medications, such as such as hypochlorite (bleach), to clean or disinfect
Clinical Implications
Over-the-counter (OTC) liquid toothache reme-
dies often contain strong chemicals. When over-
used, they may cause burns of the oral mucosa
that are more severe and painful than the origi-
nal toothache. Patients may end up with both
Figure 224 Burns. Burn on prominent bony exostosis
the toothache they were originally trying to treat
after eating hot pizza.
and a nasty mucosal burn. Burns caused by
liquids may appear diuse and irregular, corre-
sponding to uncontrolled ow of the liquid.
Nicotine Stomatitis
Nicotine stomatitis occurs in smokers and is the result
of exposure of the palate to the smoke and heat of
burning tobacco products. It generally appears as a
thick, white plaque of the hard and soft palate, con-
taining scattered, tiny, raised red (erythematous)
dots. The erythematous dots represent irritated
minor salivary gland ducts (Fig. 2-26). Nicotine
stomatitis persists as long as the individual continues
smoking. The lesion itself is benign and will regress
upon smoking cessation.
Figure 225 Aspirin powder burn. Patient with toothache
in maxillary teeth, associated burn from aspirin powder
placement.
Clinical Implications
Lesions similar to nicotine stomatitis have been
complete dentures. Any residual chemical remaining observed in nonsmokers who habitually drink ex-
from incomplete rinsing may also cause significant soft cessively hot liquids such as tea or coee.
tissue burns.
Figure 226 Nicotine stomatitis. Close-up view of Figure 227 Geographic tongue. Characteristic
dilated minor salivary ducts and hyperkeratosis of the asymmetrical well-demarcated areas of the erythematous
palate in a smoker. mucosal atrophy, surrounded by circinate white or
yellowish border.
raised, hairless areas or fibrous nodules, varying from inaccurate because they are not due to pyogenic (pus-
pink to flesh-colored or red to dark brown (Fig. 2-28). producing) bacteria and are not granulomas. Although
They often have a firm, rubbery texture and a smooth, they can occur anywhere on the body, pyogenic gran-
shiny surface. Keloids may be accompanied by severe ulomas frequently occur on the gingiva as a result of
itchiness and pain as well as changes in texture. They local irritation, such as calculus (Fig. 2-29) or ortho-
may occur at the site of minor scratches and cuts, body dontic appliances. These lesions may be either sessile
piercings, severe acne, infected wounds, surgical inci- or pedunculated masses that bleed easily on gentle
sions, or areas of repeated trauma. Treatment includes probing, due to large numbers of engorged capillaries
corticosteroids; however, the best treatment involves that produce the bright red to deep purple coloration.
prevention whenever possible. As the granulation tissue matures, there is less bleeding
and redness (Fig. 2-30). Although some lesions regress
Body Piercing with removal of the irritant, many require surgical ex-
Body piercing is currently in style in the United States; cision. They generally do not recur after removal un-
however, this form of body adornment has been prac- less the irritant remains. Although there is a female
ticed for thousands of years. Body piercing may occur predilection, pyogenic granulomas also occur in males
anywhere on the body, including face, nose, ears, and in response to local irritation.
lips. Piercings in the oral cavity, especially in adoles-
cents and young adults, are seen most commonly on Clinical Implications
the tongue and lips. Complications of piercing such as
pain, bleeding, swelling, foreign body reaction, and in- Pyogenic granulomas in the oral cavity occur fre-
fection may occur. Damage to the lingual nerve may quently in pregnant women. This may be related
be experienced at the piercing site as well as infections. to hormonal changes and/or an increased re-
Contact between metal rings and/or studs that hit the sponsiveness to local irritants. During pregnancy
teeth may result in chipped or fractured teeth. Tongue they are frequently called pregnancy tumors or
piercings have also been reported to contribute to gaps granuloma gravidarum. These terms are mis-
between the front teeth and altered periodontal status, nomers because they are not tumors. In many
including receding gingiva. cases, they resolve or become broma-like after
delivery of the baby.
Proliferative Repair Responses
Proliferative repair responses occur whenever the nor- Traumatic (Irritation) Fibroma
mal healing process becomes exaggerated and over- Traumatic (irritation) fibromas are benign lesions
production of granulation tissue and other wound composed of dense, highly fibrous connective tissue,
repair products result. similar to a scar (Fig. 2-31). They are more appropri-
ately called focal fibrous hyperplasia to denote a reactive
Pyogenic Granulomas
Pyogenic granulomas are exophytic lesions composed of
an exuberant overgrowth of granulation tissue in
response to minor chronic irritation. The name is
A
Figure 230 Pyogenic granuloma. Large pyogenic
granuloma present for several months with decreased
redness and bleeding.
Epithelial
surface
B
Figure 232 Traumatic bromas. A. Pedunculated
Dense fibroma of lower lip. B. Secondary frictional keratosis can
collagen develop on the surface.
involvement of the actual nerve ending leads to pain; that the lesion occurs in the soft tissues rather than
most nerve sheath lesions are proliferations of within the bone.
Schwann cells without the nerve fiber. Treatment is
surgical excision. Peripheral Ossifying Fibroma
Peripheral ossifying fibroma (POF) occurs on the gingiva
Verruciform Xanthoma and most often arises interproximally from the peri-
Verruciform xanthoma (VX) is a form of epithelial odontal ligament space. The appearance is similar to a
hyperplasia with a predilection for the oral cavity in pyogenic granuloma because the surface is typically ul-
middle-aged persons. It is an unusual lesion that is cerated and bleeds easily on probing. Microscopically,
exceedingly rare in children under 10 years. The term POFs contain small areas of dystrophic calcification,
verruciform means wart-like, and the term xanthoma including osteoid or bone; hence the term ossifying. It is
means yellow tumor or a subcutaneous collection of believed that these calcifications are deposited by
cholesterol. This is a misnomer because there is no osteoblasts associated with the periosteum of alveolar
cholesterol or fat present. The underlying connective bone. Therefore, unlike pyogenic granulomas, these
tissue contains large vacuolated cells that appear to lesions are seen only in soft tissues overlying bone. The
contain fat but are in fact large, foamy histiocytes. The removal of POFs can be problematic, because the origin
clinical appearance is most commonly a solitary, well- may be deep within the periodontal ligament space.
defined, mildly exophytic, rough, white or yellow to Eradication may leave bony or soft tissue defects that
reddish pink warty lesion less than 2 cm in diameter need tissue augmentation. These lesions have a propen-
(Fig. 2-33). It is frequently misdiagnosed clinically as sity to recur, especially if any residual cells remain fol-
a papilloma, although human papillomavirus infection lowing excision. Recurrent growth of this benign lesion
is not associated with VX. No definite etiology has may be quite rapid and worrisome.
been determined despite numerous investigations, but
it is believed that verruciform xanthoma is an unusual Peripheral Giant Cell Granuloma
reaction to mild epithelial trauma. Almost any part of Peripheral giant cell granuloma (PGCG) appears to be
the mouth can be involved, but the alveolar ridge and a response to irritation or trauma of the gingiva or
gingiva are the most common intraoral sites. Local alveolar ridge. The lesions rarely reach a size greater
surgical excision is almost always curative. than 2 cm and may be sessile or pedunculated. Clini-
cally, they resemble pyogenic granulomas because they
Injuries to the Gingiva have an ulcerated surface and tend to bleed easily on
The term epulis is a nonspecific term used for tumors probing. However, unlike pyogenic granulomas, they
and tumor-like masses of the gingiva. There are contain large numbers of giant cells of osteoclast origin
numerous types of epuli (pl.), some of which are and can occur only in soft tissues sites that cover bone.
described here. Not all contain the word epulis in their PGCGs contain areas of hemorrhage, which results in
name, and the term peripheral may be used to indicate deposits of brownish hemosiderin pigment. This com-
position gives the lesion a blue to deep purple col-
oration. On occasion, PGCGs may produce resorption
of the underlying bone, with a cupped pattern
observed radiographically. PGCGs require complete
excision and eradication of any stimulus. They rarely
recur after excision (Fig. 2-34).
Clinical Implications
Pyogenic granuloma of the gingiva, peripheral
ossifying broma, and peripheral giant cell gran-
uloma all have a similar clinical appearance in the
oral cavity. This can make them virtually impossi-
ble to tell apart. They are often referred to as The
Three Ps. A biopsy may be the only way to dier-
Figure 233 Verruciform xanthoma. Yellow wart-like entiate among these three lesions.
appearance of verruciform xanthoma of lateral tongue.
2577_Ch02_027-076 11/07/14 11:09 AM Page 52
Epulis Granulomatosum
Epulis granulomatosum is a postsurgical lesion that ap-
pears as a gingival mass extruding from a nonhealing
socket following extraction (Fig. 2-36). This loca-
tion is a pathognomonic feature for epulis granulo-
matosa. Under the microscope, they are identical to
pyogenic granulomas. Epulis granulomatosum may
be mistaken for a foreign body granuloma or a pyo-
genic granuloma. These lesions can be worrisome
and may resemble cancer metastasis or lymphoma.
Treatment is surgical excision with debridement of
the socket.
Figure 234 Peripheral giant cell granuloma. PGCG Pericoronitis
that bleeds easily on probing.
Pericoronitis refers to inflammation of the gingival tis-
sues around partially erupted teeth, most often third
Localized Spongiotic Gingival Hyperplasia molars. Erupting molars are often partially covered by
Localized spongiotic gingival hyperplasia (LSGH) is a a flap or hood of fibrotic tissue known as an opercu-
recently described condition first seen in children and lum. Bacteria and food debris become easily trapped
adolescents but also found in adults. Lesions are often under an operculum. Tissues become inflamed due to
misinterpreted clinically as pyogenic granulomas mechanical trauma from opposing dentition and/or
because of their localized exophytic and erythematous poor oral hygiene (Fig. 2-37). Pain and swelling in
appearance. The clinical presentation is a small, lo- pericoronitis can mimic more serious conditions.
calized, papillary or velvety, bright red gingival over- Treatment includes control of local infection, followed
growth. They typically are located on the facial by debridement, and eventual removal of the tooth if
gingiva overlying the tooth root. Features that distin- it is impacted.
guish them clinically from pyogenic granulomas in-
clude a subtle papillary or finely granular surface not Drug-Induced Gingival Overgrowth
seen in pyogenic granulomas (Fig. 2-35). Unlike pyo- (Drug-Induced Gingival Hyperplasia)
genic granuloma, LSGH appears unrelated to plaque There are many causes for gingival overgrowth or hy-
or calculus. It is refractory to conventional periodontal perplasia. Drug-induced gingival overgrowth is consid-
treatment and oral hygiene maintenance. Conservative ered a reactive phenomenon seen in patients taking
surgical excision is generally curative, but recurrence medications that stimulate collagen growth or prevent
is not rare. its breakdown. It is well documented that phenytoin
(Dilantin), cyclosporine, nifedipine, and other calcium
Figure 242 Ranula. Ranula (mucocele) in the floor of Figure 243 Supercial mucocele. Clear, bubble-like
the mouth. lesion in the buccal mucosa.
2577_Ch02_027-076 11/07/14 11:09 AM Page 55
major glands, partial or total removal of the affected and tongue. The name of the lesion reflects the un-
gland may be necessary. derlying necrosis of the minor salivary glands (necro-
tizing sialo-) and change in the ducts from cuboidal
Cysts of Blandin-Nuhn epithelium to stratified squamous epithelium (meta-
Cysts of Blandin-Nuhn are either mucoceles or mucous plasia). The etiology is believed to be vascular
duct cysts that form in the glands of Blandin and ischemia caused by trauma, vasoconstriction from
Nuhn. These mixed mucous and serous glands are em- dental injection, adjacent developing tumor, infec-
bedded in the anterior ventral surface of the tongue. tion, or other ischemic events.
Although the lesions are similar to mucoceles, they Necrotizing sialometaplasia most often is painless,
tend to be pedunculated rather than sessile and dome- but occasional discomfort or numbness has been
shaped (Fig. 2-44). Because of repeated trauma against reported. It initially appears as a single swelling or
the lower teeth, their surface may be red and white. cluster of smaller swellings that eventually ulcerate,
Treatment is surgical excision. leaving a crater-like lesion with rounded borders
(Fig. 2-45). Without treatment, these changes gradu-
ally return to normal in about 5 to 6 weeks.
Clinical Implications
Some mucoceles, mucous duct cysts, and ranulas
resolve spontaneously, especially in young chil- Clinical Implications
dren. Surgical excision of the mucocele along Necrotizing sialometaplasia may resemble oral
with the adjacent associated minor salivary squamous cell carcinoma. Keep in mind that al-
glands is recommended for lesions that persist. though the most common location for necrotiz-
Unfortunately, surgery runs the risk of cutting ing sialometaplasia is the hard palate, this is a
another duct, causing what appears to be a re- rare location for oral squamous cell carcinoma.
currence of the lesion, when in fact a new one
may have been created.
Sialolithiasis
Sialolithiasis refers to the formation of calcifications or
Necrotizing Sialometaplasia stones in the salivary glands (stone in Latin = lith).
Necrotizing sialometaplasia is a localized reactive lesion Salivary gland stones are often seen in salivary glands
that often causes concern because of its clinical re- affected by chronic infection and/or dehydration;
semblance to a neoplasm. Seventy-five percent of all however, in many cases the cause is unknown. Stones
cases occur in the minor glands of the posterior hard are most commonly found in the submandibular
palate, but the condition can be found in the minor glands, where they can obstruct Whartons duct
glands of the retromolar pads, labial/buccal mucosa, (Fig. 2-46). Prior to and during meals, when salivation
Sialadenosis the hard palate, but this can occur anywhere there is
Sialadenosis is nonneoplastic, noninflammatory sali- minor salivary gland tissue. The diagnosis is generally
vary gland enlargement, usually of the parotid gland. not suspected and only becomes apparent after surgical
The cause is believed to be peripheral neuropathy of excision.
the autonomic nerve supply of the salivary glands,
leading to disordered secretory activity in acinar cells. Denture and Prosthesis-Related Injury
Patients experience a nonpainful, slowly enlarging, Many patients wear prosthetic appliances to replace
firm swelling of the face. In 50 percent of cases, the missing teeth or oral tissues. They are constructed of
condition is associated with underlying systemic fac- many different biocompatible materials but some pa-
tors, including endocrine disorders such as diabetes, tients may be allergic to them. Worn or broken den-
hypothyroidism, bulimia, malnutrition, alcohol abuse, tures or partials may irritate the adjacent oral mucosa,
and drugs. Patients who take certain antihypertensive leading to injury, inflammation, and repair. Selected
or psychotropic drugs may experience sialadenosis as specific forms of denture injury are discussed.
a side effect. Symptoms regress once the underlying
cause is addressed. Denture Stomatitis
Denture stomatitis is a nonspecific term that refers to
Adenomatoid Hyperplasia a number of different conditions that present as
Adenomatoid hyperplasia is an increase in the size of generalized inflammation of denture-bearing tissues.
minor salivary glands due to an increase in the number The mucosa may show erythema, edema, or reactive
of gland acini. This creates a firm, nontender mass tissue hyperplasia. The most common cause of den-
covered by normal mucosa often mimicking a minor ture stomatitis is an ill-fitting denture that may lead
salivary gland tumor. The most common location is to inflammation of the mucosa (Fig. 2-48). Other
2577_Ch02_027-076 11/07/14 11:09 AM Page 57
causes of denture stomatitis include Candida albicans snugly into the palate, sometimes causing a cup-shaped
infection (see Chapter 3), allergy to denture acrylic deformity. Treatment is surgical excision before con-
(see Chapter 4), continuous wearing of the denture, struction of a new denture.
and poor denture hygiene. The lesions are rarely
Inflammatory Papillary Hyperplasia
symptomatic, but occasionally patients complain of
mild burning. Replacement or adjustment of the pros- Inflammatory papillary hyperplasia is a form of denture
thesis often leads to resolution of symptoms. stomatitis that most frequently occurs on the hard
palate under complete upper or partial dentures. It is
Epulis Fissuratum asymptomatic and, as the name implies, is papillary or
Epulis fissuratum is a benign condition that occurs pebbly. Papillary hyperplasia is erythematous and vel-
along the flange (edge) of an ill-fitting or loose den- vety smooth to the touch (Fig. 2-51). The condition is
ture. The most common location is the buccal or labial thought to occur as a result of mild chronic irritation.
vestibules. The overgrowth of tissue represents a pro- It seems to occur with greater frequency in individuals
tective attempt to minimize or prevent displacement who wear their dentures continuously or whose den-
of the denture into adjacent delicate tissues. It is more tures are ill-fitting. Poor oral hygiene and denture
frequent in the anterior oral cavity. Thick, hyperplastic cleanliness also have been implicated. Rarely, candidal
folds of tissue form in response to biting forces. These microorganisms have been isolated in papillary hyper-
folds surround denture flanges, sometimes resembling plasia but may be secondary to the condition. Inflam-
a wave breaking on the shore (Fig. 2-49). matory papillary hyperplasia has been observed in
dentate patients with narrow, high-vaulted palates.
Clinical Implications
Clinical Implications
The hyperplastic tissues of epulis ssuratum
Inammatory papillary hyperplasia is most often
perpetuate an unstable base for ill-tting den-
diagnosed based on clinical features. Care must
tures in the oral cavity. Lesions are surgically
be taken not to mistake it for candidiasis (see
removed prior to the fabrication of any new
Chapter 3). If it creates an unstable base for new
dental appliance.
denture fabrication, surgical removal may be in-
dicated. Good denture and oral hygiene home
care can help control this condition.
Fibroepithelial Polyp
Fibroepithelial polyp also forms because of an ill-fitting
denture. Instead of forming at the edge of the denture, Denture Ulcer
this lesion forms on the palate under the denture. Denture ulcers are caused by acute or chronic trauma
Characteristics include a flattened but pedunculated at the flange (edge) of a prosthesis that is rough or ill
mass that usually has a serrated edge (Fig. 2-50). It sits fitting. The appearance is that of a traumatic ulcer,
2577_Ch02_027-076 11/07/14 11:09 AM Page 58
Figure 250 Fibroepithelial polyp. Lesion on the palate Figure 252 Denture ulcer. Painful lesion with yellow
from an ill-fitting denture. necrotic center and red halo where denture rubs
continuously.
Peri-implantitis
Peri-implantitis is the term for inflammation around
dental implants. Inflammation may be confined to the
soft tissues but can extend down to the bone tissue sup-
porting the implant. Extension into bone may be a sign
of impending implant failure. When supporting bone
is involved, implant removal may be indicated.
Subpontine Hyperostosis
Subpontine hyperostosis is an overgrowth of bone occur-
ring on the edentulous ridge underneath the pontic of Figure 253 Subpontine hyperostosis. Well-defined
a fixed bridge. Patients initially complain that they are radiopacity under pontic; patient complains of inability to
no longer able to clean under the bridge with dental floss beneath it.
2577_Ch02_027-076 11/07/14 11:09 AM Page 59
+ = vital
+/- = equivocal-positive to some tests but not others
- = nonvital
PDL = periodontal ligament
Diagnostic tests are often used to assess the health and A drainage channel from the abscess to the epithelial
vitality of the pulp. Pulpitis is inflammation of the pulp. or mucosal surface can occur over time, often over or
A common symptom of pulpitis is increased sensitivity near the root apex. These channels are known as sinus
to heat or cold stimuli. Pulpitis may be due to bacterial tracts (Fig. 2-55A). The terms fistula and sinus tract are
invasion from the caries process or traumatic events. often used interchangeably, which is incorrect. Strictly
The fact that the pulp is encased within hard tooth defined, an abnormal channel originating deep within
structure (dentin) may contribute to symptoms of pul- tissues and extending to the epithelial or mucosal sur-
pitis commonly referred to as toothache or odontal- face is known as a sinus tract. A dental abscess that
gia. Inflammation of the pulp may be reversible or drains from the apex of a tooth through bone to the
irreversible, depending on the extent of damage. oral mucosal surface is an example. In the oral cavity,
Apical Abscess
Apical abscess is an inflammatory response to bacterial
infection and/or necrosis of the dental pulp. It is a form
of liquefactive necrosis that involves the formation of
purulent exudate (pus). Swelling, tissue edema, and Figure 254 Pulp polyp. Young patient with necrotic
pain are prominent features. pulp extruding from large carious lesion.
2577_Ch02_027-076 11/07/14 11:09 AM Page 60
once the drainage tract reaches the oral mucosa, a mass brain, where it can cause lethal thrombosis and block-
of granulation tissue referred to as a parulis may form age of cerebral vessels. This may result in cerebral in-
and be seen clinically. Strictly defined, fistulas are con- farction (stroke). Infection from the mandible can
sidered abnormal connections or pathways between travel down fascial planes to enclose the throat. This
two anatomic spaces or body cavities. An example is an can result in a life-threatening condition known as
orofacial fistula, in which infection from the oral cavity Ludwigs angina, in which breathing is severely
drains out through the skin surface (epithelium). compromised.
In some cases, dental abscess drainage may need to
be established by the clinician. This can be achieved in Radiolucent Periapical Pathology
two ways. The first is by establishing access through the Radiographically, lesions that affect the pulp and peri-
crown of the tooth. The second is by a procedure apical tissues may present with radiolucent features if
known as incision and drainage (I & D), in which a small the disease process destroys the periapical bone.
incision is made into the soft tissues to provide drainage
(Fig. 2-55B). Irrigation with normal saline will assist in Apical Granuloma (Chronic Apical
removal of purulent material. Then the patients im- Periodontitis)
mune defenses, combined with dental treatment (and/or Apical granuloma (chronic apical periodontitis) consists
antibiotics), should resolve the problem. of a mass of granulation tissue comprised of fibrob-
Untreated dental abscesses can produce severe and lasts and chronic inflammatory cells at the apex of a
life-threatening consequences. Infection can travel nonvital tooth caused by pulp necrosis. The tooth
long distances along fascial planes and cause what are may be asymptomatic and the lesion discovered on
known as space infections (cellulitis). In the maxilla, in- routine radiographs. Excessive granulation tissue and
fection can spread to the cavernous sinus of the inflammation result in bone loss at the apex, causing
a radiolucency (Fig. 2-56). Lesions are treated by
endodontic therapy that removes the source of the
inflammation, which is bacterial contamination from a
necrotic pulp. Endodontic treatment and host defenses
lead to repair of the apical bone.
B
Figure 255 Apical abscess. A. Drainage channel (sinus
tract). B. Incision performed to allow purulent material to Figure 256 Chronic apical periodontitis (granuloma).
drain. Necrotic pulp treated with root canal therapy.
2577_Ch02_027-076 11/07/14 11:09 AM Page 61
Clinical Implications
It is impossible to dierentiate between chronic
apical periodontitis and apical cyst on routine
radiographs. Biopsy of the aected tissues with
histopathologic evaluation is the only way to A
distinguish between them.
Apical Scar
Apical scars most often occur as radiolucent lesions
around the apices of teeth with a history of prior surgi-
cal endodontic treatment (root end resection/root end
fill). They are composed of dense fibrous connective tis-
sue and sometimes residual chronic inflammation. Api-
cal scars cannot be differentiated radiographically from
apical cysts or granulomas. Diagnosis of an apical scar
is made from excised apical tissues submitted for biopsy.
Internal Resorption
Resorption of dental hard tissues originating in the
pulp is known as internal resorption. This can be seen in
cases of trauma or injury but often the etiology is un-
known. Radiographically, there is enlargement of the
pulp chamber or canal, often with an irregular outline B
(Fig. 2-58). Occasionally, the crown of the tooth may
appear reddish or pink, a condition referred to as pink
tooth of Mummery. This is caused by hyperplastic,
highly vascular pulp tissue filling in areas that are re-
sorbed. In many cases, the resorption is only detected
radiographically and cannot be seen clinically. If left
untreated, perforation through the root or crown may
occur. Endodontic therapy can often stop this destruc-
tive process.
Condensing Osteitis
Condensing osteitis refers to localized areas of bone scle-
rosis or scarring. This is thought to represent a local-
ized bony reaction to a low-grade inflammatory
stimulus, such as from a diseased or necrotic pulp. The
adjacent bone shows increased density. Condensing os- Figure 260 Condensing osteitis. Well-defined
teitis appears as a uniform zone of increased radiopacity radiopacity developed following extraction of third molar.
2577_Ch02_027-076 11/07/14 11:09 AM Page 63
Ankylosis
A
Ankylosis is solid fixation of cementum of tooth roots
to alveolar bone caused by obliteration of the peri-
odontal ligament. Without the periodontal ligament
suspending the tooth in the socket, cementum fuses
to alveolar bone, preventing the tooth from further
eruption. The causes for ankylosis vary and include
inflammation or infection, abnormal bone metabo-
lism, excessive occlusal pressure, and physical trauma.
It is primarily seen in children and deciduous teeth
but may occur in adults (Fig. 2-63). Ankylosis can be
recognized clinically by failure of a tooth to fully
erupt. The occlusal surface of ankylosed teeth ap-
pears lower than adjacent teeth. Radiographically,
there is lack of identifiable periodontal ligament B
around the tooth roots. Ankylosis makes tooth Figure 262 Sequestrum. A. Radiographic appearance
extraction difficult. of sequestrum. B. Sequestrum between canine and
premolar.
Figure 261 Osteomyelitis. Painful expansion of Figure 263 Ankylosis. Mandibular first molar failed to
osteomyelitis into sinus space. erupt and lies below the occlusal table.
2577_Ch02_027-076 11/07/14 11:10 AM Page 64
CLINICAL AND RADIOGRAPHIC FEATURES females in the fourth to sixth decades of life, although
OF ORAL HARD TISSUE INJURY males are also affected. CODs most often appear in the
Bone Injury bone surrounding the roots of teeth; however, this is not
related to pulpal pathology.
Although we think of bone as hard and impervious, it
Lesions develop in three stages, each with unique ra-
is a vital living tissue that is subject to injury. The cells
diographic appearances. The first is the osteolytic stage.
in the bone (osteoblasts, osteocytes, and osteoclasts)
This is characterized by replacement of normal bone
are metabolically active and can attempt to repair in-
with loose fibrous connective tissue. Radiographically,
jury and restore the effects of disease. These processes
this presents as a well-defined radiolucency around the
produce changes that can be seen radiographically.
roots of a tooth or teeth (Fig. 2-64A). Early lesions often
mimic periapical pathology but differ in that the teeth
Cemento-osseous Dysplasia are vital. After the radiolucent fibrous tissue forms, the
Cemento-osseous dysplasia (COD) is a reactive disorder of second stage, the osteoblastic stage, begins with calcifica-
bone that exclusively affects the tooth-bearing areas of tion of the fibrous connective tissue. Small droplet cal-
the jaws. It is believed to arise from fibroblasts of the cifications form and coalesce. During this stage, the
periodontal ligament but may represent a defect in bone radiographic features become mixed radiolucent/
remodeling. The condition was once called cementoma, radiopaque with a radiolucent border (Fig. 2-64B). This
but this term is no longer used. It represents a series of process is progressive, with the oldest, most calcified
alterations in normal bone in which bone fails to mature portion in the center and the newer, more fibrous por-
properly (dysplasia). COD is more commonly seen in tion at the periphery. The final stage, the maturation
A B
Focal Any area Solitary mixed RL/RP Teeth in area vital; Biopsy to conrm; no
COD with RL border asymptomatic, treatment
no jaw swelling
Periapical Conned to mandibular Multiple discrete le- Teeth vital; asymp- Biopsy to conrm; no
COD anterior teeth sions; mixed RP/RL tomatic, no jaw treatment
with RL border swelling
Florid Two or more quadrants Diuse mixed RL/RP Teeth vital; will not To prevent osteomyelitis:
COD lesions with RL resorb in edentu- Do not biopsy! Conrm
border lous areas; mild on radiographic and
jaw swelling is rare clinical features ALONE
2577_Ch02_027-076 11/07/14 11:10 AM Page 66
B
Figure 267 Bisphosphonate-associated osteonecrosis
of the jaw (BRONJ). A. Non-healing extraction site of a
patient taking intravenous bisphosphonates for breast
cancer. B. Patient on oral bisphosphonates for Figure 269 Paget disease. Patient can no longer wear
osteoporosis, history of trauma to torus. denture, due to enlargement of the maxilla.
2577_Ch02_027-076 11/07/14 11:10 AM Page 68
Central Giant Cell Granuloma Any age, favors younger 1 quadrant, but may No underlying disease
than 30 years cross the midline
Cherubism (Chapter 6) Children age 25 Involves 24 Inherited autosomal
quadrants dominant
Brown Tumor of Adults; Renal dialysis Loss of lamina dura Elevated serum PTH
Hyperparathyroidism patients
(Chapter 9)
Renal Osteodystrophy
Renal osteodystrophy is a serious condition seen in pa-
tients with end-stage kidney disease and patients on
renal dialysis. The diseased kidneys are unable to reg-
ulate proper blood levels of calcium and phosphorus.
As a result, the blood becomes calcium deficient, in-
creasing extraction of calcium from the bones. This
makes the bones weak and brittle, leading to fractures
and joint pain. Bony changes in renal osteodystrophy
can begin many years before signs and symptoms
occur. Diagnosis is made based on kidney function and
blood tests and, in some cases, a bone biopsy.
Renal osteodystrophy may produce diffuse involve- Figure 272 Postsurgical hyperostosis. Patient
ment of the jaws. Marked jaw enlargement results in with history of periodontal surgery, now with bony
severe malocclusion. Radiographic findings include enlargement.
bone resorption with loss of cortical bone and lamina
dura. Condensation of bone trabeculae produces a Traumatic Bone Cavity (Simple Bone Cavity)
ground-glass appearance, closely resembling fibrous
Traumatic bone cavity, previously known as traumatic
dysplasia (see Chapter 6). Renal osteodystrophy can
bone cyst, is not a true cyst because it does not have an
be distinguished from fibrous dysplasia because renal
epithelial lining. The lesion is most often diagnosed
osteodystrophy is associated with kidney failure and/or
in young patients in the mandible. Clinically, the
renal dialysis. Jaw enlargement eventually may cease
lesion is asymptomatic and is discovered on routine
with treatment, but in some cases surgical intervention
radiographic examination. A unilocular radiolucent
is necessary.
area that scallops between tooth roots (Fig. 2-73A) is
typical. The etiology is unknown, but a history of
Post-surgical Hyperostosis trauma is sometimes elicited. It is hypothesized that
Post-surgical hyperostosis is an unusual condition the blood clot formed after a traumatic injury is de-
caused by stimulation of the osteoblasts in the pe- stroyed, leaving an empty cavity. The definitive diag-
riosteum during periodontal surgery. Osteoblasts nosis is made at surgery when an empty unlined
begin to form new bone, resulting in a tumor-like cavity is found (Fig. 2-73B).
growth at the surgical site (Fig. 2-72). The lesion is
composed of normal, vital bone and has the same Aneurysmal Bone Cyst
consistency as a torus. The treatment is to reduce the Aneurysmal bone cyst (ABC) is a destructive bony le-
mass without further stimulating the osteoblasts of sion caused by increased venous pressure with result-
periosteum. ant dilation and rupture of the local vascular network.
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Attrition
Attrition is wear that occurs as a result of tooth-
to-tooth contact. This includes occlusal and incisal
surfaces as well as interproximal contacts. Some
amount of attrition is considered within normal limits.
The wear usually continues throughout the life of the
teeth, so long as they make contact. However, attri-
tion can be exacerbated by grinding or clenching
habits (bruxism). Because attrition is a gradual
process, pulp exposure is rare (Fig. 2-74).
Abrasion
A
Abrasion is the loss of tooth structure as a result of an
externally applied force. The most common type is
due to tooth brushing with a hard-bristle brush and
abrasive toothpaste. Repetitive back-and-forth motion
at the cemento-enamel junction where enamel is thin
and softer cementum and dentin are more vulnerable
to wear, leads to notching and grooving that may ex-
tend deep into the dentin. Abrasion can be exacerbated
by the ingestion of abrasive components in the diet or
occupational exposures to grit or dust such as in farm-
ing. Because abrasion is a gradual process, pulp expo-
sure is rare (Fig. 2-75).
B Abfraction
Abfraction is wedge-shaped notching in the cervical
Figure 273 Traumatic bone cavity. A. Radiograph
region, unrelated to toothbrush abrasion. This appears
shows unilocular radiolucency; teeth are vital; no
expansion of jaw. B. At surgery, an empty cavity is found. to be caused by flexing pressures on the tooth, which
manifest at the crown and root interface. The defects
of abfraction are noncarious cervical lesions that result
in hard tissue breakdown. Dentin is exposed, which is
At the time of surgery, the tissue has been reported to then vulnerable to abrasion and erosion (Fig. 2-76).
resemble a blood-soaked sponge. It is not epithelial-
lined, so it does not represent a true cyst. Recent Erosion
studies suggest that it actually represents a neoplasm. Erosion is the loss of tooth hard tissue due to chemical
It generally arises in a pre-existing bone lesion, exposure. The pH at which the hard tissues begin to
such as fibrous dysplasia. Whether it is reactive or lose mineralization is around 5.0 to 5.5. Erosion may
neoplastic, it can be quite destructive because it occur as a result of ingesting low pH acidic foods, such
deforms the bone and may cause fractures. ABCs as carbonated beverages and sports drinks, or sucking
may cause pain and swelling. Radiographically, they on lemons or citric acidcontaining lozenges. Erosion
appear as large unilocular or multilocular radiolu- from ingested acidic foods is seen primarily on the fa-
cent lesions that expand the bone. If they occur in cial surfaces of the anterior teeth (Fig. 2-77) and some-
the maxilla, they may bulge into the sinus. If they times the buccal surfaces of the posterior teeth where
occur in the mandible, they may cause swelling of food contacts the enamel.
the ramus. Chronic vomiting of stomach acid is another cause
of erosion. This may occur with gastrointestinal re-
External Injuries to Teeth flux, vomiting during pregnancy, or self-induced
Injuries to tooth hard tissues may occur as a result of vomiting in patients with an eating disorder such as
normal mastication, caries, oral habits, and ingestion bulimia. Because the pH of stomach acid is 1.0, hard
of caustic agents (Table 2-13). tissue loss with regurgitation occurs quickly, often
2577_Ch02_027-076 11/07/14 11:10 AM Page 71
Figure 274 Attrition. Wear on the teeth from grinding, Figure 276 Abfraction. Notching of the cervical area
clenching, or other form of tooth-to-tooth contact (i.e., due to occlusal disharmony.
bruxism).
Figure 275 Abrasion. Wearing away of tooth due to Figure 277 Erosion. Dissolution of enamel due to
excessive tooth brushing. lemon sucking.
2577_Ch02_027-076 11/07/14 11:10 AM Page 72
External Resorption
External resorption is a condition in which root
structure adjacent to the periodontal ligament is lost.
External resorption may be initiated by chronic in-
flammation induced by trauma, infection, or reimplan- Figure 279 Idiopathic external resorption. Distal root
tation of an avulsed tooth. In some cases, the etiology of second molar.
is unknown. External resorption is occasionally seen
in patients undergoing orthodontic treatment due to ligament are stimulated to become odontoclasts.
mechanical forces applied on the teeth. Cysts and tu- Radiographically, the external surface of the tooth
mors adjacent to teeth may cause pressure that results appears moth-eaten along the portions of the root
in external resorption. External resorption is thought (Fig. 2-79). Endodontic therapy is not effective in re-
to occur when undifferentiated cells of the periodontal solving external resorption because the cells of the
dental pulp are not involved.
Fracture of a tooth can occur as a result of sudden
blow to the face, excessive occlussal forces, or biting a
hard object. The result is a sharp edge to the tooth that
may injure adjacent soft tissue. Fractures also may im-
pinge upon the dental pulp and lead to pulpitis and
pulpal necrosis.
B
Case 2-2: You take a panoramic radiograph on
Figure 278 Erosion from gastro-esophageal reux your 45-year-old female African American patient. You
disease (GERD). A. Patient with chronic reflux disorder;
amalgams appear to be protruding. B. Palatal surfaces of notice a diuse mixed radiolucent/radiopaque appear-
anterior teeth from chronic reflux disorder. ance to her entire mandible. Her jaw is normal size
2577_Ch02_027-076 11/07/14 11:10 AM Page 73
with no enlargement, and she has no dental com- velvety smooth to the touch. The patient reports no
plaints. All her teeth test vital. symptoms but says that her denture has not t well
lately. She does not like to take it out at night.
What condition does she most likely have in her
Review Questions
1. Which one of the following is NOT an indica- 8. All of the following are considered to be car-
tion of Paget disease of bone? dinal signs of inflammation EXCEPT
A. Elevated serum alkaline phosphatase A. heat.
B. Hypercementosis B. redness.
C. Onset of new spacing between teeth C. swelling.
D. All are indications of Paget disease. D. exudation.
6. Tissue damage as a result of ischemia is termed 13. A scaffold for tissue repair that is composed
A. hypertrophy. of chronic inflammatory cells, fibroblasts, col-
B. atrophy. lagen, and endothelial cells describes
C. infarction. A. granulation tissue.
D. caseous necrosis. B. granulomatous inflammation.
C. metastatic calcification.
7. Which of the following is NOT a basic type of D. hyperplasia.
inflammation?
A. Acute
B. Chronic
C. Granulomatous
D. Pyogenic
2577_Ch02_027-076 11/07/14 11:10 AM Page 75
14. Wounds in which edges cannot be closely op- 20. Which of the following medications has NOT
posed undergo healing by been associated with drug-induced gingival
A. primary intention. overgrowth?
B. secondary intention. A. Phenytoin (Dilantin)
C. tertiary intention. B. Nifedipine (Procardia)
D. scar tissue. C. Cyclosporine
D. Carbamezine (Tegretol)
15. Which of the following does NOT present as
a white patch in the mouth? 21. When a salivary gland duct is severed and
A. Frictional keratosis mucin spills into the surrounding submucosa,
B. Morsicatio buccarum this produces a swelling called a(n)
C. Hypermelanosis A. mucocele.
D. Linea alba B. mucous duct cyst.
C. necrotizing sialometaplasia.
16. If a pigmented lesion does not appear radi- D. adenomatoid hyperplasia.
ographically as iatrogenically implanted den-
tal amalgam 22. Which form of denture stomatitis appears as
A. the patient most likely has cancer. folds of tissue that look like waves crashing
B. the lesion should be removed and examined on the shore against the denture flange?
under the microscope (biopsy). A. Candidiasis
C. no further treatment is required. B. Inflammatory papillary hyperplasia
D. it should be radiographed again in 6 C. Epulis fissuratum
months. D. Fibroepithelial polyp
17. The loss of surface epithelium with exposure 23. Which of the following will NOT appear as
of the underlying connective tissue describes an apical radiolucency?
A. geographic tongue. A. Condensing osteitis
B. an ulcer. B. Periapical cyst
C. hematoma. C. Chronic apical periodontitis (granuloma)
D. external resorption. D. Apical scar
18. An exuberant growth of granulation tissue in 24. Florid osseous dysplasia is a potentially seri-
response to a minor irritant is termed ous disease because patients are at risk for
A. traumatic ulcer. A. bone cancer.
B. traumatic neuroma. B. jaw fracture.
C. pyogenic granuloma. C. a serious jaw infection if they have a tooth
D. traumatic fibroma. extracted.
D. rampant caries.
19. The term epulis means that the lesion is
A. found on the gingiva. 25. Loss of dental hard tissue due to exposure to
B. causing swelling. chemical agents is termed
C. expressing pus when pressure is applied. A. abfraction.
D. seen as radiolucency on a periapical radi- B. erosion.
ograph. C. attrition.
D. abrasion.
2577_Ch02_027-076 11/07/14 11:10 AM Page 76
C h a p t e r
3
Infectious Diseases
Learning Outcomes
At the end of this chapter, the student will be able to:
3.1. Explain the causative microorganism 3.8. Describe the pathogenesis of pri-
and route of transmission for each mary, latent, and recurrent herpes
infectious disease presented. simplex infection; recognize its
3.2. Explain the concept of indigenous stages; provide appropriate educa-
microflora and how they may either tion to a patient with herpes simplex
prevent or cause disease. infection of the lips or oral cavity.
3.3. Recognize infections associated with 3.9. Compare and contrast the etiology
skin rashes. and clinical manifestations of her-
3.4. Give examples of infectious diseases pangina and hand-foot-and-mouth
that may be harmful to a developing disease.
fetus. 3.10. Give examples of the five oral mani-
3.5. Distinguish between clinical and festations strongly associated with
pathologic features of latent versus HIV infection.
active tuberculosis. 3.11. Distinguish among the various
3.6. Outline the steps in the clinical human papillomavirus infections
progression of untreated syphilis. that can affect the oral cavity.
3.7. Recognize and explain clinical mani-
festations of various forms of oral
candidiasis and their treatment.
77
2577_Ch03_077-118 09/07/14 3:14 PM Page 78
BASIC PRINCIPLES OF INFECTIOUS agents cause diseases with oral, skin, or constitutional
DISEASES symptoms that dental hygienists must be able to recog-
Pathogenic Microorganisms nize in their patients. Constitutional symptoms, includ-
ing fever and malaise, indicate that a disease is systemic.
and Infectious Disease
Although many diseases discussed here can be consid-
Infectious diseases are caused by microorganisms too ered rare, the dental hygienist must be aware that new
small to be viewed by the unaided eye. Broad cate- immigrant populations as well as immunocompromised
gories of microorganisms include bacteria, viruses, patients may present with infectious diseases seldom
fungi, prions, protozoa, helminthes, and arthropods. seen in the general population.
Not all microorganisms are pathogenic. Maladies
caused by pathogenic microorganisms are termed in-
Opportunistic Infection
fectious diseases. Infectious diseases are responsible
for more than 20 percent of deaths globally and are a An opportunistic infection is caused by microorgan-
major health burden in underdeveloped countries, isms that usually do not produce disease in a person
particularly among young children. Despite medical with a healthy immune system. When the immune sys-
advances and an increasing knowledge of microbiol- tem is compromised in some way, and the hosts natural
ogy, antibiotic resistance and increased air travel defenses against the pathogen are reduced or elimi-
continue to facilitate the transmission of infectious nated, these microorganisms may cause disease. In
diseases. Microorganisms also evolve at a much faster other words, the microorganisms wait for an oppor-
rate than the host, requiring constant development of tunity to thrive and cause disease. Some of the op-
new antimicrobial therapies. Emergence of new in- portunities that allow for infection in an otherwise
fections (e.g., methicillin-resistant Staphylococcus au- healthy host include immunosuppressive drugs, such as
reus [MRSA]) and resurgences of old infections (e.g., corticosteroids, or drugs given to transplant patients to
tuberculosis) are killing increasing numbers in both prevent organ rejections; cancer chemotherapy; admin-
resource-poor and resource-rich countries. Addition- istration of antibiotics; damage to the skin or mucosal
ally, certain infections are now being implicated in aber- barriers; emotional or physical stress; malnutrition;
rant host reactions that may cause chronic immune- pregnancy; and some genetic diseases. Opportunistic
mediated diseases or malignancies. infections are especially common in patients with im-
The specific microorganism that causes disease is re- munosuppressive conditions such as AIDS. Once the
ferred to as the etiologic agent or infectious agent. immune system is restored and the infection is treated,
For example, the etiologic agent of tuberculosis is the the patient generally recovers; however, in some cases
bacterium Mycobacterium tuberculosis. Although some the opportunistic infection may cause death. For exam-
infectious diseases are the result of specific exogenous ple, patients with AIDS generally do not succumb to
pathogens, infections may also be caused by indige- the HIV virus but to an opportunistic infection or other
nous microflora. Indigenous microflora is present at disorder that their immune system cannot control.
birth. The external and internal exposed surfaces of the
body become colonized by organisms that establish the Carrier State
resident microflora (mostly bacteria). These microor- When a person comes into contact with an infectious
ganisms are usually protective but may become patho- agent, the healthy immune system may eliminate the
genic with a change in host environment or immunity. agent so the person may not even know they were in-
For example, colonization of the intestine normally fected. However, if the immune system cannot elimi-
prevents infection by other microorganisms due to lack nate the agent, the person will most likely become ill.
of available living space. However, systemic antibiotic Some individuals immune systems neither eliminate
therapy may inadvertently eliminate some of the pro- the agent nor become ill. In this case, the person be-
tective microflora, allowing overgrowth of other bac- comes a carrier. A carrier can transmit the micoorgan-
teria that in small numbers are of low pathogenicity. ism to someone else who becomes ill. Specific types of
The result can be antibiotic-associated diarrhea. carriers are the incubator carrier, a person who is in-
The most common infectious agents in the oral cavity fected but is not yet showing clinical signs (prodromal
are periodontal pathogens and cariogenic microorgan- period), or a convalescent carrier who has passed the
isms. These agents are not discussed in this text because clinical stage of the disease but can still transmit it.
they typically comprise a separate topic of study within The classic case of a carrier state is the legendary
the dental hygiene curriculum. Many other infectious Typhoid Mary. Typhoid Mary carried the bacterium
2577_Ch03_077-118 09/07/14 3:14 PM Page 79
Fungal hyphae
Spore germination
Fruiting
body
protective; however, wounds and burns that open the easily in crowded, indoor environments through
skin can allow entry of microorganisms (Fig. 3-4). coughing and sneezing, which release respiratory
Some microorganisms multiply in epithelial cells at the droplets containing infectious particles that may be
site of entry without further spread and become local inhaled by others. Infections transmitted by the
infections. Others spread from the site of entry into the fecal-oral route are common in young children, par-
lymph or blood and become systemic infections. ticularly in the setting of poor hygiene and insuffi-
Globally, the most common infections are trans- cient public health sanitation. Urogenital tract
mitted from one human to another through three infections spread by direct mucosal contact during
main routes: 1) respiratory/salivary, 2) fecal-oral, sexual activity are referred to as venereal or sexually
or 3) venereal (sexual). Respiratory infections spread transmitted diseases (STDs).
Disease transmission of some microorganisms from
animals to humans is possible. A disease that normally
exists in animals but that can infect humans is referred
Saliva to as a zoonotic infection. An example is Lyme dis-
Respiratory
tract
ease. A vector is an animal that transfers an infective
agent from one host to another. Arthropods, including
blood-sucking insects, ticks, and mites, are the most
common vectors and deliver diseases by piercing the
skin while they feed. Ingestion of contaminated shell-
GI tract fish or meat is another zoonotic route. Domestic pets
may also carry a number of pathogens that may be
spread by bites, scratches, or handling contaminated
Urogenital feces, such as toxoplasmosis.
tract
Pathogenicity is the ability of a microorganism to
produce pathologic changes or disease. The clinical
Skin outcome of infection is determined by a complex in-
terplay between the organism, host defenses, and the
local environment (Fig. 3-5). A given microorganism
seldom causes the same clinical picture in all individuals
who are infected by it. Some infections may be totally
asymptomatic in immunocompetent people but severe
in the immunocompromised. In any given immuno-
Figure 34 Pathways of entry. Pathways of infection; competent population, those who develop clinically ev-
potential routes of entry for microorganisms. ident disease may display a wide range in severity of
2577_Ch03_077-118 09/07/14 3:14 PM Page 81
Local environment
pH
Temperature
Figure 35 Variables aecting outcome of
Wet or dry
exposure to infectious agent. Multiple factors
Denture wearing
related to the organism, local environment, and host
Recent antibiotic use
interact to determine the outcome of infection in a
given individual.
symptoms. For example, the common cold may pro- agents. For some infections, a strong presumptive di-
duce few symptoms in some individuals but cause oth- agnosis may be made based on the clinical features
ers to be bedridden for several days. Other infections alone. Others require additional testing to confirm the
cause significant disease regardless of the immune sta- etiologic microbial agent. The diagnosis may be en-
tus of the host. hanced by: 1) isolation and culture of microorganisms,
2) identification of microbial products, or 3) detection
Clinical Signs and Symptoms of Infection of the patients antibodies specific to a pathogen.
A sign is an objective measurable assessment of dis- Microbiology laboratories examine infected speci-
ease, as opposed to a symptom, which is the subjec- mens to identify the presence or absence of microbes.
tive assessment perceived by the patient. Signs and Bacteria and fungi can be cultured, a method of mul-
symptoms of infection may be caused by the organism tiplying microbial organisms by allowing them to re-
directly (e.g., by bacteria secreting exotoxins) or by the produce in special nutrient media, usually in a Petri
host response to being infected. Infections may be lo- dish, under controlled laboratory conditions. Culture
calized, systemic, or disseminated, so there is a wide techniques depend on multiplication of the organism
variety of clinical signs of infection. Localized infec- resulting in visible colonies and may require at least
tions tend to cause pain in a specific part of the body. 24 hours for a preliminary result. Samples of the
For example, if a cut on the skin is infected with bac- colonies are examined under the microscope to visualize
teria, pain will occur at the site of the infection. The and identify specific organisms. Viruses are difficult to
lesion will show redness, swelling, heat, edema, pos- grow under routine laboratory conditions. To test for a
sibly a foul odor, and some form of exudate, such as suspected virus, material thought to contain the virus is
pus. Systemic and disseminated infections tend to placed in a medium that contains cells that the suspected
show extreme fatigue, weight loss, low-grade or spik- virus can infect. This is called a tissue culture. If the cells
ing fever, night sweats and chills, and generalized show changes, known as cytopathic effects, then a culture
body aches. Specific signs and symptoms of infections is positive. Another faster method for detecting viral in-
are discussed in this chapter. fections is serologic testing, in which the patients blood
serum is examined for the presence of antibodies pro-
Diagnostic Procedures duced against a virus (see Chapter 4). If the antibodies
Accurate diagnosis is paramount to treatment outcome. are found, the infection can be confirmed.
One of the functions of microbiological testing is to Examination of a patients tissue by cytology and
generate information to help determine the appropri- light microscopy are other techniques that may be use-
ate treatment by using the appropriate antimicrobial ful in the diagnosis of infectious diseases. Fungi and
2577_Ch03_077-118 09/07/14 3:14 PM Page 82
some bacterial organisms can be seen by special stain- use killed organisms or their fragments. The objective
ing techniques (Fig. 3-6). Due to their extremely small of immunization is to prime the host so that a quick,
size, viruses cannot be seen at the light microscopic effective immune response will be generated upon
level, but visualization of cellular changes resulting subsequent exposure to the organism. This requires
from infection, such as giant cells in herpes virus in- both B and T lymphocytes to form immunologic
fections, is possible (Fig. 3-7). Electron microscopy memory (see Chapter 4).
can detect virus particles but is rarely used in the Antimicrobial drugs are used to treat infections in
pathology laboratory. individuals who have contracted the infection. An-
timicrobials include antibiotics for bacterial infections,
Disease Control antivirals for viral infections, and antifungals for fungal
Vaccines are used whenever possible to prevent infec- infections. Appropriate antimicrobial use and selec-
tious diseases within a population. The most success- tion is important because antibiotics do not have any
ful vaccines are generally those containing live effect on viruses, antivirals do not have any effect on
attenuated microorganisms. Attenuation takes a living fungal infections, etc. For example, the common prac-
microbial agent and alters it so that it becomes harm- tice of administering antibiotics for the common cold
less but still recognizable by the body. The body then that is caused by a virus serves no useful purpose.
can make antibodies against it. Other types of vaccines Overuse or inappropriate use of antibacterials con-
tributes to the natural selection of drug-resistant bac-
terial strains. This type of antibacterial misapplication
has led to the rapid development of resistant bacteria
through either random mutation or genes acquired
from other microorganisms.
The goal of all antimicrobial therapy is selective
toxicity, which means that the drug should affect the
microbe but not the host. Antibiotics are generally
the most successful antimicrobial drugs because bac-
terial cells have many features that can be targeted
without affecting human cells. Different classes of
antibacterial drugs target different bacterial proper-
ties, such as metabolic pathways, nucleic acids, pro-
teins, or the cell wall. Antibacterial drugs may be
bactericidal (kill bacteria) or bacteriostatic (inhibit
Figure 36 Periodic acid Schi stain. Candida albicans growth of bacteria). Bacteriostatic drugs ultimately
smear taken from the oral cavity.
rely on host immunity to resolve the infection and
therefore may not be the best choice for immuno-
compromised patients.
Compared to bacteria, fungi have more cellular fea-
tures in common with humans, making selective toxi-
city difficult to achieve. Consequently, the number of
available antifungal drugs is extremely limited and
treatment can be very challenging. Most antifungals
target intracellular membranes in fungi. Drug resist-
ance is increasing and deep fungal infections are a
major cause of death and disease among immunosup-
pressed patients. The search for safer, more effective
antifungals is a high priority.
Viruses are difficult to target due to their intra-
cellular lifestyle. Antiviral drugs must enter host
Figure 37 Viral eect: herpes. A smear containing cells and selectively damage the virus but not the
keratinocytes infected with herpes simplex virus; note host. All currently available antivirals are virustatic,
enlargement of the nucleus caused by the virus. which means they target viral enzymes involved in
2577_Ch03_077-118 09/07/14 3:15 PM Page 83
nucleic acid or protein synthesis and stop viral repli- sometimes lead to noma (discussed next). Although
cation. Over the past 15 years, the number of avail- the disease has been called ANUG (acute necrotizing
able antivirals has increased dramatically, with most ulcerative gingivitis) in the past, the preceding word
of these being used in the treatment of HIV-infected acute has fallen out of favor because no chronic
individuals. form of the disease exists.
NUG is most often seen in teenagers and young to
middle-aged adults. Although the clinical distribution
ORAL MANIFESTATIONS
varies, the anterior gingiva is involved more often than
OF INFECTIOUS DISEASES
posterior sites. The interdental papillae become swollen,
The dental hygienist is expected to recognize the oral hemorrhagic, and ulcerated, imparting a blunted,
manifestations of infectious diseases in order to pre- punched out appearance (Fig. 3-8A). The gingival sur-
vent the inadvertent transmission of the disease to face is covered by a pathognomonic gray pseudomembrane
himself or herself, as well as to other patients. Some composed of necrotic debris (Fig. 3-8B), accompanied
infectious diseases do not have oral manifestations but by intense pain and foul odor. Despite severity of symp-
can be recognized by signs and symptoms that may toms, no gingival attachment loss is seen. NUG usually
present during a dental hygiene appointment. Aware- resolves quickly after removal of the offending bacteria.
ness of these diseases may help reduce the risk of trans- Gingival debridement, usually with anesthesia, is the
mission in the dental office and assist in making the most important therapeutic measure. Analgesics and
proper referral for treatment.
Bacterial Infections
Bacteria may be beneficial, harmless, or pathogenic.
They range from beneficial bacteria in the gut and
harmless flora on the skin to pathogenic foodborne,
airborne, and sexually transmitted bacteria. Some of
the most common bacterial infections with oral
manifestations are discussed here, as well as some
rare bacterial infections of interest to health-care
providers.
chlorhexidine or warm salt water rinses can be useful ad- Nonbullous impetigo is the more common form and
juncts. Systemic penicillin or metronidazole may also be typically develops in school-aged children. It is also re-
helpful. ferred to as impetigo contagiosa because it is easily spread
from one person to another. Although the legs are the
most common site of involvement, the facial skin may
Clinical Implications be affected, particularly around the mouth and nose.
Early cases of NUG may be subtle, with ulceration The lesions begin as red papules, which later form
involving only the very tips of the interdental blisters that rupture and become covered by a thick,
papillae. Aected areas are extremely tender to amber-colored crust (Fig. 3-9). These areas are non-
periodontal probing. Early recognition can pre- painful but pruritic (itchy). Regional lymphadenopathy
vent a great deal of discomfort for the patient. is common. Treatment for limited nonbullous im-
NUG may sometimes lead to necrotizing ulcera- petigo involves applying a topical antibiotic ointment
tive periodontitis or noma if the patient is im- such as mupirocin after carefully removing the over-
munocompromised. lying crusts with a warm, wet washcloth.
Bullous impetigo, sometimes called staphylococcal scalded
skin syndrome, usually occurs in infants and newborns,
Noma (Cancrum oris) most often affecting the extremities, trunk, and skin of
Noma is a destructive opportunistic infection whose the face and perioral area. S. aureus is the primary etio-
name comes from the Greek word nomein, which logic agent. Involved areas develop large bullae that be-
means to devour. The etiologic microorganisms are come purulent and then rupture to become covered by
components of the patients normal resident oral mi- a thin, brown crust. (See Chapter 4 for a description of
croflora that become pathogenic when immunity is bullae.) Systemic symptoms such as fever, malaise, and
compromised. Noma classically develops after measles diarrhea may be present. Bullous impetigo is treated
or another significant acute illness. Most experts be- with systemic antibiotics and may require hospitaliza-
lieve the disease begins as NUG and represents exten- tion to prevent dehydration and electrolyte imbalance.
sion of the infection into the surrounding oral soft
Erysipelas
tissues. Noma usually progresses rapidly. The mucous
membranes of the mouth develop ulcers, after which Erysipelas is a skin infection caused by Streptococcus bac-
rapid, painless tissue degeneration can destroy the teria affecting the deep epidermis with extension into the
bones in the face. Anatomic barriers to spread of the lymphatic system. It is seen most frequently in patients
infection are violated and involved tissue develops who have immune deficiency, diabetes, alcoholism, skin
black discoloration and yellow necrosis. Patients pres- ulceration, fungal infections, and impaired lymphatic
ent with large areas of facial and oral tissues destroyed drainage following surgery. The face is involved in
with exposure of the oral and sometimes nasal and 20 percent of cases. It may be present in dental patients
sinus cavities. Additional clinical features include mal-
odor, pain, fever, and lymphadenopathy.
Noma is extremely rare in developed countries. The
population most affected is malnourished young chil-
dren in sub-Saharan Africa, but it can occur in adults
with an underlying debilitating disease in any demo-
graphic location. The mortality rate is still significant
and survivors suffer major facial disfigurement.
Impetigo
Impetigo is a very contagious superficial skin infection
caused by Streptococcus pyogenes and/or Staphylococcus au-
reus. Most cases develop in an area of skin damage such
as a cut, scrape, or insect bite, and can spread rapidly.
Impetigo tends to be most prevalent in regions with Figure 39 Impetigo. Dermatitis associated with
warm, humid climates. Two main clinical patterns superficial infection of the skin caused by Staphylococcus
exist: nonbullous and bullous impetigo. aureus.
2577_Ch03_077-118 09/07/14 3:15 PM Page 85
Clinical Implications
Cutaneous diphtheria presents as nonhealing skin
ulcers and can occur even in vaccinated patients.
The diagnosis should be kept in mind for patients
who have recently traveled to countries where
diphtheria is endemic.
Actinomycosis
Actinomycosis is an opportunistic infection caused by
Actinomyces israelii. About two-thirds of cases are diag-
nosed in the head and neck region, a condition called
Figure 312 Red strawberry tongue. Smooth, erythematous cervicofacial actinomycosis. The Actinomyces species are
appearance of the dorsal tongue with hyperplastic fungiform found ubiquitously in tonsillar crypts, carious lesions,
papillae.
oral biofilm, and calculus. They are members of the
resident oral microflora that can cause disease upon
access to areas they would not normally colonize. The
Diphtheria organisms gain entry via an injury or break in local
Diphtheria is a potentially fatal infection caused barrier, such as through an extraction socket, soft tis-
by Corynebacterium diphtheria. The organism is sue trauma, nonvital tooth, or blow to the jaw. Unlike
transmitted through direct contact and produces most infections, actinomycosis spreads by direct ex-
a deadly exotoxin that results in a spreading tissue tension through tissues, ignoring the usual lymphatic
necrosis. Vaccination against diphtheria has been and vascular channels.
universal since the 1920s. Cases are extremely The most common location for cervicofacial actin-
uncommon in the United States and are usually omycosis is the soft tissue overlying the angle of the
seen in immunosuppressed patients or persons mandible (Fig. 3-13). The process is usually chronic,
who did not receive the full schedule of recom- slowly progressing, and nonpainful. A wooden area
mended booster vaccines. According to the CDC, of fibrosis develops initially, followed by formation
no confirmed cases of diphtheria have been of a central abscess. The suppurative phase of the in-
reported in the United States since 2003. However, fection contains characteristic yellow flecks or parti-
the potential for cases to reemerge increases cles called sulfur granules. The fibrotic nature of
as more parents decline to have their children affected areas makes it difficult for antibiotics to
vaccinated.
Among the unvaccinated, symptoms of diphthe-
ria include low-grade fever, sore throat, headache,
and vomiting. Mucosal surfaces of the nose and
throat are often affected. Intraorally, a thin yellow-
ish exudate forms over the tonsils and evolves into
an adherent necrotic membrane. Eventually, the
pseudomembrane becomes green or black in color
and may spread to the soft palate and larynx. My-
ocarditis, neuropathy, and airway obstruction are
frequent potential complications. The presence of
C. diphtheria is diagnosed by laboratory culture.
Treatment consists of antibiotics and diphtheria an-
titoxin, which is available in the United States only
through the CDC. The death rate from diphtheria Figure 313 Actinomycosis. Fibrotic or wooden phase
has improved from 50% to 5% since the develop- with erythema and swelling of the skin overlying the
ment of antitoxin. mandible.
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Tuberculosis
Clinical Implications Tuberculosis (TB) is a potentially life-threatening in-
fection caused by Mycobacterium tuberculosis. One-third
The initial site of skin trauma in cat-scratch dis-
of the worlds population is infected with TB. Although
ease is often resolved by the time lymphadenopa-
the incidence of TB in the United States continues to
thy develops. This may make clinical diagnosis
decline every year, disproportionally high rates are oc-
challenging due to a low index of suspicion for
curring among minorities and foreign-born persons.
contact with a cat. Presentation of lymphadenopa-
The organism is acquired by inhalation of airborne
thy adjacent to the mandible may be suggestive of
droplets from someone with active disease. TB infection
a dental infection. Asking children or parents
is not synonymous with active TB disease. A persons
about contact with pets and looking for evidence
initial exposure to M. tuberculosis causes a localized,
of scratches may be helpful in the absence of den-
chronic inflammatory reaction in the lungs called pri-
tal disease.
mary TB. The body creates a physical, granulomatous
2577_Ch03_077-118 09/07/14 3:15 PM Page 88
Inhale
A bacteria
TB granuloma
Inhaled
bacteria
Necrosis
Macrophages
and lymphocytes
contain bacteria
D E
Bacteria sequestered Granulomas visible
in granulomas lung on chest x-ray
If patient becomes
immunocompromised,
granulomas break down
and release bacteria
throughout the body
Figure 319 Congenital syphilis: oral manifestations. Figure 320 Leprosy. Tuberculoid form presents with
Hutchinsons incisors and mulberry molars. maculopapular and hypopigmented skin lesions. (From
Barankin, B: Derm Notes. F.A. Davis, Philadelphia, 2006,
p 195.)
in a biopsy specimen. Syphilis is treated with intra- and infected individuals are no longer contagious
muscular or intravenous penicillin. The formulation after as few as 2 weeks of treatment.
and dose schedule is tailored to the individual
based on stage, immune status, and clinical
features.
Fungal Infections
Fungi are primitive vegetable forms that include
Leprosy mold and mildew. Fungi live in air, in soil, on plants,
Leprosy (Hansens disease) is a granulomatous infec- in water, and in the human body. Only about half of
tious disease characterized by disfiguring skin le- all types of fungi and their spores are harmful. Fun-
sions, nerve damage, upper respiratory mucosal gal spores may be inhaled or acquired on the skin
lesions, and progressive debilitation. It is caused by and mucous membranes. As a result, fungal infec-
the organism Mycobacterium leprae. Leprosy is not tions often start in the lungs, on the skin, or in the
very contagious and has a long incubation period. mouth. Fungal infections are more common in pa-
The exact mode of transmission is unknown. tients with a weakened immune system due to illness
Approximately 95 percent of people are naturally im- or immunosuppressive medications or in those tak-
mune and do not contract the illness when exposed ing antibiotics that alter normal oral flora.
to the bacterium. Leprosy is common in temperate,
tropical, and subtropical climates. Approximately Candidiasis
100 cases per year are diagnosed in the United States, Candidiasis is the most common superficial fungal in-
mainly in the South, California, Hawaii, and U.S. fection seen in the oral cavity. It also has been called
island possessions. candidosis and moniliasis in the past. Formerly, it was
Leprosy has two common forms, tuberculoid and considered to be strictly an opportunistic infection,
lepromatous. Both forms produce skin lesions but but it is often detected in otherwise healthy persons.
the lepromatous form is most severe, producing Most cases of candidiasis are mild and localized, al-
large, disfiguring nodules. The skin becomes pale though it can disseminate and become life-threaten-
(Fig. 3-20) with gradually decreasing sensation to ing in an immunocompromised patient. There are
touch, heat, and pain. Repeated injury that cannot more than 40 known species of Candida. The most
be felt may lead to loss of hands or feet. In the lep- common causative fungal organism, Candida albicans,
romatous form of the disease, large disfiguring nod- is a part of the indigenous oral microflora in about
ules develop. In advanced stages, destruction of the half of the general population. In addition to C. albi-
nasal bridge is characteristic, resulting in a saddle- cans, species known to cause infection in the oral cav-
nose deformity. Antibiotics are used to treat leprosy ity include C. krusei, C. glabrata, C. tropicalis, and C.
2577_Ch03_077-118 09/07/14 3:15 PM Page 92
Pseudomembranous Candidiasis
Pseudomembranous candidiasis is characterized by
adherent, white cottage cheese-like plaques that Figure 321 Pseudomembranous candidiasis. Multiple
consist of C. albicans organisms, superficial epithelial white plaques on the buccal mucosa.
cells, and debris (Fig. 3-21). The plaques can be wiped
off with dry gauze. Underlying mucosa may be ery-
thematous but is in tact. This clinical presentation is
often referred to as thrush. The most common pre-
cipitating factors for developing thrush are use of
broad-spectrum antibiotic therapy or immunosup-
pression either from underlying disease or therapeutic
immunosuppression with corticosteroids or other im-
munomodulary medications. Use of steroid asthma
inhalers is an increasingly common cause of oral can-
didiasis localized to the soft palate where the medication
initially contacts the tissues (see Figs. 3-22 and 3-23).
Infants whose immune systems have not fully devel-
oped are also susceptible. The lesions are commonly
distributed on the buccal mucosa, palate, and dorsal Figure 322 Pseudomembranous candidiasis. Patient
tongue. Symptoms vary: patients may be completely with asthma developed pseudomembranous candidiasis of
asymptomatic, complain of an unpleasant taste, or ex- the uvula and tonsillar pillars after using corticosteroid-
perience oral burning. containing inhaler.
Clinical Implications
Coated tongue represents an increase in the
thickness of keratin on the dorsal tongue that is
often mistakenly attributed to candidal infection.
Coated tongue is common among smokers and
individuals with a soft diet, suggesting an etiol-
ogy related to either increased production or de-
creased desquamation of keratin. Patients who
have coated tongue but do not complain of oral
burning or other symptoms most likely do not
have candidiasis.
A
Figure 324 Acute atrophic candidiasis. Generalized
erythema and atrophy of the dorsal tongue papillae.
B
Clinical Implications
Figure 325 Central papillary atrophy. A and B.
A patient with denture stomatitis who complains Smooth, bald appearance localized to the mid-dorsal
of pain and tenderness should be tested for oral tongue.
candidiasis. Table 3-1 describes how to perform a
cytologic smear.
or an inadequate vertical dimension of prosthesis are
particularly prone to angular cheilitis due to the skin
Angular Cheilitis creases that develop near the corners of the mouth.
Angular cheilitis is a term used to describe redness and Saliva wicks out into these folds, creating a constant,
cracking at the corners of the mouth (Fig. 3-28). Con- moist environment that predisposes the patient to can-
trary to popular belief, this problem is not related to didal infection. More extensive involvement of the lip
vitamin deficiency, but caused by infection with C. al- vermillion and perioral skin called cheilocandidiasis or
bicans and/or S. aureus. Patients with reduced vertical candidal cheilitis can occur and is usually associated with
dimension of occlusion due to loss of posterior teeth thumb sucking or habitual lip licking (Fig. 3-29).
2577_Ch03_077-118 09/07/14 3:15 PM Page 94
2. Write the patients name in PENCIL (not pen) on the frosted end of the glass slide.
2577_Ch03_077-118 09/07/14 3:15 PM Page 95
3. If the patient has a very dry mouth, have them rinse gently with a few drops of
water before the specimen is collected.
4. Using a damp (not soaked) wooden tongue blade, rmly wipe the area. Use
enough strokes to collect a visible accumulation of oral uids.
5.Transfer the uids to a clean, dry glass slide. Smear the sample across the slide to
make a thin coat.
6. Hold the glass slide up to the light. If you can see the sample on the slide, you
have enough. Do not overload the slide. Repeat in another area, if necessary, to
obtain an adequate sample.
7. Spray Cytox or alcohol-based hair spray on the slide from a distance of about
1 foot. DO NOT spray too closely to prevent rinsing the cells o the slide. Do not
overspray; one or two swipes should be enough.
8. Allow the slide to dry for a few minutes and place the slide in the slide container.
Continued
2577_Ch03_077-118 09/07/14 3:15 PM Page 96
EXPR
ESS
PRIO
RITY
A
Figure 328 Angular cheilitis. Erythema, cracking, and
subtle white plaques at the corners of the mouth in a
patient with reduced vertical dimension of occlusion.
Aspergillosis
Aspergillosis is a fungal infection that primarily affects the
respiratory tract. Aspergillus fumigatus is extremely com-
mon in the environment and is responsible for most
cases. Aspergillus can be found in soil, dust, plants, and
building materials and is an occupational hazard for con-
struction workers in the setting of building construction,
which can stir up Aspergillus spores from the soil.
Aspergillosis occurs in noninvasive and invasive forms.
Noninvasive aspergillosis is a superficial disease that affects
the respiratory tracts of otherwise healthy patients. It may
present as either allergic fungal sinusitis or a tangled mass
of organisms in the sinus called an aspergilloma. Healthy
Figure 335 Histoplasmosis. Numerous Histoplasma patients with noninvasive aspergillosis are treated with
capsulatum spores in an oral biopsy of an HIV-positive surgical debridement. Invasive aspergillosis is a common
patient are highlighted by Grocott-Gomori methenamine opportunistic infection and is usually limited to immuno-
silver stain.
suppressed patients. Most patients with invasive as-
pergillosis have pulmonary involvement. Patients often
acquire aspergillosis as a nosocomial infection while in
the hospital. Aggressive surgical debridement and sys-
temic antifungal therapy is indicated for invasive as-
pergillosis, which is uniformly fatal if untreated.
Clinical Implications
Extraction or endodontic treatment of a maxil-
lary molar tooth may cause minor damage to the
maxillary sinus lining and predispose a patient to
developing oral aspergillosis. The gingival sulcus
has been suggested as the most likely route of
entry. Clinical features include painful gingival
ulceration surrounded by swollen, red-purple
Figure 336 Mucormycosis. Large, branching Mucor mucosa which becomes necrotic if untreated.
hyphae tend to occlude small blood vessels.
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Cryptococcosis
Cryptococcosis is a fungal infection caused by Cryptococcus
neoformans, an encapsulated yeast that can live in both
plants and animals. Disease occurs primarily in im-
munosuppressed patients. The organism usually affects
the CNS, causing meningitis in the majority of cases.
Cryptococcosis is a major cause of death in AIDS pa-
tients who are not receiving appropriate treatment.
Protozoal Infections
Protozoa are one-celled organisms that move about
by means of appendages known as cilia or flagella.
Protozoal infections are most common in rural areas
of underdeveloped countries in Africa, Asia, and
South America. They are relatively rare in the
United States and other industrialized nations, but
do occur among Western travelers to developing
countries. One protozoal infection seen in the
United States is toxoplasmosis, caused by Toxoplasma
gondii. The organisms are present in cat feces and Cat eats infected prey and
may be ingested by other animals or accidentally by passes protozoa to litter box
humans who have close contact with infected cats. Figure 337 Toxoplasmosis. A. Person with
Lymphadenopathy of the buccal or submental nodes compromised immune system develops toxoplasmosis seen
in cervical lymph nodes. B. Person with normal immune
in the head and neck with an unknown etiology may system with no signs of toxoplasmosis. C. Pregnant woman
be toxoplasmosis in a susceptible individual. Toxo- passes disease to child who is born with congenital
plasmosis rarely causes problems in healthy patients, toxoplasmosis.
2577_Ch03_077-118 09/07/14 3:15 PM Page 100
HHV-1 Herpes simplex virus-1 Oral and/or genital herpes (predom- Close contact, including sexual
(HSV-1) inantly orofacial) contact
HHV-2 Herpes simplex virus-2 Oral and/or genital herpes (predom- Close contact, primarily sexual
(HSV-2) inantly genital) contact
HHV-3 Varicella zoster virus (VZV) Chickenpox and shingles Respiratory and close contact,
including sexual contact
HHV-4 Epstein-Barr virus (EBV) Infectious mononucleosis, Burkitts Close contact, transfusions,
lymphoma, CNS lymphoma in tissue transplant, and
AIDS patients, post-transplant congenital
lymphoproliferative syndrome
(PTLD), nasopharyngeal carci-
noma, HIV-associated hairy
leukoplakia
HHV-5 Cytomegalovirus (CMV) Infectious mononucleosis-like Saliva
syndrome
HHV-6 Roseolovirus, herpes Roseola infantum Respiratory and close contact
lymphotropic virus
HHV-7 Roseolovirus Roseola infantum Respiratory and close contact
HHV-8 Kaposis sarcoma- Kaposis sarcoma Close contact, including sexual
associated herpes virus contact
(KSHV)
2577_Ch03_077-118 09/07/14 3:15 PM Page 101
A
Figure 340 Herpes labialis. Widespread lesion of
the lips.
B
Clinical Implications
Mechanical rupture of vesicles should be avoided,
as this can cause spread of the virus to other
areas. Accordingly, dental treatment should be
postponed while patients are in the vesicle stage.
Clinical Implications
If dental treatment is a known trigger for a patient,
then prophylactic antiviral therapy should be con-
sidered before dental appointments. Antiviral ther-
apy given after the prodrome is not eective.
C
Figure 341 Herpes labialis. A. Coalesced vesicles.
Recurrent Intraoral Herpes (Herpes Stomatitis) B. Same lesion 6 days later with ruptured vesicles and
ulceration. C. Crust forms over the ulcer.
Recurrent intraoral herpes or herpes stomatitis is a less
common form of HSV-1. Lesions are limited to the
keratinized attached mucosa of the gingiva or hard medications that may be applied intraorally. Systemic
palate. Like herpes labialis, herpes stomatitis presents medications administered after the outbreak begins are
as a cluster of tiny vesicles that coalesce and then rup- not effective.
ture to leave erosions or ulcerations rather than a crust
(Fig. 3-43A, B, and C). Symptoms are usually less Herpetic Whitlow
severe than herpes labialis; however, some outbreaks Herpetic whitlow is a painful herpes infection of the nail
cause burning and pain, limiting the patients ability beds and fingers (Fig. 3-44). This was an occupational
to eat. Treatment is limited because there are few hazard among dental care providers before the advent
2577_Ch03_077-118 09/07/14 3:15 PM Page 103
Herpetic Conjunctivitis
Herpetic conjunctivitis can also be a risk for dental care
providers who do not wear eye protection. The virus
may be carried to the eye when aerosols are created
from a patient with an active HSV-1 lesion. Before the
routine use of protective eyewear, this condition was
common among dental personnel. Central nervous
system involvement may also occur, where the virus
causes life-threatening herpes encephalitis, predomi-
nantly in the elderly and immunocompromised.
Clinical Implications
Aphthous ulcers are often mistakenly diagnosed
as a herpetic infection. Lesions of recurrent in-
traoral herpes are seen only on the keratinized
attached tissues, whereas aphthous ulcers are
usually limited to nonkeratinized moveable mu-
cosa. Aphthous ulcers can be distinguished from
ulcers of HSV-1 infection by their lack of a pro-
drome, absence of vesicles, and by their loca-
tion. See Table 4-3 in Chapter 4 for comparison
of recurrent aphthous ulcers and herpes simplex Figure 343 Herpes: intraoral. A. Cluster of vesicles on
infection. keratinized tissue of palatal gingiva. B. Vesicles coalesce
and begin to rupture. C. Ulcers; intraoral lesions do not
form a crust.
Varicella-Zoster Virus
Varicella-zoster virus (VZV or HHV-3) is spread approved for use in the United States. Despite the
through either direct contact or respiratory droplets. vaccine, cases are still seen due to a national vaccina-
VZV is highly contagious and has a 10- to 20-day in- tion rate of only 85 percent.
cubation period. Primary infection with VZV is usu- Varicella usually affects school-age children and is
ally symptomatic and causes varicella, or chickenpox. characterized by rhinitis, or runny nose, sore throat,
The incidence of varicella has decreased dramatically fever, and a pruritic skin rash often appearing in several
since the mid-1990s when an effective vaccine was crops. Skin lesions start as clear vesicles with a red base,
2577_Ch03_077-118 09/07/14 3:15 PM Page 104
Clinical Implications
Figure 344 Herpetic whitlow. Herpes vesicles and
If the ophthalmic division of the trigeminal nerve
ulcers on finger of dental health-care worker causing pain
and scarring. is involved, the patient should be referred to an
ophthalmologist immediately. Lesions present
on the tip of the nose are an indication of poten-
resembling a dewdrop on a rose petal (Fig. 3-45). tial ocular infection that can lead to blindness if
These may progress to form pustules before crusting left untreated.
over. The rash develops first on the trunk and face and
then spreads to the extremities. Oral involvement is
common, especially on the lips and palate. Lesions are
present for about 4 days. A person is considered conta-
gious until all vesicles have crusted. Uncomplicated
varicella in healthy children is usually treated sympto-
matically with non-aspirin analgesics and diphenhy-
dramine (Benadryl). Varicella in adults tends to be
much more severe and is associated with serious poten-
tial complications, such as varicella pneumonitis and
encephalitis. Use of antiviral medications in adults may
be helpful if started early.
VZV establishes latency in the dorsal root ganglia
and may be reactivated later in life as herpes zoster, also
called shingles. About 30 percent of the population suf-
fers from shingles. Reactivation usually occurs only once Figure 346 Herpes zoster. Neck of an adult.
and the risk increases with the increasing age of the pa-
tient. A prodrome of unilateral, intense pain typically
precedes the clinical lesions and develops within the as-
sociated dermatome, which is an area of skin innervated
Figure 348 Herpes zoster. Lesions on face of the same Figure 349 Infectious mononucleosis. Palatal petechiae
patient, confined to V2. in a young person with infectious mononucleosis.
The acute phase of herpes zoster resolves within blood studies. Most cases resolve within 4 to 6 weeks
2 to 3 weeks; however, 15 percent of the population may and supportive care with nonaspirin analgesics is rec-
experience continued pain known as postherpetic neural- ommended to manage symptoms.
gia, which is the chronic phase. Systemic antiviral med-
ications given during the acute phase may decrease the Oral Hairy Leukoplakia
likelihood of developing postherpetic neuralgia. Half of Oral hairy leukoplakia (OHL) is associated with the
patients older than 60 years who develop zoster will Epstein-Barr virus (EBV) mainly in people with
progress to the chronic phase. Most cases of posther- HIV, both immunocompromised and immunocom-
petic neuralgia resolve within a year. Topical capsaicin petent. Among HIV-positive patients, the prevalence
provides relief for most patients. A vaccine called of OHL is higher in smokers and among homosexual
Zostavax is now available for people older than age 60 males. It can affect patients who are HIV negative
who had chickenpox as a child to help reduce the risk but are otherwise immunosuppressed. Cases are seen
of zoster reactivation. in those with acute lymphocytic leukemia, as well as
heart, kidney, and bone marrow transplants.
Epstein-Barr Virus OHL is often asymptomatic, with most patients
Epstein-Barr virus (EBV or HHV-4) is a member of the unaware of its presence. Some patients experience
herpes virus family and one of the most common symptoms including mild pain, dysesthesia, alter-
human viruses. As a herpes virus family member, it es- ation of taste, and the psychological impact of its cos-
tablishes a lifelong, persistent infection. As many as metic appearance. OHL may appear and regress
95 percent of adults between 35 and 40 years of age spontaneously. Unilateral or bilateral white lesions
have been infected. are classically seen on the lateral tongue (Fig. 3-50),
but may also occur on the dorsal or ventral surfaces
Mononucleosis or on buccal mucosa. The lesions may vary in appear-
Mononucleosis is caused by the Epstein-Barr virus ance from smooth, flat lesions to irregular and finely
(EBV). EBV is spread by direct contact with infected folded areas. There is no associated erythema or
saliva, which has resulted in the common nickname edema of the surrounding tissue. Unlike lesions
kissing disease. Young adults are prone to sympto- caused by tongue chewing, also called morsicatio lin-
matic primary infection characterized by a prodrome guarum, that show horizontal folds of tissue, OHL
of prominent fatigue, followed by fever, sore throat, often has vertical folds, thus distinguishing it from
and lymphadenopathy. Hepatosplenomegaly may also morsicatio linguarum.
be seen in some cases. Oral clinical features include As a benign lesion with low morbidity, OHL does
swollen tonsils with a yellowish exudate. Palatal pe- not require specific treatment. Institution of highly ac-
techiae (Fig. 3-49) and NUG are also possible. The di- tive antiretroviral therapy (anti-HIV or HAART) in
agnosis of mononucleosis should be confirmed by HIV and AIDS is useful in eliminating OHL.
2577_Ch03_077-118 09/07/14 3:15 PM Page 106
Enteroviruses
Enteroviruses enter the body through the gastroin-
testinal tract. They represent a diverse group of RNA
viruses that include echoviruses, coxsackievirus, polio
viruses, and others. Infections can range from asymp-
tomatic to life-threatening. Transmission occurs
mainly through the fecal-oral route by ingestion, but
saliva and respiratory droplets can be infectious during
acute illness. Most symptomatic enterovirus infections
are seen in young children and tend to occur in epi-
demics. Enteroviruses are extremely common and
everyone is at risk. Individuals may become infected
multiple times by different enterovirus strains. Most
Figure 350 Oral hairy leukoplakia. Leukoplakia with clinically evident infections are self-limiting, but the
vertical white striations along the lateral border of the
tongue of an HIV-positive adult. severity depends on the particular strain of virus.
Herpangina
Herpangina is usually caused by a coxsackievirus and is
EBV-Related Cancer characterized by fever, sore throat, and oral lesions that
EBV is also considered to be an oncogenic virus. It is develop on the soft palate, uvula, and tonsillar pillars.
associated with Burkitts lymphoma and nasopharyn- About 2 to 12 lesions are usually present and begin as
geal carcinoma, which are discussed in Chapter 7. small vesicles on nonkeratinized tissue that rupture to
form shallow ulcers. Symptoms are fairly mild and le-
Cytomegalovirus sions resolve within 1 week.
Cytomegalovirus (CMV or HHV-5) is a ubiquitous
herpesvirus that typically causes symptomatic disease
only in newborns and immunocompromised pa- Clinical Implications
tients. Neonates may acquire it through the placenta, Herpangina may be confused with primary HSV-1
during delivery, or through breastfeeding. According infection. However, herpangina does not occur
to the CDC, about 1 out of every 150 children in the on keratinized tissue, nor is the gingiva painful,
United States is born with CMV infection and red, or swollen.
80 percent are asymptomatic. Symptomatic disease
may be present at birth or develop around age 2.
CMV infection during childhood manifests as Hand-Foot-and-Mouth Disease
hearing and vision loss or mental disability. Devel- Hand-foot-and-mouth disease is a common pediatric
opmental tooth defects, typically enamel hypoplasia, infection most often caused by coxsackievirus A16. Oral
are seen. involvement precedes the skin rash and is accompanied
Most individuals will have contracted CMV by by flu-like symptoms. Oral lesions are similar in ap-
sometime in adulthood. The majority of primary pearance to that of herpangina but are more numerous
CMV infections are asymptomatic in normal, and can affect almost any intraoral site (Fig. 3-51). Skin
healthy patients. The virus may remain latent in sali- lesions that classically develop on the hands and feet
vary ductal cells, endothelium, or chronic inflamma- begin as red macules that form tiny vesicles which heal
tory cells. CMV is the most common opportunistic without rupturing. The disease resolves within a week
viral infection among AIDS patients. CMV co-infec- and symptomatic relief is the only treatment indicated.
tion is identified in 90 percent of MSM who are
HIV-infected. Infection often manifests as CMV co- Rubeola (Measles)
litis, a bloody diarrhea; or CMV chorioretinitis, in- Measles is a disease caused by an extremely contagious
flammation of the retina that can lead to blindness. paramyxovirus transmitted through respiratory droplets.
Chronic oral ulcerations are seen frequently in AIDS Widespread use of the measles, mumps, and rubella
patients and may be caused by co-infection with (MMR) vaccine has led to more than a 99 percent re-
CMV and HSV. duction in the prevalence of measles in the United States
2577_Ch03_077-118 09/07/14 3:15 PM Page 107
Parotid gland
Accessory
parotid gland
Parotid duct Swollen
neck and
glands
Figure 353 Mumps. Involvement of the parotid glands is typical, causing pain and swelling of
the soft tissues of the posterior cheeks and neck.
2577_Ch03_077-118 09/07/14 3:15 PM Page 109
status. Men having sex with men (MSM) is the single clotrimazole troches are the ideal choice if the pa-
biggest risk factor for HIV infection and accounts for tient is receiving antiretroviral treatment and has
about half of all new infections. a CD4 count of at least 50. Systemic fluconazole
is recommended for patients with a CD4 count
under 50.
Clinical Implications Oral hairy leukoplakia. Oral hairy leukoplakia is
Although occupational transmission to dental an EBV infection that causes adherent white
providers is rare, oral uids can be infectious. plaques that characteristically develop on the lateral
The risk of HIV seroconversion after percuta- tongue of HIV-infected patients. The clinical ap-
neous skin puncture exposure with HIV-infected pearance may range from subtle white streaks to a
blood is 0.3 percent. If exposure does occur, a shaggy keratotic plaque. The presence of oral hairy
persons risk of infection can be further reduced leukoplakia is often a sign of disease progression
by prompt initiation of post-exposure prophy- and should prompt referral for re-evaluation in an
laxis with antiviral medications. HIV-positive patient.
Periodontal diseases. Three unusual clinical patterns
of periodontal disease are seen in HIV-infected pa-
A few weeks after initial exposure to HIV, more tients: linear gingival erythema, necrotizing ulcerative
than half of patients develop the acute viral syndrome. gingivitis, and necrotizing ulcerative periodontitis.
The clinical features mimic mononucleosis and may Linear gingival erythema is limited to the free gingival
include lymphadenopathy, fever, headache, sore margin and may represent an atypical presentation of
throat, and malaise. The acute viral syndrome resolves oral candidiasis (Fig. 3-55). Because the clinical pres-
after a few weeks, but some patients continue to have entation is similar to marginal gingivitis, the diagnosis
persistent generalized lymphadenopathy (PGL). PGL is should only be made for erythema that is resistant to
usually the only potential sign of HIV infection during improved oral hygiene. The presentation of NUG in
the asymptomatic phase of HIV, which follows the HIV-infected patients is similar to the appearance
acute viral syndrome. People who are HIV-infected seen in immunocompetent patients. Necrotizing ulcer-
may appear and feel healthy for many years. ative periodontitis (NUP) is gingival necrosis accompa-
Acquired immunodeficiency syndrome (AIDS) is diag- nied by rapid, severe loss of supporting alveolar bone
nosed when an HIV-infected persons CD4 count (Fig. 3-56). Focal defects are more likely to be seen
drops below 200 cells per microliter. The clinical pres- than diffuse involvement. Treatment of NUG and
entation is variable but often includes weight loss, per- NUP consists of irrigation and debridement of all
sistent diarrhea, and opportunistic infections. Many necrotic tissue, followed by long-term use of prophy-
infections that are asymptomatic or mild in immuno- lactic chlorhexidine rinse.
competent patients (such as HSV and CMV) can be
devastating for AIDS patients. Pneumonia caused by
Pneumocystis jirovecii (formerly P. carinii) is highly sug-
gestive of AIDS and leads to the initial diagnosis in a
significant percentage of cases. The following oral
manifestations are considered to be strongly associated
with HIV infection and will be discussed further.
Candidiasis. Candidiasis is the most frequent oral
sign of HIV infection. The two most common
clinical patterns seen are pseudomembranous and
erythematous candidiasis (discussed earlier in this
chapter). Pseudomembranous candidiasis may be
extensive and is more likely to develop when the
patients CD4 count drops below 200. Treatment
of candidiasis in AIDS patients can be challenging.
Topical treatments are associated with a high re- Figure 355 Linear gingival erythema. Prominent
currence rate and systemic antifungals may inter- erythema at the gingival margin in an HIV-positive
act with other medications. As a general guideline, patient.
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Figure 357 Kaposis sarcoma. Discovered on routine Figure 358 HIV sialadenitis. Firm, nontender bilateral
dental examination. swelling of the parotid glands in an HIV-positive patient.
2577_Ch03_077-118 09/07/14 3:16 PM Page 111
HAART is very expensive and therefore not accessible Skin and intraoral verruca vulgaris lesions may be
for most HIV-infected patients in resource-poor coun- treated by conservative surgical excision or curettage
tries. Additionally, it causes significant side effects and down to the base of the lesion. Skin lesions are
may not be effective in all patients. amenable to liquid nitrogen cryotherapy and topical
application of salicylic acid or lactic acid. For obvious
Human Papillomavirus reasons, these agents should not be used in the mouth.
There are more than 40 types of human papillomavirus Recurrence is seen in a small proportion of treated
(HPV) that may affect the genitals, oral cavity, and cases. Most will disappear spontaneously within 2 years.
pharynx. Other HPV types cause infections of the skin. Lesions do not transform into cancer.
HPV infection is usually asymptomatic, and the virus
is therefore unknowingly transmitted. Most HPV in- Condyloma acuminatum
fections are cleared by the bodys immune system Condyloma acuminatum is considered to be a sexually
within a period of about 2 years, but some may persist transmitted disease (STD) most commonly found on the
and cause a wide spectrum of clinically evident disease. genitalia. However, the lesions can also occur in the
Table 3-3 summarizes the clinical manifestations of the oral cavity at sites of sexual contact. They are caused
most common intraoral HPV lesions. by HPV-6 and HPV-11, but are often mixed with
other HPV types. Nevertheless, they are classified as
Squamous Papilloma low-risk HPV types that are highly transmissible. The
Squamous papilloma is a benign mucosal mass pro- clinical appearance is a pink, papillary, exophytic mass
duced by HPV-6 and HPV-11. These types also cause that may be indistinguishable from squamous papil-
skin warts but are not among those associated with ma- loma in the early stages (Fig. 3-63A), but some lesions
lignancy or precancer. Squamous papilloma has an ex- enlarge and become confluent with other lesions over
tremely low virulence and infectivity rate and is not time, covering a large surface area (Fig. 3-63B).
considered contagious. Lesions are painless and may Condyloma acuminatum can occur in healthy pa-
develop on any mucosal surface, with the tongue and tients, but may also be one of many potential oral man-
soft palate affected frequently. Squamous papillomas ifestations of HIV infection (Fig. 3-64). Treatment for
occur singly and often are pedunculated. They are usu- genital lesions includes topical application of caustic
ally pink or white and may have long finger-like to medication that burns the lesions off. Oral lesions
cauliflower-like projections or short raspberry-like may be treated with surgical excision, electrocauteri-
projections (Figs. 3-59 and 3-60). zation, or laser therapy. Care should be exercised when
Conservative surgical excision including the base of using electrocauterization and laser therapy on these
the lesion is adequate treatment and recurrence is un- highly transmissible lesions, due to the potential for
likely. Lesions may go untreated for years with no aerosolization of viral particles.
change or spread to other parts of the body. There are
no reports of transformation into malignancy. Focal Epithelial Hyperplasia (Hecks Disease)
Hecks disease (focal epithelial hyperplasia) is caused by
Verruca Vulgaris HPV-13 and HPV-32, which are among the low-risk
Verruca vulgaris (vulgaris = common) is an infection of HPVs. Lesions are characterized by pinkish papules
the skin commonly known as a wart (Fig. 3-61), most that occur diffusely on the mucous membrane of the
often caused by the subtypes HPV-2, HPV-4, and lips, tongue, or less commonly the buccal mucosa,
HPV-40. The skin of the hands is the most common floor of the mouth, and palate. They are soft, pain-
site. Verruca vulgaris is contagious and capable of less, sessile papules about 1 to 4 mm in diameter that
spreading to other parts of an affected persons skin occur in clusters (Fig. 3-65) and can be present on
or mucous membranes through autoinoculation. Ver- more than one intraoral site at a time. Individual le-
ruca vulgaris of the oral mucosa is typically seen on sions are broad based and may appear as a smooth-
the vermilion border, labial mucosa, or anterior surfaced plaque rather than a verrucous or papillary
tongue (Fig. 3-62). The appearance of the lesion may lesion (Fig. 3-66).
be identical to a squamous papilloma, but it is often The condition usually occurs in children and
characterized by white pointed or verruciform surface young adults and may have familial predilection. Le-
projections and a broad base. They seldom are more sions may last for several months, sometimes years,
than 5 mm in diameter. before running their course. The patient may elect to
Table 3.3 Classification of Intraoral Human Papilloma Virus Lesions
Oropharyngeal and Tonsillar
2577_Ch03_077-118 09/07/14 3:16 PM Page 112
HPV 6, 11 HPV 2, 4, 6, 7, 40 HPV 13, 32 HPV 2, 6, 11, 53, 54 HPV 16, 18, 31, 33
1 Growing on a stalk.
2 The base is the same size as or larger than the lesion itself.
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Figure 359 Papilloma. Pedunculated mass with Figure 362 Verruca vulgaris. Sessile lesion with
prominent cauliflower-like appearance. prominent finger-like projections.
B
Figure 361 Verruca vulgaris. Single sessile Figure 363 Condyloma. A. Small early papilloma-like
exophytic rough-surfaced lesion of the lower lip of condyloma on the ventral tongue. B. Condyloma of
a child. commissure and buccal mucosa.
2577_Ch03_077-118 09/07/14 3:16 PM Page 114
manage these problems. Your clinical exam reveals clinical exam is notable for swollen tonsils covered
that the patient wears a maxillary removable partial by a yellow-white exudate.
denture. The mucosa underlying the denture is in-
Review Questions
1. Infections caused by microorganisms that are 3. Which of these is NOT one of the three main
not normally pathogenic in persons with a routes of human disease transmission?
healthy immune system are known as A. Sexual
A. attenuated. B. Fecal-oral
B. pathogens. C. Respiratory
C. opportunistic. D. Animal vector
D. compromised.
4. Serological testing performed to diagnose a
2. A type of microorganism that can only cause viral infection most often involves the detec-
disease after entering and replicating in a host tion of
cell is A. antibodies.
A. bacteria. B. toxins.
B. virus. C. genetic material (DNA or RNA).
C. fungi. D. components of the cell wall.
D. protozoa.
2577_Ch03_077-118 09/07/14 3:16 PM Page 116
5. A disease control agent that uses live or killed 11. The most common fungal infection seen in
microorganisms or their components to stim- the oral cavity is
ulate the immune system and create memory A. histoplasmosis.
is known as a(n) B. candidiasis.
A. antibiotic. C. actinomycosis.
B. antiseptic. D. blastomycosis.
C. vaccine.
D. antibody. 12. Central papillary atrophy and angular cheilitis
are both manifestations of
6. Necrotizing ulcerative gingivitis is A. syphilis.
A. usually asymptomatic. B. herpes simplex virus.
B. associated with an intense malodor. C. candidiasis.
C. treated with systemic antiviral medication. D. human papillomavirus.
D. most often found in elderly patients.
13. Which of these therapies is NOT an antifun-
7. Which of the following bacterial infections gal agent?
may be reactivated later in life if a person be- A. Nystatin
comes immunocompromised or debilitated? B. Penicillin
A. Cat-scratch disease C. Amphotericin B
B. Tuberculosis D. Fluconazole
C. Scarlet fever
D. Actinomycosis 14. A fungal infection that may be acquired by an
immunocompromised patient during a hospi-
8. Which of the following diseases is NOT tal stay, particularly in the setting of building
caused by a streptococcal infection? renovation, is
A. Impetigo A. aspergillosis.
B. Scarlet fever B. histoplasmosis.
C. Erysipelas C. actinomycosis.
D. Diphtheria D. mucormycosis.
9. Strawberry tongue and a skin rash resem- 15. Among healthy patients, most primary herpes
bling a sunburn with goose bumps is char- simplex virus infections are
acteristic of A. acute herpetic gingivostomatitis.
A. measles. B. herpes labialis.
B. herpes zoster. C. central papillary atrophy.
C. scarlet fever. D. asymptomatic.
D. erysipelas.
16. Recurrent intraoral herpes simplex virus
10. Cervicofacial actinomycosis is most often infections are almost always located on the
seen in which of these anatomic locations? A. attached gingiva or hard palate.
A. Skin overlying the mandibular angle B. tongue.
B. Cervical lymph nodes C. buccal or labial mucosa.
C. Maxillary sinus D. soft palate or oropharynx.
D. Hard palate
2577_Ch03_077-118 09/07/14 3:16 PM Page 117
C h a p t e r
4
Immunologic Disease
Learning Outcomes
At the end of this chapter, the student will be able to:
4.1. Distinguish between an antigen and 4.7. Distinguish between primary and sec-
an antibody, differentiate between ondary immune deficiency disorders.
self and nonself antigens, and define 4.8. Recognize the clinical features of al-
major histocompatibility complex. lergic disorders including contact
4.2. Give examples of the cells of the normal dermatitis, contact stomatitis,
immune system and their functions. plasma cell gingivitis, cinnamon
4.3. Differentiate between the humoral stomatitis, and hypersensitivity to
and cellular immune responses, and dental restorative materials.
give an example of each. 4.9. Differentiate among the key features
4.4. Differentiate between active and of minor recurrent aphthous ulcers
passive immunity. and herpetiform aphthous ulcers.
4.5. Recognize descriptions of four types 4.10. Give examples of systemic disorders
of hypersensitivity and give an associated with major aphthous ulcers.
example of each. 4.11. Differentiate among the clinical
4.6. Explain autoimmunity and list at least features of the three forms of lichen
two autoimmune diseases. planus.
Continued
119
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4.12. Differentiate among the diseases 4.15. Recognize the oral manifestations
that cause desquamative gingivitis. of granulomatous disorders of
4.13. Discuss the oral manifestations of immunity.
systemic immunodysregulation 4.16. Discuss the key clinical features of
leading to hives and angioedema. Sjgrens syndrome and the long-
4.14. Recognize the key features of term outlook for Sjgrens patients.
systemic and discoid lupus
erythematosus.
Bone marrow
B cell
Lymphoid
progenitor
Hematopoietic T cell
stem cell
Natural
killer cell
Myeloid
progenitor
Neutrophil
Granulocytes
Red blood
cells Eosinophil
Platelets
Basophil
tissues, including the tonsils. Each B cell is pro- Immunoglobulin G, or IgG (gamma globulin), is a
grammed to produce one specific antibody, a mol- circulating antibody that works by coating microbes
ecule that can bind to one specific antigen. When a and speeding their uptake by phagocytes. IgG ac-
B cell encounters the type of antigen that matches counts for approximately 80 percent of all antibod-
the antibody it can produce, the B cell becomes ac- ies in serum.
tive. It multiplies and creates plasma cells, which are IgM is the first antibody produced in response to an
cells that produce antibodies. The activated B cell antigen. It is found on the surface of B cells and can
also produces some B cells that will remain in the effectively kill bacteria. IgM is the largest-sized an-
body and retain the memory of the encountered tibody and makes up 5% to 10% of the antibodies
antigen. in serum.
Antibodies, also called immunoglobulins (Igs), IgA is found in body fluidstears, saliva, and the
are released into the bloodstream. All Igs have the secretions of the respiratory and digestive tracts
same basic structure, with two identical heavy and guards the entrances to the body. IgA accounts
polypeptide chains and two identical light chains for 10% to 15% of antibodies in serum.
arranged in a Y-shaped molecule (Fig. 4-4A). The end IgE is found in the skin and mucous membranes and
of the polypeptides located at the tips of the Ys arms triggers the release of histamine, an important pro-
represents the variable regions of the antibody, which tein of the inflammatory response. Another func-
differ from one antibody to the next and allow one tion is to naturally protect against parasitic
antibody to recognize its matching antigen. The rest infections. It is also responsible for the symptoms
of the molecule is the constant region, common to an- of allergy. IgE antibodies make up only 0.002 per-
tibodies of the same type. Five different types of anti- cent of total serum antibodies.
bodies exist with different combinations of heavy and IgD remains attached to B cells and participates in
light chains, and different structures and functions regulating B cell responses. IgD antibodies make up
(Fig. 4-4B). only about 0.2 percent of the total serum antibodies.
2577_Ch04_119-162 11/07/14 6:11 PM Page 123
(in the paracortex). Like macrophages, dendritic cells (in which case the cytokine is called a chemokine), and
in lymphoid tissues act as antigen-presenting cells and activating immune cells. As mentioned previously, cy-
help stimulate T cells. tokines produced by monocytes and macrophages are
Granulocytes are named because they contain granules called monokines. Conversely, cytokines produced by
filled with potent chemicals, such as digestive enzymes lymphocytes are called lymphokines. Cytokines in-
and reactive oxygen species, which can destroy microor- clude a diverse assortment of interleukins, interferons,
ganisms. The predominant granulocyte, the neutrophil, and growth factors (Table 4-1).
is also a phagocyte. The main function of neutrophils is
to ingest microorganisms and digest them with the con- Normal Immune Response
tent of their granules. Other granulocytes, eosinophils As pathogens attempt to enter our body, the immune
and basophils, function by releasing the content of their response is set in motion in an orchestrated course of
granules toward harmful cells or microbes that would events, with different players called to action as time
be too big to be ingested, such as parasites. Mast cells are progresses (Fig 4-5). In effect, the immune response
the tissue equivalents of basophils but are found in the can be broken down into two main phases: nonspecific
lungs, skin, tongue, and linings of the nose and intestinal immunity and specific immunity.
tract, rather than the bloodstream. Both mast cell and
basophil granules contain histamine, which contributes Nonspecific Immunity
to inflammation and allergy. B and T cells represent a very powerful defense sys-
tem, but their initial activation when first encountering
Cytokines an antigen requires time. Therefore, the body also has
Immune cells may communicate by direct physical defense mechanisms that are either always in place
contact or by releasing and responding to proteins (acting as preventive measures) or that can act rapidly.
called cytokines. Cytokines have various functions, in- These defense mechanisms include physical barriers
cluding carrying information to and from immune (such as skin, mucous membranes, nasal hairs, sneez-
cells, enhancing cell growth and differentiation, at- ing, coughing); chemical barriers (such as tears, sweat,
tracting cells to migrate to an area by chemotaxis saliva); the inflammatory response; the clotting system;
With the integrated efforts of humoral and cellular may receive an injection of gamma globulins to boost
immunity, the initial response to a new antigen is called their immune response against potential hepatitis
primary response. The primary response may take up to viruses.
2 weeks to fully develop and for the antibody level in
the blood (or antibody titer) to reach its peak. During
IMMUNOPATHOLOGY
a second exposure to the same antigen, the antigen will
trigger a secondary response that will be more rapid, ex- Immunopathology represents the study of diseases
tensive, and prolonged than the primary response. caused by dysfunctions of the immune system. The
Memory B and T cells left behind after the primary re- diseases can be grouped into three main categories:
sponse are primed, which means that they become hypersensitivity reactions, autoimmune diseases, and
active more rapidly and can produce a more effective immune deficiency diseases.
response during a second encounter with the antigen.
Hypersensitivity Reactions
Active and Passive Immunity In hypersensitivity reactions, also called allergic re-
Depending on the type of antigen (e.g., bacterial or actions, the immune system produces an exaggerated
viral), the amount of antigen, and the route of entry response that leads to body tissue destruction. Often
into the body, the immune response may be strong or the exaggerated response is directed against an antigen
weak, short-lived or long-lasting. The immune re- that would not be otherwise harmful. This type of
sponse is also influenced by inherited genes, and not antigen is also called an allergen. These allergic reac-
all individuals respond to an antigen similarly. Some tions usually stop when the allergen is removed. These
may easily overcome an infection, others may not. Im- reactions are classified into four main types, based on
munity of a low-responding individual may be boosted the nature of the immune reaction that causes them.
in two different ways: active immunity or passive
immunity. Type I Hypersensitivity: Anaphylactic or
In active immunity, antibodies are produced in re- Atopic Reactions
sponse to an antigen. Active immunity happens natu- In type I hypersensitivity, the immune reaction occurs
rally when the body fights an infection, such as with within minutes of an exposure to a previously encoun-
the varicella-zoster virus which causes the childhood tered allergen, such as pollen, latex, penicillin, or cat
disease chickenpox. Active immunity may also occur hair. These antigens are usually not harmful to the
artificially, through vaccination. Vaccines contain ei- general population, only to specific individuals in
ther killed or weakened live bacteria or viruses, or which plasma cells produce IgE in response to the
chemically altered toxins called toxoids. Memory cells antigen. IgEs bind to the surface of mast cells, which
produced in response to the vaccine remain in the then release their granules that contain histamine. His-
body, usually for life. Booster shots can be given to en- tamine increases dilation and permeability of blood
sure that the memory remains strong. Typical vaccines vessels, and causes constriction of smooth muscle cells
include polio, tetanus, hepatitis A and B, measles, in bronchioles of the lungs.
mumps, and rubella. When the reactions are limited to the skin, such as
In passive immunity, the antibodies are not pro- insect stings or latex allergy, or to upper respiratory
duced by the host but are rather received from another manifestations, such as hay fever, they are called
source. Passive immunity occurs naturally in infants atopic reactions. Symptoms depend on where the al-
who are born with weak immune responses but are lergen comes into contact with the body and are usu-
protected for the first few months of life by antibodies ally treated with antihistaminic drugs. When the
they receive from their mothers through the placenta. reactions are generalized and systemic, they are called
Babies can also receive some antibodies from breast anaphylactic reactions. These can be life-threatening
milk that help to protect their digestive tracts. How- if the individual has difficulty breathing. Anaphylactic
ever, passive immunity lasts only a few weeks or reactions must be recognized and treated promptly.
months because it is not associated with the produc- Treatment options include oral antihistamines, corti-
tion of memory cells in the body. Passive immunity can costeroids, and epinephrine injections in the most se-
also occur when someone is given an injection of anti- vere cases. Individuals who are aware of their allergies
serum or gamma globulins (IgGs). For example, dental should remain on constant alert and avoid any contact
health-care workers who incur a needle stick injury with the allergens.
2577_Ch04_119-162 11/07/14 6:11 PM Page 127
Type II Hypersensitivity: Cytotoxic Reactions which is why they are also called delayed hypersensitivity
Type II hypersensitivity reactions, called cytotoxic reac- reactions. In these reactions, small antigenic molecules,
tions, are also caused by antibodies, usually IgGs or called haptens, combine with the proteins of a specific cell
IgMs. In cytotoxic reactions, the antibodies bind to a type and trigger a T-cell reaction, resulting in direct
specific type of cells, either foreign cells that have been tissue destruction by the T cells or by a secondary
introduced to the body, or cells that are wrongly rec- inflammatory reaction. Examples of cell-mediated
ognized as foreign. As a result, the cells can no longer hypersensitivity include contact dermatitis (skin reaction)
function well and may even be killed. Examples of and contact stomatitis (reaction in the mouth). Examples
cytotoxic reactions include: of substances that may cause a contact allergic reaction
include natural rubber latex, poison ivy, metals, cosmet-
Reactions to transfusions when a person is given an ics, and some chemicals used in toothpastes and mouth
incompatible blood type. rinses. The most common dermatologic symptom is the
Erythroblastosis fetalis, or Rhesus reaction, during development of an erythematous vesicular rash, which
a second pregnancy of an Rh-negative mother with is extremely pruritic (itchy). In the mouth, symptoms in-
an Rh-positive fetus. clude diffuse erythema, ulcers, and vesicles. These symp-
Hyperthyroidism, or Graves disease, in which thy- toms will not stop until the antigen is eliminated or all
roid cells are damaged but not destroyed by a cyto- areas touched by the antigen have been destroyed, which
toxic reaction and end up producing an excess of may take several days or weeks. Symptoms may be alle-
thyroid hormones. viated with the use of topical corticosteroids.
Autoimmune disorders resulting from a change in Allergic contact dermatitis is a type IV hypersensitiv-
the MHC molecules of a specific cell type, such as ity reaction, caused by contact with an allergen such
in pemphigus vulgaris, cicatricial pemphigoid, and as residues of chemicals used in manufacturing latex
acute rheumatic fever. In these conditions, the cause gloves. The delayed reaction will occur in a sensitized
of the change in the MHC molecules is usually individual between 24 and 48 hours after exposure to
unknown and irreversible. the offensive substances. Symptoms are similar to
those observed in irritant contact dermatitis, although
Type III Hypersensitivity: Immune Complex- irritant contact dermatitis is not a hypersensitivity re-
Mediated Reactions action. Irritant contact dermatitis may be caused by
In type III hypersensitivity reactions, immune com- chemicals such as surface disinfectants, mechanical
plexes form between IgM, IgA, or IgG antibodies and rubbing of the gloves against the skin, or by the pow-
circulating antigens such as bacteria, viruses, drugs, der added to some gloves to make them easier to put
chemicals, or endogenous molecules. Normally, on. Changing from latex to latex-free gloves may help
immune complexes are rapidly removed from the determine what type of dermatitis is present.
bloodstream; however, sometimes they continue to Autoimmune diseases resulting from type IV hy-
circulate and eventually become trapped in the base- persensitivity include host-versus-graft rejection, type
ment membranes of tissues of the kidneys, lungs, skin, 1 diabetes, and possibly Sjgrens syndrome.
joints, or blood vessels. There, they initiate an inflam-
matory response that leads to localized or systemic Autoimmune Diseases
tissue destruction. Autoimmune examples of immune In autoimmune diseases, the immune system loses its
complex-mediated reactions include systemic lupus ability to distinguish self (normal tissues) from nonself
erythematosus, rheumatoid arthritis, and some forms (foreign antigens), or a change occurs in the MHC
of kidney disease, including glomerulonephritis. markers of self on some tissues. This triggers activated
B cells to produce antibodies against self-antigens,
Type IV Hypersensitivity: Cell-Mediated or called autoantibodies, and/or cytotoxic T cells that at-
Delayed Hypersensitivity Reactions tack normal tissues. The result is an immune and
Type IV hypersensitivity reactions are also called cell- inflammatory response that destroys normal body cells,
mediated hypersensitivity reactions because they result from tissues, and/or organs. This response manifests as a type
the action of T cells rather than antibodies. Neither B II, III, or IV hypersensitivity reaction similar to that oc-
cells nor antibodies are involved in this type of hyper- curring in an allergic reaction. However, in an allergy
sensitivity reaction. In addition, these reactions usually the immune system reacts to external, normally innocu-
take more time to develop, from 24 to 72 hours or more, ous substances, whereas in autoimmune disorders, the
2577_Ch04_119-162 11/07/14 6:11 PM Page 128
immune system reacts to normal body tissues. The African American, Hispanic American, and Native
cause for this abnormal reaction is unknown. American women, have an increased risk of developing
Because autoimmune diseases tend to run in families, autoimmune diseases. Moreover, for unknown reasons,
they are probably associated with predisposing genes. the prevalence of autoimmune diseases is increasing,
Exposure to specific environmental triggers, such as affecting approximately 5% to 8% of adults in North
bacteria or drugs, combined with genetic susceptibility, America and Western Europe.
may be responsible for the necessary changes leading to More than 80 types of autoimmune diseases have
autoimmunity. Women of childbearing age, particularly been identified (Table 4-2). Some may result in the
Graves disease Thyroid Tiredness, depression, sensitivity to cold, weight gain, muscle
weakness and cramps, dry hair, tough skin, constipation.
Sometimes, none.
Rheumatoid arthritis Joints; also lungs and Inammation of the joints (hands), muscle pain, deformed
skin joints, weakness, fatigue, loss of appetite, weight loss.
Hashimotos thyroiditis Thyroid Insomnia, irritability, weight loss without dieting, heat sensi-
tivity, sweating, ne brittle hair, weakness in muscles, light
menstrual periods, bulging eyes, shaky hands.
Sometimes, none.
Vitiligo Skin melanocytes White discolored patches appearing in dierent parts of the
skin, mucous membranes, and retina; premature graying of
scalp hair, eyelashes, eyebrows, and beard.
Type 1 diabetes Pancreatic Islets of Increased thirst and frequent urination, extreme hunger,
Langhans weight loss, fatigue, blurred vision.
Pernicious anemia Stomach Lack of intrinsic factor production resulting in vitamin B-12 de-
ciency and anemia. Symptoms of anemia due to vitamin B-12
deciency, including shortness of breath, fatigue, dizziness,
and pale skin; heart murmurs, fast heartbeats, arrhythmias, an
enlarged heart (cardiomegaly), or even heart failure.
Nervous system symptoms, including feelings of numbness,
tingling, weakness, lack of coordination, clumsiness, impaired
memory, and personality.
Sometimes, none.
Multiple sclerosis Nervous system Weakness; trouble with coordination, balance, speaking, and
walking; paralysis; tremors; numbness and tingling feeling in
arms, legs, hands, and feet.
Glomerulonephritis Kidneys Blood in the urine, foamy urine; swelling of the face, eyes,
ankles, feet, legs, or abdomen; also abdominal pain, cough,
diarrhea, general ill-feeling, fever, joint and muscles aches,
loss of appetite, shortness of breath.
Lupus (systemic lupus Skin, joint, kidneys, Swelling and damage to the joints, skin, kidneys, heart, lungs,
erythematosus) heart, brain, red blood vessels, and brain; buttery rash across the nose
blood cells, others and cheeks; rashes on other parts of the body; painful and
swollen joints; sensitivity to the sun.
Sjgrens syndrome Salivary glands, tear Dry mouth (xerostomia), diculties swallowing, cavities,
glands, joints periodontal disease, mouth sores and swelling, stones
and/or infection of parotid glands; eye dryness; joint pain or
inammation.
2577_Ch04_119-162 11/07/14 6:11 PM Page 129
who often suffer from multiple infections. These in- HIV infection, and many more. Critically ill, older, or
fections may be: hospitalized patients are also more susceptible to de-
Mild, but could take longer than usual to treat. velop secondary immunodeficiencies. Moreover, com-
Caused by microorganisms that would not other- mon viral infections, including influenza, infectious
wise cause infections in a child with a normal mononucleosis, and measles, as well as blood transfu-
immune system, in which case they are known as sions, surgeries, smoking, and stress, may all result in
opportunistic infections. temporary immunodeficiencies. The major manifesta-
May be recurrent, which means that they keep tions of secondary immunodeficiency are recurrent,
coming back. opportunistic infections. In the oral cavity, these may
May also be severe and even life-threatening. manifest as infections with the fungus Candida albicans
or as aggressive periodontal disease. Usually, the im-
Other health problems may develop as complica- munodeficiency disappears when the underlying con-
tions of the immune deficit, including delayed growth, dition is resolved. Therefore, treatment focuses on the
anemia, arthritis, autoimmunity, or cancer. Some chil- underlying disorder.
dren may have no symptoms at all and the disease may One common form of secondary immunodeficiency
not be discovered until later in life, during adolescence is acquired hypogammaglobulinemia, in which patients
or young adulthood. have very low but detectable levels of immunoglobulins.
Primary immunodeficiency diseases can usually be The origin of this condition is unknown, and it mani-
treated and cured, especially if they are diagnosed early fests in young adults in the form of recurrent infections.
and therapy is started promptly to limit permanent It can generally be treated with antibody replacement
damages and complications. Therefore, discovering therapy. Another common cause of secondary immun-
these diseases early is very important. The goals of odeficiency is corticosteroid therapy, which is used
treatment are to clear up the current infection, avoid in the treatment of autoimmune disorders such as
microbes, prevent exposure to new infections, and cor- rheumatoid arthritis or systemic lupus erythematosus.
rect the immunodeficiency. Current infections may Currently, the most prominent secondary immunode-
require aggressive treatments, such as long-term use ficiency is AIDS.
of antibiotics and/or antifungal medications, and pre- HIV can destroy or disable vital T cells, paving the
ventive measures. When standard medications fail, way for a variety of immunologic shortcomings. The
hospitalization may be required to administer antibi- virus can also hide out for long periods (becomes
otics and other drugs intravenously. Good hygiene and latent) in immune cells. As the immune defenses falter,
nutrition practices, as well as avoiding individuals with a person develops AIDS and falls prey to unusual,
colds or infections and large crowds, will help avoid often life-threatening infections and rare cancers.
and minimize contact with microbes. In severe cases,
correcting the immunodeficiency may necessitate a
bone marrow transplant, a procedure that transfers bone
CLINICAL FEATURES OF IMMUNE
marrow from a healthy individual to the patient. In
DISORDERS
many cases, antibody replacement therapy, consisting of The central function of the immune system is to dis-
the regular infusion or injection of immunoglobulins tinguish foreign antigens from self-components of
taken from healthy blood donors (see passive immu- body tissues. Sometimes there is failure to discriminate
nity) will be effective. between harmful and harmless antigens. Also, there
can be failure to discriminate between self and nonself
Secondary Immunodeficiency body proteins. Inappropriate immune responses are
By definition, acquired or secondary immunodeficien- responsible for a wide variety of conditions. Depend-
cies develop after birth and are not due to a genetic ing on the inappropriate reaction, there can be a vari-
defect. They are more common than primary ety of clinically apparent signs and symptoms, ranging
immunodeficiencies and may be caused by a variety of from a skin rash to failure of a major organ system.
factors, including therapies that suppress the immune
system, such as chemotherapy, radiation, corticosteroid Allergic Disorders
therapy, and any prolonged serious illness, such as dia- An allergy is a disorder acquired by the immune sys-
betes, renal disease, malnutrition, cancer, tuberculosis, tem, also referred to as hypersensitivity disorder. This
2577_Ch04_119-162 11/07/14 6:11 PM Page 131
means the bodys immune system is overly reactive to product with an allergen that comes into contact with
a usually harmless antigen, termed an allergen, which the skin around the mouth (Fig. 4-8). Initial exposure
in another individual may provoke no response at all. to the substance does not cause a reaction; it sensi-
Antibodies are produced to the allergen and the sub- tizes the skin. Subsequent exposure causes lesions
sequent attack is experienced as symptoms by the pa- that are confined to the area of contact. Symptoms
tient. Allergic reactions occur to normally harmless include blisters or a pruritic (itchy) red rash that
substances in the environment including grasses, pol- appears within 1 to 2 days post exposure and may take
lens, animal dander, and occasionally medications and as long as 4 weeks to resolve completely if left
dental materials. untreated (Fig. 4-9). Corticosteroids, such as hydro-
cortisone, may be applied to small areas of the skin.
Allergic Contact Dermatitis If the reaction covers a relatively large portion of the
Contact dermatitis is inflammation of the skin (der- skin or is severe, systemic medication, such as the
matitis) caused by direct contact of an offending antihistamine diphenhydramine or prednisone, may
agent on the skin. When inflammation is the result be prescribed.
of direct contact with an allergen, it is termed allergic
contact dermatitis and is considered a type IV hyper- Allergic Contact Mucositis
sensitivity reaction. Substances that cause allergic Contact mucositis is inflammation of mucous membranes
contact dermatitis include such things as poison ivy, (mucositis) caused by direct contact of an offending
base metals, detergents, chemical preservatives, allergen. When inflammation is the result of direct ex-
industrial chemicals, or latex rubber. Patients who posure to an allergen, it is termed allergic contact
wear metal watch bands often report tenderness and mucositis. Substances that cause allergic contact mucosi-
itching around their wrists (Fig. 4-6). Latex allergy is tis may include dental materials such as denture base
a common problem among health-care workers who acrylic, latex rubber dam material, or orthodontic
treat patients (Fig. 4-7). Perioral dermatitis may re- elastics; oral hygiene products such as flavoring agents;
sult when a patient uses a new dentifrice or cosmetic detergent additives; or foods. Often the allergen
Figure 47 Contact dermatitis. Health-care worker with Figure 49 Contact dermatitis. Rash of the skin in
an allergy to latex gloves. (From Barankin, B: Derm Notes. response to contact with an allergen. (From Barankin, B:
F.A. Davis, Philadelphia, 2006, p 67.) Derm Notes. F.A. Davis, Philadelphia, 2006, p 166.)
2577_Ch04_119-162 11/07/14 6:11 PM Page 132
remains unknown until the patient undergoes allergy many red-colored cinnamon-flavored gums, candies,
testing. and oral health-care products. Patients present with
Clinically, contact mucositis appears as an erythe- tender or painful burning mucosa to which the agent
matous, edematous, or atrophic zone of mucosa at the has contact, such as a focal area of buccal mucosa, dif-
point of contact. When the source is toothpaste or fuse gingival lesions, or chapped lips. Because pa-
mouth rinses, patients will complain of a generalized tients generally do not associate the pain with the
discomfort and burning sensation, with mild swelling, allergen, they continue to use the product. This
erythema, and superficial sloughing of the surface ep- chronic exposure causes the initial erythematous le-
ithelium. Deep ulcers or blisters generally do not sions to transform to thick white plaques that may re-
occur. main painful (Fig. 4-11). This pain helps to distinguish
Some forms of oral allergic contact mucositis are cinnamon stomatitis from other red and white lesions
common enough to warrant their own clinical termi- that occur in the oral cavity. A similar reaction may
nology and are described next. occur as a result of contact with peppermint,
spearmint, or neem oils. Discontinuation of the prod-
Plasma Cell Gingivitis uct brings immediate relief, with resolution within
Plasma cell gingivitis has become relatively common hours or days.
since oral health-care products containing essential
oils, herbal additives, whitening, and anti-calculus
agents are readily available. Although their therapeu- Clinical Implications
tic effect is often beneficial to patients, they may Lesions of cinnamon stomatitis may resemble
cause a hypersensitivity reaction. Plasma cell gingivi- squamous cell carcinoma or an autoimmune mu-
tis appears as a diffuse enlargement of the free and cositis. The key to the diagnosis is sudden onset
attached gingiva characterized by erythema and loss of pain and tenderness with the use of a product
of stippling (Fig. 4-10). Other intraoral sites may that contains cinnamon aldehyde.
also demonstrate areas of tenderness and erythema.
The name of the condition reflects the presence of a
large number of plasma cells present when the tissue Hypersensitivity Reaction to Dental
is biopsied. Treatment involves discontinuing the use Restorative Materials
of the allergen. Topical anti-inflammatory agents Hypersensitivity reaction to dental amalgam is seen in
may be needed to resolve the mucositis. about 1% to 2% of individuals with amalgam restora-
tions. The word amalgam means several elements
Cinnamon Stomatitis merged into a single compound. Dental amalgams
Cinnamon stomatitis is an allergic reaction to cinnamon are composed of silver, copper, zinc, beryllium,
oil (cinnamon aldehyde) used as a flavoring agent in
nickel, mercury, or other materials in varying quan- allergic to cobalt and chromium. Porcelain-fused-
tities depending on the manufacturers specifications. to-metal (PFM) crowns and RPD frameworks con-
Patients may be allergic to one or more of these tain many of these metals. Contact hypersensitivity
metals. In hypersensitive (allergic) individuals, red reactions to these metals are seen adjacent to
and white tender lesions develop wherever the amal- the restoration or appliance when it is in place. In-
gam contacts the mucosa. The buccal mucosa and tense gingival erythema at the margin of a PFM
lateral borders of the tongue are the most common crown that does not respond to conventional gingi-
sites (Fig. 4-12). Treatment involves removing the val therapy may represent an allergic reaction to one
amalgam and replacing it with a composite or other of the metals in the framework of the restoration
nonmetallic material. (Fig. 4-13). Allergy testing for dental materials may
be necessary to determine which material causes the
reaction. Treatment involves replacing the restora-
tions with non-allergenic materials.
Clinical Implications
When a patient suers from an allergic reaction
Ulcerative Conditions
to the exposed metal margin of a crown, lesions
An ulcer is defined as the loss of epithelial continuity
are typically observed contacting the area on ad-
with exposure of the underlying connective tissue
jacent gingiva, buccal mucosa, or lateral tongue.
(Fig. 4-14 A, B, and C). A crater forms that fills in
Enhanced oral hygiene rarely brings improve-
with fibrin, granulation tissue, and necrotic debris,
ment. Patients who experience allergic reactions
giving the ulcer the characteristic yellow center with
to dental metals may also react to base metals in
erythematous halo (Fig. 4-15). Although ulcers may
jewelry. A quick and dirty way to help deter-
be caused by trauma (see Chapter 2) and viruses
mine this is to ask if they can wear cheap jew-
(see Chapter 3), there are several common immune-
elry or metal watch bands (refer back to Fig 4-6).
related ulcerative conditions. It is clinically important
If they report developing a rash, they may be al-
to distinguish among ulcer types because the success
lergic to nickel or other metals. Allergy testing
of their management and relief of symptoms depends
should be considered.
Figure 412 Contact mucositis to dental amalgam. Figure 413 Allergy-crown margin. Tender gingival
Hypersensitivity reaction to dental amalgam. Lesion makes lesions developed within weeks of crown placement.
direct contact lower molar restoration when mouth is at rest. Lesions resolved with removal of restoration.
2577_Ch04_119-162 11/07/14 6:12 PM Page 134
Aphthous Ulcers
The term aphthous (pronounced AFF-thus) comes
from the Greek word aphtha, meaning ulcer. So, the
term aphthous ulcer means ulcer-ulcer. This fitting
term reflects current lack of a precise etiology. The
A term recurrent aphthous ulcer (RAU) is used for several
different forms of ulcers that share an immune dysreg-
ulation etiology, the presence of painful ulcerations,
and a propensity to recur.
Three basic immune defects that may predispose the
patient to the development of RAU are: 1) decreased
mucosal barrier, 2) increased antigenic exposure and 3)
inherited or acquired immunodysregulation. Patients
B with one or more of these factors may be at increased
risk for developing RAU. If these patients have concur-
rent allergies, blood abnormalities, hormonal imbal-
ance, certain infections, nutritional deficiencies, or
experience emotional or physical stress, the risk in-
creases. Any combination of these factors may be re-
sponsible for destruction of the epithelium that leads
to the development of RAUs. Because no precise eti-
C ology exists for RAUs, successful treatment often
remains elusive.
Figure 414 Histopathologic and clinical features of a
nonspecific ulcer. A. Clinical appearance: Yellow necrotic
center with erythematous halo. B. Ulcers are craters
caused by tissue destruction. C. Crater fills with necrotic Clinical Implications
debris and inflammatory cells, which will be responsible
for tissue repair. Traumatic ulcers should clearly be distinguished
from RAUs based on the patients history. The pa-
tient will report injuring himself or herself in the
area of the ulcer or the clinician might observe a
defective restoration or prosthesis contacting the
ulcer. Treating a traumatic ulcer with topical anti-
inammatory medication, an acceptable treat-
ment for RAU, may lead to delayed healing and
possible infection in a traumatic ulcer.
Table 4.3 Clinical Features of Recurrent Aphthous Ulcers Versus Herpes Simplex
Feature Minor RAU Herpetiform RAU Major RAU Herpes
Etiology Localized immune Localized immune Systemic immune Herpes simplex
defect defect disorder virus
Prodrome No No No Yes
Intraoral location Nonkeratinized Nonkeratinized Nonkeratinized Keratinized
mucosa* mucosa* mucosa* mucosa*
Preceding vesicle No No No Yes
Ulcer appearance Single; Cluster 1 - single, large deep, Cluster that
less than 5 mm less than 5 mm irregular rolled coalesces into
border large ulcers
2 - cluster
Remission period Yes Yes No Yes
Extraoral sites No No No Yes
Diagnosis Clinical features Clinical features Systemic disease Clinical features;
consultations cytologic smear
Treatment Topical anti- Topical anti- Systemic manage- Topical antiviral
inammatory inammatory ment of underlying drugs during pro-
drugs drugs disease; drome only;
systemic im- systemic antiviral
munomodulary drugs for severe
drugs for idiopathic recurrent cases
etiology
Clinical Implications
Women who suer from frequent RAUs often re-
port a dramatic remission from the lesions dur-
ing pregnancy. This is most likely due to
increased epithelialization of the oral mucosa in
response to pregnancy hormones, thereby in-
creasing the integrity of the mucosal barrier.
defect of the immune system that leads to major RAUs This defect is seen primarily in patients with Turkish,
is more complex than minor RAUs. Frequently, these Japanese, or Eastern Mediterranean ancestry, but not
indicate underlying systemic disorders that must be in- limited to these groups. An estimated 15,000 to 20,000
vestigated. Some examples include Behets disease, Americans have been diagnosed with Behets disease.
cyclic neutropenia, HIV/AIDS, and Crohns disease. In the United Kingdom, there are less than 1,000 cases;
but in Japan, Behets disease is one of the leading
Behets Disease causes of blindness due to eye involvement.
Behets disease (pronounced Beh-SETS) is a multisys- The disease first appears in young adults, and often
tem condition that initially affects the oral mucosa and is seen but not recognized before puberty. Onset is
eventually the genitals, skin, joints, eyes, or central marked by the appearance of major RAUs of the oral
nervous system (Fig. 4-19). It is an autoimmune vas- cavity. Oral lesions are present in 99 percent of cases
culitis, or inflammation of the blood vessels, that, if left and are the first sign in nearly 75 percent of cases.
untreated, can involve the gastrointestinal tract, pul- Many years may pass before patients develop other Be-
monary, musculoskeletal, and neurological systems. To hets lesions, so the suspicion may be very low, espe-
avoid serious life-threatening complications, medical cially if there is no family history. Patients with oral
treatment is necessary. ulcers of Behets disease (Fig. 4-20) without other
Behets disease is considered a rare disease. The signs may be misdiagnosed until one of the other signs
cause remains unknown. Persons with a defect of a spe- develops. This lack of multiple features of Behets dis-
cific gene in the immune system, called antigen HLA- ease may delay the actual diagnosis for years.
B51, are predisposed to developing Behets disease. Ulcers can vary in size, but patients may have as
many as six ulcers at the same time, and are rarely
without an ulcer. The diagnosis may be suspected
when the ulcers respond to systemic immunosuppres-
sive therapy but not to topical therapy or other pallia-
tive measures. When they are misdiagnosed as having
an allergy, patients with Behets lesions rarely respond
to avoidance of the suspected allergen. Biopsy of the
oral ulcers rarely leads to the precise diagnosis because
the ulcers have nonspecific histopathologic features.
Proper testing for Behets disease includes blood tests,
eye examinations, and skin tests.
Clinical Implications
Patients with major RAUs should be referred to
an immunologist or rheumatologist to determine
if Behets disease is the cause. The diagnosis is
not made in a dental oce.
Cyclic Neutropenia
Cyclic neutropenia is a rare disease of childhood caused
by a mutation of one of the genes called ELA2 that reg-
ulates neutrophil growth and development. Neu-
trophils are the first line of defense in a wide range of
infections and other injuries. Although most patients
are children, acquired forms of cyclic neutropenia exist
in adults. The disease is characterized by reduction or
Figure 419 Spectrum of systemic involvement in loss of neutrophils in a regular cycle that occurs every
Behets disease: Eye lesions, major aphthous ulcers, 21 days for 3 to 6 days duration. During this time,
genital ulcers, arthritis, and skin rashes. patients experience infection, including gingivitis and
2577_Ch04_119-162 11/07/14 6:12 PM Page 138
Clinical Implications
An underlying systemic disease is not identied in
about 50 percent of cases of major aphthous ul-
cers. These lesions can be treated empirically with
topical or locally injected anti-inammatory
agents. Systemic corticosteroids should be re-
served for severe outbreaks. Keep in mind that
most patients are children or adolescents, so
Figure 420 Behets disease. Typical large long- treatment with systemic corticosteroids should be
standing recurrent ulcer seen in Behets patient. limited due to growth and development consider-
ations. Alternative treatments, including pen-
toxyphiline and tacrolimus, should be considered.
severe periodontitis, and a decreased ability to heal
after minor trauma. Fever and malaise are characteristic
of cyclic neutropenia, as well as recurrent oral ulcera- Erythema Multiforme (EM)
tions. Cyclic neutropenia is unusual in that the ulcers Erythema multiforme (EM) (pronounced multi-
occur on keratinized mucosa. This helps distinguish FORMEE) is a self-limiting disease that causes a char-
them from RAUs. When neutrophils return to normal, acteristic skin rash and often severe outbreaks of
the symptoms regress until the next cycle begins. painful ulcerations of the mucous membranes. EM
most commonly occurs in young adults and affects
males more often than females. EM differs from the
Clinical Implications three forms of RAU in that EM has a more generalized
Cyclic neutropenia is dicult to detect in the distribution of ulcerations and erosions accompanied
dental oce due to its cyclical nature. However, by severe pain. Approximately 50 percent of patients
when a child presents to the dental oce with re- who experience erythema multiforme have had a re-
current severe gingivitis with ulceration, the par- cent exposure to a known trigger. The exact immuno-
ent should be asked if the child experiences an logic mechanism is not fully understood, but it is
unusual number of recurrent infections of the uri- suspected that once the triggering agent causes pro-
nary tract, pharynx, or other organ system. This duction of antibodies, those antibodies are inappropri-
can dierentiate cyclic neutropenia from primary ately identified as foreign by the immune system. The
herpetic gingivostomatitis (see Chapter 3). Pa- immune system then produces autoantibodies to try to
tients must see a physician to have serial blood remove them. For example, one of the common trig-
counts over several weeks or months to deter- gers for EM is herpes simplex virus. When the patient
mine if their neutrophil counts change over time. develops a herpetic lesion, anti-herpes antibodies are
produced. After the infection is resolved, the anti-her-
pes antibodies are seen as foreign instead of normal
HIV and AIDS proteins by the patients immune system. Autoantibod-
As discussed in Chapter 3, patients with undiagnosed ies against these proteins appear in the blood stream 2
or untreated HIV infection may experience recurrent to 3 weeks after the herpes outbreak clears. They er-
aphthous-like ulcerations. The ulcers may resemble roneously destroy some of the patients normal tissues,
minor RAU, but herpetiform RAU, the least common resulting in the lesions of EM.
of the three types, is common in this population. As Besides herpes simplex, triggers include but are not
immunosuppression becomes more severe, ulcerations limited to other infectious agents such as Mycoplasma
become more frequent and also appear on keratinized pneumoniae, Bartonella henselae (which causes cat-
mucosa. Patients on HAART (highly active anti-retro- scratch disease), Salmonella spp., Treponema pallidum,
viral therapy) tend to have few recurrences of their and Chlamydia. Some drugs that have been implicated
2577_Ch04_119-162 11/07/14 6:12 PM Page 139
Clinical Implications
If a patient with EM reports herpes simplex as
their trigger, use of systemic antiviral medication
may help prevent herpes outbreaks and hence
recurrence of EM.
Lichen Planus
Figure 423 Erythema multiforme. Painful ulceration of
Lichen planus is a common disease of both the skin or the gingiva and labial mucosa.
mucous membranes or both. Its name is derived from
the appearance of the reticular form of the disorder that
resembles lichens that grow on rocks and trees The etiology of lichen planus is still under investi-
(Fig. 4-27A and B). Despite its name, it is not related gation. We do know that cytotoxic T-cells (discussed
to parasitic or fungal organisms, nor is it infectious or earlier in this chapter) are produced by the host and
contagious. It is most commonly seen in middle-aged recognize, as yet undetermined, proteins in the basal
women, but men comprise a large portion of patients. layer of stratified squamous epithelium as foreign.
2577_Ch04_119-162 11/07/14 6:12 PM Page 140
Figure 428 Lichen planus. Purple, polygonal, pruritic Figure 430 Lichen planus. Wickham striae of the
plaques with overlying Wickhams striae. buccal mucosa.
the diagnosis is established, the only management is believe that spicy foods cause their condition, but
close follow-up for any changes over time. Patients avoiding them brings little relief. Lesions of erosive
with reticular lichen planus do not tend to progress to lichen planus present with painful shallow ulcers with
the more severe forms, but tend to regress and remit red and white borders characterized by peripheral
over several years. Treatment with systemic immuno- striae (Fig. 4-34A). Details of erosions are seen in Fig-
suppressive medications may produce undesirable side ure 4-34B. Biopsy will identify features similar to other
effects detrimental to the patients oral health. forms of lichen planus, but clinically, large zones of ep-
ithelium will be eroded or ulcerated with exposure of
Atrophic Lichen Planus underlying connective tissue, a process that is respon-
Atrophic lichen planus is recognized by mucosal thinning sible for the intense pain experienced by the patient.
and erythema with evidence of Wickhams striae at the Loss of surface epithelium and intense pain may be
periphery of the lesion (Fig. 4-33). Patients complain produced by other conditions, so a biopsy is needed to
of pain and tenderness, especially when eating spicy confirm the diagnosis.
foods or strong mints. Despite the pain, the mucosa Once a diagnosis of erosive lichen planus is estab-
remains intact. The gingiva, lateral tongue, and buccal lished, the patient may require systemic anti-inflam-
mucosa are the most common areas affected. matory or immunosuppressive medications to relieve
Atrophic lichen planus may be confined to the gin- the lesions. Topical medications are often not potent
giva, where it is often mistaken for recalcitrant gingivi- enough to lead to re-epithelialization. However, once
tis that does not respond to routine nonsurgical re-epithelialization has occurred, topical medications
periodontal therapy, including chlorhexidine glu- may be adequate for maintenance.
conate rinses. A biopsy is needed to establish the diag-
nosis because other conditions may mimic atrophic
lichen planus and not respond to lichen planus treat-
ment. Because the lesions are symptomatic, treatment
with topical immunosuppressive and anti-inflamma-
tory medications may be necessary.
B
Figure 434 A. Erosive LP gingiva, tongue, and buccal
mucosae. A patient with extensive erosive lichen planus
Figure 433 Atrophic lichen planus. Painful atrophy of involving most mucosal surfaces. B. Close-up of erosive
gingiva makes brushing difficult. lesion of gingiva.
2577_Ch04_119-162 11/07/14 6:12 PM Page 143
Clinical Implications
MMP does not directly cause periodontitis; how-
ever, poor oral hygiene that results from inability
to perform proper home care may predispose the
patient to periodontal disease.
Other forms of pemphigoid aect the skin
and eyes. Bullous pemphigoid occurs mainly on
the skin, though it can occur intraorally. It is B
characterized by large bullae that rupture to
leave hemorrhagic-crusted lesions that heal Figure 437 Mucous membrane pemphigoid. A. Vesicle
formation B. Painful sloughing lesions of the oral mucosa.
2577_Ch04_119-162 11/07/14 6:12 PM Page 145
Pemphigus Vulgaris
The term pemphigus refers to a group of autoimmune
blistering diseases of the skin and mucous
membranes. The most common form, pemphigus
vulgaris, is a potentially life-threatening mucocuta-
neous (both skin and mucous membranes) disorder
that often manifests initially in the oral cavity. Pem-
phigus is derived from the Greek word pemphix,
meaning bubble or blister. Autoantibodies are
produced against proteins called desmogleins, which Figure 440 Pemphigus vulgaris. Painful blistering
form the desmosomes, the structural units that bind lesions of the skin. (From Barankin, B: Derm Notes. F.A.
epithelial cells to each other. When the autoantibodies Davis, Philadelphia, 2006, p 128.)
2577_Ch04_119-162 11/07/14 6:12 PM Page 146
Clinical Implications
Although specic treatment for autoimmune
vesiculobullous disorders will vary depending on
the specic diagnosis, most treatment involves
use of topical corticosteroids or other im-
munomodulary agents during some phase of
treatment. For patients whose lesions are con-
ned to the gingiva, fabrication of medication
carriers, similar to custom uoride trays, are
helpful in delivering the medication directly to
Figure 442 Pemphigus vulgaris. Desquamative lesions
of the gingiva in a patient with pemphigus. the gingiva. These carriers help improve compli-
ance and also reduce the amount of mediation
required (Fig. 4-44).
Although topical immunosuppressive therapy
for sepsis and death due to loss of significant amounts
is the mainstay for many of these conditions,
of skin, similar to victims of severe burns.
they have side eects, such as promoting intrao-
Linear IgA disease ral growth of Candida albicans. Patients should
be instructed to use topical medications spar-
Linear IgA disease can cause widespread damage to
ingly to help reduce these side eects.
the skin and mucous membranes, similar to that seen
in pemphigus. It may affect the eyes and lead to blind-
ness, much like cicatricial pemphigoid, and intraoral Clinical Implications
lesions heal with scarring. Linear IgA disease, as
If a patient being treated with topical corticos-
its name suggests, is characterized by deposits of
teroids reports that their symptoms have wors-
autoantibodies of the IgA class along the epithelial-
ened despite compliance to their medication
connective tissue junction, causing loss of adhesion,
regimen, he or she may be experiencing the
which results in vesicle and bullae formation. Like
symptoms of candidiasis. An oral cytological
pemphigus vulgaris, patients may develop large areas
smear is helpful in making this diagnosis. Individ-
of skin loss. Unlike others in this group of diseases,
uals who test positive for candida should be
there has been some association with administration of
placed on antifungal therapy. Dramatic improve-
medications prior to the onset of the disease. Because
ment in symptoms often results.
linear IgA disease shares several features with other
2577_Ch04_119-162 11/07/14 6:12 PM Page 147
Hives
Hives, also referred to as urticaria, is a type 1 hyper-
sensitivity reaction that is produced by histamine and
other compounds released from mast cells. Histamine
causes fluid to leak from the local blood vessels, lead-
ing to swelling (edema) in the skin. Hives appears as
a cluster of circular, red, spongiotic lesions called
B wheals, surrounded by areas of erythema called
flares. Hives may appear on any skin surface, but
Figure 443 Chronic ulcerative stomatitis. A and B.
usually spares the palms of the hands and soles of the
Chronic desquamative lesions of the gingiva that did not
respond to nonsurgical treatment or topical feet. Hives can vary in size from a few millimeters to
corticosteroids. involvement of an entire extremity (Fig. 4-45). Le-
sions tend to change size and move around, disap-
pearing and reappearing in a matter of hours. An
outbreak that looks severe may appear during the day
and be gone hours later, only to reappear later the
same day.
Marys bone
marrow is Mary's cells are
the graft. placed in John.
John's body rejects
Mary will Mary's cells.
donate her
bone marrow.
Clinical Implications
When bone marrow transplant recipients present
with oral pain and tenderness, they should be ex-
amined for candidiasis. If symptoms persist, re-
ferral to the physician for GVHD assessment is
essential.
Figure 447 Discoid lupus. Skin lesions of patient
diagnosed with chronic localized lupus of the skin.
Systemic Lupus Erythematosus
Systemic lupus erythematosus (SLE) is a multisystem dis-
ease in which patients develop autoantibodies against
proteins within the nucleus of their cells (anti-nuclear
antibodies or ANAs). Detection of the anti-Sm (Smith)
ANA is the most specific test for the diagnosis of SLE.
Additional skin and blood tests are used with clinical
evaluation of signs and symptoms to confirm the
diagnosis.
SLE is a complex disease with many features that
may not be present in all patients. A characteristic skin
presentation, the butterfly rash, is often present on
the face of a patient with SLE. The butterfly rash is
a photosensitive erythematous dermatitis that covers
the bridge of the nose and extends bilaterally onto the
cheeks, resembling the shape of a butterfly. The most
serious problems associated with SLE involve the kid- Figure 448 Systemic lupus erythematosis. Extensive
ney and heart. About half of all SLE patients develop facial lesions showing loss of pigment with surrounding
proteinuria, followed by nephrotic syndrome in which erythema in a patient with systemic lupus erythematosus.
tiny blood vessels in the kidney become leaky, allowing
protein to leave the body in large amounts. Patients
may go on to develop end-stage renal failure. Other
patients develop damage to the heart valves that may be advised to use sunscreen and avoid prolonged out-
predispose the patient to bacterial endocarditis, which door exposure.
can have life-threatening consequences. Most patients Oral manifestations of both SLE and discoid lupus
suffer from arthritis and myalgia. develop in less than half of all patients but can be rec-
Chronic cutaneous lupus (CCL) is a milder form of ognized in the dental office. In general, patients with
lupus that presents with no systemic life-threatening SLE present with signs and symptoms similar to ero-
symptoms. CCL dermatitis is characterized by mul- sive lichen planus (Fig. 4-49). Occasionally, white
tiple scaly patches surrounded by an erythematous plaques or erosive areas with a finely speckled keratotic
halo termed discoid lupus erythematosus (Figs. 4-47 and rim rather than Wickhams striae are seen on the buc-
4-48). Some patients with SLE also develop discoid cal mucosa. Desquamative gingivitis may also develop
lupus, but not all patients with discoid lupus develop (Table 4-4). Patients with SLE and oral lesions must
SLE. In light-skinned individuals, the patches tend receive medical attention to resolve their lesions. Pa-
to become hyperpigmented. In dark-skinned individ- tients with discoid lupus in the oral cavity may respond
uals, the patches tend to become hypopigmented. to topical corticosteroids or other immunomodulary
Sunlight exacerbates the lesions, so the patient should drugs, such as tacrolimus.
2577_Ch04_119-162 11/07/14 6:12 PM Page 151
Sarcoidosis
Sarcoidosis is a complex multisystem granulomatous
disorder of unknown etiology for which the antigenic
stimulus has yet to be identified. It is likely that several
different antigens are responsible, which affect patients
who have a genetic predisposition for development of
the disease. Sarcoidosis can be life-threatening if it af-
Figure 449 Discoid lupus. Painful desquamative lesions
of discoid lupus resembling erosive lichen planus. fects vital organs and is left undiagnosed. The diagnosis
of sarcoidosis is often made from a lymph node biopsy.
Patients experience an increase in serum or urinary cal-
Granulomatous Diseases cium, which can be accompanied by muscle pain and
Granulomatous inflammation is a very specific type of weakness. Sarcoidosis primarily affects two groups of
inflammation characterized histologically by the pres- adults: those aged 20-40 and those older than age 60.
ence of granulomas, which are masses of epithelioid Because it is a multisystem disease, clinical signs and
histiocytes containing numerous multinucleated giant symptoms are numerous and vary from patient to patient
cells supported by a fibrous matrix. These granulomas (Fig. 4-50). Signs of sarcoidosis that may be observed in
replace normal tissues and form irregular firm the dental office include lupus pernio, a cluster of in-
swellings. Granulomatous inflammation is a response durated, violaceous (purple) exophytic lesions of the skin,
to a number of specific conditions, such as foreign especially on the skin of the face (Fig. 4-51). Uveitis and
body reactions (see Chapter 2); infections, including conjunctivitis may be seen in the eyes, along with loss of
tuberculosis, histoplasmosis, and blastomycosis lacrimal gland function, causing xerophthalmia (dry eyes).
Crohns Disease
Crohns disease is a chronic autoimmune granulomatous
disease of the gastrointestinal (GI) tract, including the
mouth, with an uncertain etiology. It primarily affects
Figure 450 Multi-system involvement in sarcoidosis. teenagers and middle-aged adults. Evidence of a
Clockwise: Swelling of the lips; swelling of the salivary hereditary component to Crohns disease exists,
glands; swelling of the lacrimal glands; violaceous skin
lesions of the face; gingival enlargement. though many patients have no relatives with the dis-
order. It is also known as regional enteritis because it al-
ternately affects and skips some portions of the GI
tract, from the mouth to the anus, causing swelling and
ulceration that leads to abdominal pain, diarrhea, nau-
sea, and weight loss.
Oral signs and symptoms include diffuse nodular
swellings that produce a cobblestone appearance
(Fig. 4-53) of the buccal mucosa and nonpainful
swelling of the lips. Deep ulcers affect the colon and
may also be seen intraorally in the form of painful
major aphthous ulcers (Fig. 4-54). Some of these ul-
Figure 451 Sarcoidosis. Cutaneous red to brown cerations present with raised rolled borders and de-
plaques of the face. (From Barankin, B: Derm Notes. F.A. velop along linear folds in the buccal and labial
Davis, Philadelphia, 2006, p 141.) vestibules. Crohns disease has no cure, but symptoms
can be controlled using appropriate systemic anti-in-
When paratracheal lymph nodes along the trachea in the flammatory and immunomodulary therapy. Tumor
neck are affected, the patient may present with hoarse- necrosis factor alpha (TNF) is one crucial mediator
ness or a change in voice quality. involved in this abnormal immune response, and bio-
Intraoral lesions are rare and vary widely in appear- logical therapies targeting TNF, including inflix-
ance, from discrete papules to hyperkeratotic plaques imab, have significantly improved the management of
(Fig. 4-52). If granulomatous inflammation destroys Crohns disease.
2577_Ch04_119-162 11/07/14 6:12 PM Page 153
Orofacial Granulomatosis
Clinical Implications Orofacial granulomatosis (OFG) can affect any oral tis-
sue, but most frequently involves the lips, which
Crohns disease should be considered when a demonstrate sudden onset of unilateral or bilateral
young patient presents with major aphthous ul- nonpainful enlargement called cheilitis granulomatosum.
cers. Oral ulcers may develop before GI symp- (Fig. 4-55). Swelling often remits and recurs, with each
toms appear. episode lasting weeks or months. Eventually, lip en-
largement becomes permanent, leaving the lips with a
firm or rubbery texture. Melkerson-Rosenthal syndrome
is an unusual related condition that has as its main fea-
tures cheilitis granulomatosum, facial nerve paralysis,
and fissured tongue.
Linear hyperplastic folds that resemble epulis fissur-
atum (see Chapter 2) may develop in the buccal
and labial vestibules of patients with OFG, despite the
fact that the patient does not have dentures. These ex-
ophytic, hyperplastic folds of tissue may develop ulcers
that are mistaken for RAUs. These are clinically indis-
tinguishable from orofacial Crohns disease and
sarcoidosis, so additional diagnostic testing is necessary.
Recent studies have shown that up to 60 percent
of children with OFG have subclinical signs of Crohns
disease.
Occasionally, the etiology of OFG can be identified.
Cinnamon aldehyde and benzoate preservatives have
been implicated as triggers of OFG. Avoidance of sus-
pected triggers often brings relief. When the etiology
Figure 453 Crohns disease. Cobblestone appearance
of OFG cannot readily be identified, cortisone is the
of the buccal mucosa in a patient with Crohns disease.
most common therapy. Surgery may be required for
severe permanent swelling that interferes with esthet-
ics or oral functioning. Spontaneous remission may
occur but is rare.
Ocular Oral
Signs At Least One At Least One Histopathology Autoantibodies
Schirmer's test < 5 mm/ Unstimulated saliva Labial salivary gland Anti-SSA(Ro) or
5 min 1.5 mL /15 mins Focus Score 1 Anti-SS-B (La)
Positive vital dye staining Abnormal parotid per 4 mm 2
4 sialography
Abnormal salivary
scintigraphy
For a primary Sjgrens syndrome diagnosis:
a. Any 4 of the 6 criteria; must include either Histopathology or Autoantibodies
or
b. Any 3 of the 4 criteria for Signs
For a secondary Sjgrens syndrome diagnosis:
In patients with another well-dened major connective tissue disease, the presence of one symptom plus 2 of the 3 signs
are indicative of secondary Sjgrens syndrome.
Exclusion Criteria
Past head and neck radiation treatment
Hepatitis C
HIV/AIDS
Pre-existing lymphoma
Sarcoidosis
Graft-versus-host disease
Current use of anticholinergic drugs
Gland being
replaced by
lymphocytes Normal gland such as SLE, rheumatoid arthritis (RA), or sclero-
derma. These patients develop the same features seen
in primary Sjgrens syndrome.
The most common symptom of Sjgrens syndrome
is xerostomia and its associated problems of dysphagia
and dysgeusia. Patients with dentures may be unable
to wear them because of lack of moisture necessary to
create a seal. Patients may complain of dryness of the
eyes accompanied by a sandy or gritty feeling.
Depending on the degree of oral dryness, patients may
develop rampant dental caries, including Class V le-
sions that seemingly appear overnight (Fig. 4-59). Fab-
rication of custom fluoride trays, dietary counseling,
and reinforcement of plaque control methods are es-
Figure 458 Sjgrens syndrome. A biopsy of the minor sential for maintaining optimal oral health. Oral
salivary glands shows destruction of normal gland acini health-care products formulated for patients with xe-
with replacement by lymphocytes. rostomia are available.
2577_Ch04_119-162 11/07/14 6:12 PM Page 156
Explain why brushing with baking soda did not help. sensation experienced while wearing them. After
Is there anything that can be done to help him? Who several days of preclinical activities, you notice
should provide that help? your hands become dry, a little scaly, and some-
what itchy. You apply several different over-the-
counter hand lotions, but nothing seems to help. It
now appears you are developing a rash.
Case 4-3: Your 54-year-old female patient pres-
ents for routine care. During your initial oral inspection, What is the most likely cause for this problem? Please be
you notice a white lace-like pattern to the left and right specific in terms of the biological changes associated
buccal mucosae. The patient states that she is unaware with this suspected problem.
of the changes; there is no pain, tenderness, or reaction What two steps should you take to determine the source
when she eats hot or spicy foods. The lesions were not of the problem?
present at her last visit 9 months ago. Since that time, How do you think this experience will alter the way you
she reports she is now taking two new medicines for practice dental hygiene in the future?
hypertension. Her history also includes an allergy to
several environmental agents, including grasses.
List three possible causes for this unusual appearance to
his lower lip. What are two possible explanations for your aunts
complaints?
In addition to a biopsy of the lip performed by an oral
surgeon, what other specialists should this patient visit? What is causing the swelling of her face?
Why can she not taste food the way she used to?
How does her recent dental history relate to her new
complaints?
After dinner, she takes you aside and asks you what you
Case 4-5: You recently enrolled in your dental know about why she is getting so many new cavities.
What will you say to her?
hygiene program. During preclinical activities, you
are asked to wear gloves to get used to the tactile
2577_Ch04_119-162 11/07/14 6:12 PM Page 158
Review Questions
1. All of the following are true about the immune 7. Which one of the following is found in body
response EXCEPT: fluids, including tears and saliva?
A. The immune system cannot mistake self for A. IgG
nonself. B. IgM
B. If the immune defenses are misdirected C. IgA
they can initiate allergic disease. D. IgE
C. Microorganisms are perceived as antigens
by the immune system. 8. All of the following are true about activated T
D. Part of a microbe or toxin produced by bac- cells EXCEPT:
teria can be perceived as antigenic. A. They can remain in the body permanently
to retain the memory of an antigen.
2. Which one of the following is important in B. They can directly attack infected or cancer-
minimizing transplant rejection? ous cells
A. germinal centers C. They may direct and regulate the immune
B. MHC markers response.
C. passive antibodies D. Activated T cells release histamine, respon-
D. TCF markers sible for the symptoms of allergy.
16. In autoimmune diseases 22. Your patient has an acute extensive blistering,
A. the immune system is activated to distin- erosive disease of the oral mucosa that
guish self from nonself. erupted suddenly. There are large pruritic,
B. activated B cells produce antibodies against red, concentric circular macules on his arms
self-antigens called autoantibodies. and neck. He is in pain and cannot eat. Which
C. immunity occurs when cytotoxic T cells at- one of the following most likely precipitated
tack abnormal tissues. your patients current condition?
D. symptoms usually manifests as a type I hy- A. A recent episode of recurrent herpes labi-
persensitivity. alis
B. Recent exposure to a child with chickenpox
17. All of the following are examples of primary C. A hereditary vascular disorder
immunodeficiency diseases EXCEPT D. An HIV infection
A. selective IgA deficiency.
B. hypergammaglobulinemia.
C. DiGeorge syndrome.
D. erythroblastosis fetalis.
2577_Ch04_119-162 11/07/14 6:12 PM Page 160
C h a p t e r
5
Developmental Disorders
of the Orofacial Complex
Learning Outcomes
At the end of this chapter, the student will be able to:
5.1. Discuss how disturbances during 5.2. Explain the etiology of developmental
orofacial embryological development abnormalities of the teeth.
contribute to abnormalities of the 5.3. Explain how disturbances in tooth
head, neck and oral cavity. development affect oral health.
Continued
163
2577_Ch05_163-192 11/07/14 11:16 AM Page 164
5.4. Discuss the origins of the most com- 5.7. Recognize and describe the clinical
mon odontogenic cysts. and/or radiographic characteristics of
5.5. Explain the difference between an inflam- developmental cysts.
matory odontogenic cyst (see Chapter 2) 5.8. Explain the difference between true
and a developmental odontogenic cyst. cysts and nonepithelial cyst-like
5.6. Discuss the origins of nonodonto- entities.
genic cysts of the head and neck.
FUNDAMENTALS OF OROFACIAL rise to the neural tube and covers the interior of the
EMBRYOLOGY AND DEVELOPMENT forming primitive mouth, or stomodeum. Inside the
An understanding of embryological development primitive mouth, ectoderm becomes the oral mucosa
is useful in explaining developmental disorders. De- and eventually contributes to the formation of teeth.
velopmental disorders are defined as those that In the early stages, the mouth is separated from the in-
occur during development of a part or organ. Inter- testinal tube (primitive gut) by the buccopharyngeal
ference with development may occur that causes the (oropharyngeal) membrane, which consists of two lay-
organ or part to be too small, too large, malformed, ers: ectoderm on the mouth side and endoderm on the
or absent. The cause of interference is referred to gut side. Normally, this membrane ruptures around
as the etiologic agent. Etiologic agents in the envi- the fourth week to allow communication between the
ronment that cause abnormalities are known as mouth, throat, and gastrointestinal tract. The tonsillar
teratogens. This chapter focuses on disorders re- pillars mark the point where this membrane existed.
sulting from environmental disturbances during
development. It is important to note that the precise Development of Neural Crest Cells
cause of many developmental disorders is not clear. The frontal prominence contains the developing
Inherited developmental disorders are discussed in brain. The brain, along with the rest of the central
Chapter 6. nervous system, arises in the neural tube that extends
from the developing head toward the tail of the em-
Facial Development bryo (Fig. 5-2). The neural tube is formed by infold-
Embryology is fundamental in understanding devel- ings called neural folds, which arise from ectoderm
opmental anomalies of the face and oral cavity. The during the third week in utero. Special cells, called
origins of tissues, formation of organs and body parts, neural crest cells, develop along the lateral margins
and the timeline of their development are precise. A (crests) of the neural tube. In the head and neck
primitive head is observed in utero in the fourth week region, these cells undergo extensive migration and
of gestation, as the embryo begins to lengthen and form tissue known as ectomesenchyme. Ectomes-
bend (Fig. 5-1). The developing embryo is covered enchyme contributes to the formation of craniofacial
with a layer of tissue called ectoderm. Externally, ec- connective tissues, including bone and cartilage, der-
toderm forms the skin, its appendages (hair, sweat mis, and tissues making up the tooth, except enamel
glands), fingernails, and toenails. Ectoderm also gives (Fig. 5-3).
Developing
Frontal brain
prominence Stomodeum
Buccopharyngeal Gut
membrane
Figure 51 Human embryo, 4 weeks. Primitive
Heart Notochord head at 4 weeks, covered by ectoderm. Oropharyngeal
membrane separates primitive mouth from gut.
2577_Ch05_163-192 11/07/14 11:16 AM Page 165
Frontal
process
Primitive Medial nasal
mouth process
Maxillary Lateral nasal
process of process
first arch
Maxillary
Mandibular process of
process of first arch Figure 55 Formation of the
first arch face. Contributions of the frontal,
Mandibular
Hyoid arch process of mandibular, and maxillary processes
Heart first arch to the development of the face.
Growth of the medial nasal processes produces a palatine processes. A disturbance at this time may
wedge of tissue known as the intermaxillary segment. result in a cleft palate.
Contact and fusion of this segment with the medial as-
pect of the maxillary processes forms what is known as Development of the Tongue
the primary palate, or premaxilla (Fig. 5-6). The pri- and Thyroid Gland
mary palate eventually contains the lateral and central The tongue develops from contributions of the first,
incisors. Outgrowths from the surfaces of the maxillary second, and third pharyngeal arches, which form the
processes form two lateral palatine processes, or floor of the primitive mouth. At the fourth and fifth
shelves. At the eighth week in utero, these elevate over weeks in utero, tissue swellings from these arches
the forming tongue and, at weeks 9 to 12, fuse to form appear (Fig. 5-7). A central swelling in the floor of
the secondary palate. Fusion of these processes with the the mouth, the tuberculum impar, arises along with
primary palate completes palate formation. The mid- two lateral (lingual) swellings. The lateral swellings
palatal suture marks the line of fusion of the lateral grow rapidly and merge with the tuberculum impar
Intermaxillary
segment
Figure 56 Formation of the
palate. Fusion of premaxilla
Lateral palatine (primary palate) with the lateral
process of palatine processes to form the
maxilla palate.
A B
Figure 57 Development of the tongue. A. Pharyngeal arches that contribute to development of the tongue.
B. Mature tongue with pharyngeal arch contributions to the anterior and posterior tongue.
2577_Ch05_163-192 11/07/14 11:16 AM Page 167
to form the anterior two-thirds of the tongue. observing 700 births per group, clefts occur in one
Another midline swelling from the third arch, the birth among Caucasians, almost four births among
hypobranchial eminence, rapidly forms and fuses to Native Americans, and about two births among
form the posterior third of the tongue. The complex the Japanese. African Americans have a low rate of
contributions of more than one pharyngeal arch to 0.3 clefts per 700 births.
tongue development explain its innervation by mul- In general, clefts of the face, lip, and/or palate are
tiple cranial nerves. Failure of one or more of these thought to be multifactorial in terms of etiology.
arches to develop properly may result in a tongue Both inheritance and environmental factors are
that is too small or absent. important. Environmental factors linked to develop-
A V-shaped groove on the dorsum of the tongue, ment of clefts include drug and alcohol abuse, ciga-
called the terminal sulcus, marks the boundary be- rette smoke, chemicals such as insecticides, and
tween the posterior and anterior tongue. This sulcus microorganisms such as treponema pallidum and
terminates posteriorly in a depression called the cytomegalovirus. Cleft lip and cleft palate may occur
foramen cecum. alone or together.
The thyroid gland begins its formation at the fora- Cleft lip results from impaired fusion of the
men cecum. Thyroid-forming cells develop in this median nasal process with the maxillary process.
area and migrate along the thyroglossal tract (duct) Clefting of the lip is depicted in Fig. 5-8. It may be
to the site of the future thyroid gland in the neck. partial or complete (Fig. 5-8 B and C), unilateral or
Failure of the cells to migrate may result in ectopic bilateral (Fig. 5-8 D). It almost always involves the
thyroid gland development or formation of cysts upper lip. Clefts of the lower lip are rare. Cleft lip
along the tract. occurs early in embryonic life, between the sixth and
ninth weeks during lip formation. After birth, it ap-
ABNORMALITIES OF THE FACE pears as either a small gap or an indentation in the
AND ORAL CAVITY lip at the philtrum. A partial cleft lip involves only
Defects present at birth or shortly after birth are re- the lip, but a complete cleft will involve the lower
ferred to as congenital abnormalities. Some con- portion of the nose. In decades past, the condition
genital abnormalities are not observed until the was sometimes referred to as harelip, but that term
development of a body part is complete. Teratology is no longer used.
can be broadly defined as the study of developmental
abnormalities, both congenital and developing after
birth. Inherited mechanisms and/or agents called
teratogens interfere with embryonic development,
resulting in developmental malfunctions or defects.
The precise etiology of many developmental defects
is unknown; however, some well-known examples of
environmental teratogens include drugs (thalidomide,
alcohol), radiation (x-rays), and microorganisms
(rubella, syphilis). Teratogens often affect forming
tissues only at specific stages of development. A Normal lip B Partial cleft lip
Clinical Implications
Figure 513 Macroglossia. Large tongue that overfills
the mouth with scalloping of borders from pressure on Lingual thyroid is most often asymptomatic, an
teeth. incidental nding, and requires no treatment. In
unusual cases, it can interfere with normal
(Fig. 5-14). Fissured tongue may be seen in both swallowing and breathing, which necessitates re-
children and adults, and its prevalence in the general moval. However, because removal of a lingual thy-
population has been estimated at around 5 percent. roid may result in hypothyroidism (see Chapter 9),
Fissured tongue has been associated with geographic the presence of adequate thyroid gland tissue in
tongue (see Chapter 2), with many patients having the neck must be carefully evaluated.
both conditions. Fissured tongue is a component of
Melkersson-Rosenthal syndrome (see Chapter 4).
Food particles and oral debris may become trapped in Lingual Varicosities
the deep crevices of a fissured tongue, creating an oral Varicosities (varicose veins) are swollen, twisted, and
hygiene problem. Oral health-care procedures that sometimes painful veins that have filled with an
address this issue include cleaning the tongue by abnormal collection of blood. They are seen most
brushing and rinsing. commonly on the legs. Lingual varicosities are sim-
ilar in appearance to varicose veins in the legs. They
present as nonpainful, large, dilated, blue masses
on the ventral surface of the tongue (Fig. 5-16)
and are more common in older individuals, with
few if any seen in children and adolescents. Most
FUNDAMENTALS OF TOOTH
DEVELOPMENT
Tooth development begins at approximately the sixth
week in utero and ends with the eruption of the third
molars. Teeth are vulnerable to deleterious environ-
Figure 516 Lingual varicosities. Tortuous veins on mental influences during this time. Ectoderm covering
ventral tongue. the external surface of the embryo and lining the prim-
itive mouth as oral mucosa, is vital for tooth develop-
lingual varicosities are asymptomatic and require no ment. Around the sixth week in utero, two horseshoe
treatment. bands of thickened epithelium called dental lamina
appear over the upper and lower alveolar ridges
Hemifacial Microsomia (Fig. 5-18A). Ten localized epithelial buds in the max-
illa and ten in the mandible extend downward into
Hemifacial microsomia is a congenital condition in which
the underlying connective tissue to form germs of the
one-half of the face is underdeveloped (Fig. 5-17). Also
primary (deciduous) teeth (Fig. 5-18B). They are teth-
called Goldenhar syndrome, or Oral-Mandibular-
ered to the overlying epithelium by the dental lamina.
Auricular syndrome, it is the second most common
Lingual outgrowths from these epithelial buds will
birth defect after facial clefting. The affected struc-
form 20 successor (succedaneous) or permanent teeth.
tures include the mouth, mandible, and ears. The
Twelve additional permanent tooth buds develop from
condition is believed to arise around the fourth
continued distal growth of the dental lamina.
embryonic week due to a decreased vascular supply
to the affected region that compromises growth.
Enamel Organ
Bud, cap, and bell stages comprise the earliest tooth
development. Epithelial buds grow down into the un-
derlying ectomesenchyme to form a cap shape which
further proliferates into the shape of a bell (Fig. 5-18C).
This structure is called the enamel organ. Each enamel
organ is composed of inner and outer enamel epithelium,
with intervening stellate reticulum and stratum inter-
medium. The inner enamel epithelium later differenti-
ates into ameloblasts that will form enamel.
Rests of Serres
At the end of the bell stage, the dental lamina is no
longer needed, so it degenerates. Small fragments of
epithelium, referred to as rests of Serres (Fig. 5-18D),
are left behind. Later in life, these residual fragments
of epithelium may develop into cysts.
Developing
maxillary arch
Developing
dental lamina Dental
lamina
Stomodeum
Developing
mandibular arch
Tooth bud
(germ)
Oral epithelium
forming a tooth bud
A B
Surface ectoderm
Dental
lamina
Rests of
Serres
Enamel
organ
Dental
lamina
C D
Figure 518 Fundamentals of tooth development. A. Dental lamina in bud stage. B. Formation of the
dental laminas umbilicord-like structure. C. Bell stage. D. Breakdown of the dental lamina and formation of
the Rests of Serres.
reduced enamel epithelium. This forms a covering that Eventually, the root sheath breaks apart, allowing ce-
protects the enamel surface during tooth eruption. mentum formation to proceed along the root surface.
Ameloblasts
Odontoblasts
Outer enamel
epithelium
Inner enamel
epithelium
Cementoenamel
junction
Hertwigs
epithelial
A root sheath Figure 520 Hypodontia. Congenitally missing
premolars and molars.
Enamel
associated with abnormalities of ectoderm. Because
teeth arise from ectoderm, their formation is affected
Dentin in these syndromes.
Cementoenamel
Clinical Implications
junction Congenitally missing teeth may not be apparent
Rests of
Malassez until radiographic evaluation reveals the failure
of tooth formation. Depending on the number
and location of missing teeth, clinical implica-
Hertwigs
B root sheath tions may include aesthetics, speech, and masti-
Figure 519 Hertwigs epithelial root sheath. A. Hertwigs cation of food. Interdisciplinary management of
root sheath is made up of cells of the inner enamel and anodontia or hypodontia often incorporates the
outer enamel epithelium. B. Rests of Malassez are specialties of pediatric dentistry, orthodontics,
remnants of Hertwigs root sheath when it breaks down and prosthodontics.
during root formation.
development, most common tooth anomalies are the Hyperdontia (Supernumerary Teeth)
result of local or systemic factors that affect the devel- Extra teeth are called supernumerary teeth. They occur
oping teeth. when extra tooth buds develop from dental lamina, or
when tooth buds erroneously divide. There may be
Congenitally Missing Teeth one or many supernumerary teeth present in the den-
Variations in the number of teeth may occur. Failure tition (Fig. 5-21). The most common supernumerary
of all teeth to form is known as anodontia. Failure of tooth is the mesiodens, which occurs in the midline
some teeth to form, leaving a fewer than normal num- between the maxillary central incisors and often has a
ber, is termed hypodontia or oligodontia. Hypodontia can conical or peg shape. A paramolar is a supernumerary
range from one to multiple missing teeth (Fig. 5-20). tooth lying lingual to, or buccal to, maxillary or
It may affect either the permanent or primary denti- mandibular molars (Fig. 5-22). Supernumerary teeth
tions, but is more common in the permanent dentition. may block other teeth from erupting successfully.
The most common congenitally missing permanent Crowding of developing teeth may impinge on the
teeth are the third molars, maxillary lateral incisors, tooth germ or Hertwigs epithelial root sheath and
and mandibular second premolars. Anodontia or hy- result in altered crown and root shapes. Supernumer-
podontia may be familial or inherited as a part of ary teeth are commonly impacted. They may be seen
a syndrome. Ectodermal dysplasia (see Chapter 6) in inherited disorders, such as cleidocranial dysplasia
describes a group of inherited syndromes that are or Gardners syndrome (see Chapter 6).
2577_Ch05_163-192 11/07/14 11:17 AM Page 174
Mesiodens
B
Paramolar
Figure 523 Fusion. A. Clinical photo of child with
fusion of laterals and canines bilaterally. B. Radiograph of
Figure 522 Common examples of microdontia. same child.
Mesiodens and paramolar.
Peg Lateral
Peg lateral describes an alteration in the shape of max-
illary lateral incisors that causes the lateral incisor
to have a conical shape. Peg laterals are smaller than
normal laterals and are considered microdonts. One
or both laterals may be affected (Fig. 5-27). Peg lat-
erals may require restoration to improve aesthetics.
Talon Cusp
An extra cusp on the lingual or palatal surface of an
anterior tooth in the cingulum area is known as talon
cusp (Fig. 5-28). This occurs as an extra outgrowth of
the enamel organ during crown formation. The cusp
is often triangular-shaped and thought to resemble
the talon of an eagle. Talon cusps are susceptible to
wear and fracture and contain an extension of pulp.
Noncarious or restorative pulp exposures may occur,
resulting in pulpal necrosis and apical pathology.
Endodontic treatment may be necessary.
Figure 525 Concrescence. Multiple teeth joined by
cementum.
Dens Evaginatus
Dens evaginatus is formation of a supernumerary cusp,
most often on a posterior tooth. It may occur unilaterally
or bilaterally on any tooth, most frequently on the oc-
clusal surface of premolars (Fig. 5-29). An extension of
pulp is usually present within the extra cusp. There is in-
creased risk of pulp exposure with cusp wear or fracture.
This can result in pulpitis, pulpal necrosis, and apical
pathology. Endodontic treatment may be required.
Enamel Pearl
Enamel pearl most commonly occurs as a round concre-
tion, resembling a small pearl, in or adjacent to the fur-
cation area of the maxillary molars (Fig. 5-31). These
are often first detected on radiographic examination as Figure 531 Enamel pearl. Group of molars showing
enamel pearls.
Dilaceration
Dilaceration is a condition in which a tooth root ex-
hibits abnormal curvature (Fig. 5-32). This may occur
Figure 529 Dens evaginatus. Cusp-like protrusion in anywhere along the root, from the cemento-enamel
central groove of mandibular premolar. junction to the apex. Dilaceration is thought to result
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Supernumerary Roots
Supernumerary or extra tooth roots arise from addi-
tional extensions of Hertwigs root sheath. The exact
cause is unknown. They are most often an incidental
finding on radiographic examination, but may be dif-
ficult to detect. Supernumerary roots may occur on
any tooth, but tend to be more common in the
Figure 532 Dilaceration. Abnormal curvature of the
apical one-third of the root. mandibular and maxillary third molars (Fig. 5-34).
They may complicate endodontic therapy or tooth ex-
traction, but otherwise cause no problems.
from trauma to Hertwigs root sheath, causing dis-
placement of forming tissues. Dilacerated teeth are not
usually a significant finding, unless they pose problems Abnormalities of Tooth Color
during tooth extraction or endodontic treatment. Coloration of teeth may be inherited as well as influ-
enced by environmental factors. Extrinsic stain or
Taurodontism exogenous stain on external surfaces of the teeth may
Taurodontism is an anomaly of molar teeth caused by al- be due to exposure to foods such as tea and coffee;
teration of Hertwigs root sheath. Clinically, it may not vitamins, such as iron; and habits, such as smoking
be apparent and is often an incidental finding on or chewing tobacco. Extrinsic stain can usually be
radiographic examination. Taurodontism is character- removed. Over time, stains may become incorporated
ized by an increase vertically in the size of the body within cracks, crevices, and defects of enamel. Intrinsic
(crown) of the tooth at the expense of the roots. Pulp (internal) staining, also known as endogenous staining,
chambers appear elongated, with short, pointed roots is incorporated during tooth development. Endoge-
located in the apical one-third of the tooth (Fig. 5-33). nous stains may result from ingested medications or
The prefix tauro- means bull. The teeth are named systemically produced pigments.
after their similarity to bovine teeth, which typically
have large crowns and short roots. Taurodontism is Tetracycline Stain
Tetracycline is a broad-spectrum antibiotic that chelates
calcium ions and becomes incorporated within any
Figure 533 Taurodont. Mandibular molars showing Figure 534 Supernumerary root. Maxillary molar with
increase in size of pulp chamber with small roots. five roots.
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Eruption cyst Tooth follicle and reduced enamel Bluish swelling in gingiva over erupting tooth; no
epithelium radiographic features
Gingival cyst (also called Rests of Serres Bluish swelling of gingiva over root surface; no radi-
dental lamina cyst) ographic features
Nasopalatine duct cyst Nasopalatine duct of the incisive Heart-shaped radiolucency between
(also called incisive canal maxillary central incisors; often intraoral
canal cyst) swelling
Median palatine cyst Remnants of fusion of palatine Radiolucency along midline of palate; often
processes intraoral swelling
Globulomaxillary cyst Remnants of fusion between pre- Unilocular radiolucency between maxillary
maxilla and lateral palatine processb lateral and canine
vs. odontogenic epithelium
Extraosseous
Nasolabial cyst Remnants of fusion of lateral nasal Soft tissue swelling along nasolabial fold
and maxillary processes during lip
formation
Thyroglossal duct cyst Remnants of the thyroglossal duct or Soft tissue swelling in midline of neck
tract, route of migration of thyroid above thyroid cartilage; may move
gland upon swallowing
Cervical lymphoepithelial cyst Unknown; remnants of developing Soft tissue swelling of the lateral neck
lymphoid tissue within developing
salivary gland within the upper
neck
Oral lymphoepithelial cyst Remnants of developing lymphoid Small white or yellow nodules of oral
tissue entrapping surface epithelium in Waldeyers ring
epithelium
Epidermal inclusion cyst Surface epithelial remnants Small white or yellow nodules of oral
inadvertently implanted within epithelium in any oral location
the submucosa
Epidermoid cyst Hair follicle epithelium Dome-shaped soft tissue swelling of
facial skin
Dermoid cyst Surface skin entrapped in submu- Soft tissue swelling of the midline floor of
cosal tissues the mouth
aNonodontogenic cysts arise from tissue in the orofacial region, not including tooth tissues.
bSource is disputed. Most globulomaxillary cysts are other odontogenic cysts (Table 5-1).
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Other non-epithelial-lined spaces and anatomical embedded within alveolar bone, the cyst is referred to
defects that mimic cysts are found in the jaws; how- as a dentigerous cyst. Radiographically, a well-defined
ever, these are not true cysts because they lack an unilocular radiolucency is present around the crown of
epithelial lining. They are best called pseudocysts. an unerupted tooth, attached at the cemento-enamel
Table 5-3 lists several common pseudocysts encoun- junction (Fig. 5-40A and B). These occur most fre-
tered in the jaws. quently around impacted third molars, maxillary ca-
nines, and mandibular second premolars. Treatment is
Odontogenic Cysts
Odontogenic cysts arise from epithelial remnants
of the developing tooth germ, called rests. Rests of
Serres are epithelial remnants of dental lamina that are
found in the gingiva. Rests of Malassez are remnants of
Hertwigs epithelial root sheath that are found in the
periodontal ligament space. Epithelial cells in these rests
multiply when they are stimulated, most often by the
inflammatory response. They multiply to form small
clusters of epithelium around a center that attracts fluid
from adjacent connective tissues. This results in a cystic
sac that gradually expands as fluid accumulates. For rests
of Serres, the pressure from the growing cyst will cause
a visible soft tissue swelling of the gingiva. For rests of
Malassez, the pressure from the growing cyst will cause
resorption of adjacent bone that appears radiographi-
cally as a well-defined radiolucency. This section in-
cludes a brief discussion of the commonly encountered
odontogenic cysts in the oral cavity.
A
Follicular Cysts: Dentigerous Cyst
and Eruption Cyst
There are two types of follicular cysts. Both occur when
the reduced enamel epithelium surrounding the crown
of an unerupted tooth (the follicle) becomes separated
from the enamel surface and fluid accumulates within
the newly formed space (Fig. 5-39). If the tooth is
Clinical Implications
Dentigerous cysts may become very large in size
due to continued expansion. They are capable of
resorbing surrounding bone and/or adjacent Figure 541 Eruption cyst. Molar under soft tissue
tooth roots. The diagnosis of dentigerous cyst has failed to erupt. Note the bluish appearance to the
cannot be made until the cyst is removed and soft tissue.
examined microscopically. Other pathological
conditions, such as odontogenic tumors, may
mimic dentigerous cysts radiographically. Re- mandible. They only occur within bone. OKCs are
moval of the aected tooth and/or cystic tissue well-defined unilocular (Fig. 5-42) or multilocular ra-
is the usual treatment. diolucencies that may displace teeth or resorb tooth
In most cases of eruption cysts, the tooth even- roots. Radiographically, their location may suggest
tually breaks through the overlying tissue and no dentigerous, primordial, or other odontogenic cysts or
treatment is necessary. If the cyst and/or dense tis- tumors. Microscopically, the epithelial lining of OKCs
sue continue to hinder eruption, an opening may is uniquely parakeratinized (Fig. 5-43). Tiny satellite
be created surgically to allow the tooth to erupt. cysts arising away from the main cyst lining make
OKCs difficult to eradicate. These cysts have a much
higher recurrence rate than any other cyst. Their
aggressive behavior and propensity for recurrence
Primordial Cyst
Primordial cysts are believed to arise from degenera-
tion of tooth germs during embryological development.
Remnants of the enamel organ are thought to be the
source of the epithelial lining. Primordial cysts are
located in the space where a tooth should have formed.
Clinical history is most often that of a missing tooth that
never developed. The posterior mandible, especially the
third molar region, is the most common site. Radi-
ographic appearance is that of a unilocular, well-defined
radiolucency in place of a tooth. Primordial cysts most
frequently turn out to be odontogenic keratocysts,
discussed next.
Odontogenic Keratocyst
Odontogenic keratocysts (OKCs) occur anywhere in the Figure 542 OKC. A large, well-corticated radiolucent
jaws, but are most commonly found in the posterior lesion.
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Clinical Implications
Multiple OKCs have been associated with the ge-
netically linked nevoid basal cell carcinoma syn-
drome. Individuals with this syndrome have been
reported to develop an initial OKC at an early
age and then multiple cysts throughout their life-
time. See Chapter 6 for more details on identify-
Figure 545 Gingival cyst. Soft fluctuant mass on facial
ing this syndrome.
surface at mucogingival junction. Note bluish coloration.
Lateral Periodontal Cyst cysts are treated surgically and are not known to
Lateral periodontal cysts arise in the periodontal liga- recur.
ment space from the rests of Malassez, most frequently
in the mandibular premolar area. Pressure causes re- Calcifying Odontogenic Cyst
sorption of alveolar bone lateral to the tooth (Fig. 5-44). Calcifying odontogenic cysts, also known as Gorlin cysts, are
Radiographically, they appear as small unilocular ra- most common in the second to third decades of life,
diolucencies between premolars. Lateral periodontal but can be seen at almost any age. Radiographically,
cysts are treated surgically and rarely recur. this cyst differs from other odontogenic cysts in that it
has a mixed radiolucent/radiopaque appearance caused
Gingival Cyst by irregular calcifications within the cyst lining. Other
Gingival cysts are located in soft tissues surrounding odontogenic tumors and cysts may need to be ruled out
the teeth, most often on the facial gingiva (Fig. 5-45). depending on the location and appearance. Surgical
Believed to arise from the Rests of Serres, they are excision is curative and recurrence is not expected. This
often referred to as dental lamina cysts. Gingival cysts cyst is not found as a part of a syndrome.
may be seen in newborns at or shortly after birth, or
in adults. They are typically small, fluctuant, and Buccal Bifurcation Cyst
may have a bluish tint. Because they do not involve Buccal bifurcation cysts are most commonly associated
bone, they do not appear on radiographs. Gingival with the mandibular first molar. They are most likely
2577_Ch05_163-192 11/07/14 11:17 AM Page 184
Nonodontogenic Cysts
During embryonic and fetal development, numerous
processes must meet and fuse. Small fragments
of residual ectoderm at these points of fusion may
remain trapped within the underlying connective
tissues. These embryonic rests can be stimulated in
the same fashion as the rests of Malassez and Serres
to form cysts. The resulting developmental cysts are
unrelated to tooth development and are referred to
as nonodontogenic cysts. Below are some of the
more common nonodontogenic cysts that occur in
the head and neck.
Lingual thyroid
Thyroglossal duct
Hyoid bone
Thyroglossal duct
cyst attached to the
hyoid bone
Thyroid cartilage
Thyroid gland
Review Questions
1. Clefts are considered the most common devel- 5. Hemifacial microsomia
opmental facial abnormality. They occur dur- A. rarely develops before embryonic week 16.
ing embryonic life between weeks B. is the most common birth defect in the
A. one to three. head and neck.
B. three to five. C. commonly presents as underdevelopment
C. six to eleven. of one-half of the face.
D. twelve to twenty-four. D. has few cosmetic implications for the patient.
2. Tethering of the anterior tongue to the floor 6. Which one of the following is the most com-
of the mouth is called mon supernumerary tooth?
A. ankyloglossia. A. Maxillary lateral incisor
B. aglossia. B. Mesiodens
C. microsomia. C. Maxillary third molar
D. macroglossia. D. Mandibular central incisor
3. Which one of the following is often associated 7. In which one of the following is less than the
with Down syndrome? normal number of teeth present?
A. Commissural lip pits A. Fusion
B. Hemifacial microsomia B. Gemination
C. Lingual thyroid C. Peg lateral
D. Macroglossia D. Mesiodens
9. The most important dental complication of 15. The most useful clinical feature for determin-
both dens invaginatus and evaginatus is ing the type of cyst is
A. pulp exposure and subsequent apical A. size.
pathology. B. location.
B. aesthetics and malocclusion. C. duration.
C. increased incidence of gingivitis and peri- D. degree of discomfort or pain.
odontal disease.
D. calculus and dental plaque retention. 16. Cysts with an origin related to tooth develop-
ment are termed?
10. Radiographically, enamel pearls may be mis- A. Intraosseous
taken for B. Extraosseous
A. caries. C. Odontogenic
B. internal resorption. D. Pseudocysts
C. tori.
D. calculus. 17. Which cyst is always located around the
crown of an unerupted tooth?
11. Which one of the following conditions is A. Dentigerous
characterized by an elongated pulp chamber B. Primordial
and short, pointed roots? C. Odontogenic keratocyst
A. Dens-in-dente D. Lateral periodontal
B. Macrodontia
C. Taurodontism 18. A firm, pink or bluish swelling over an erupt-
D. Dens invaginatus ing tooth best describes which cyst?
A. Dentigerous
12. The union of one or more teeth by cementum is B. Primordial
A. fusion. C. Odontogenic keratocyst
B. concrescence. D. Eruption
C. gemination.
D. ankylosis. 19. A patient with multiple odontogenic kerato-
cysts should be evaluated for
13. Which one of the following conditions devel- A. cleidocranial dysplasia.
ops in utero and results in intrinsic staining of B. Gardners syndrome.
the primary dentition? C. nevoid basal cell carcinoma syndrome.
A. Contemporaneous enamel hypoplasia D. contemporaneous enamel hypoplasia.
B. Fluorosis
C. Turner tooth 20. Which one of the following cysts may occur
D. Erythroblastosis fetalis anywhere from the foramen cecum to the
suprasternal notch?
14. Enamel hypoplasia affecting the facial surface A. Static bone
of multiple, anterior permanent teeth in a B. Simple bone
band-like pattern is known as C. Thyroglossal tract
A. contemporaneous enamel hypoplasia. D. Oral lymphoepithelial
B. fluorosis.
C. Turner tooth.
D. erythroblastic enamel hypoplasia.
2577_Ch05_163-192 11/07/14 11:17 AM Page 191
C h a p t e r
6
Disorders of Genetics
and Inheritance
Basic Principles of Genetic and Inherited Disorders Inherited Disorders Limited to the Dentition
Basics of Genetics Disorders of Enamel: Types of Amelogenesis
Modes and Patterns of Gene Transmission Imperfecta
Syndromes Hereditary Disorders of Dentin Formation
Genetic and Inherited Disorders of the Head Disorders of Cementum: Odontohypophosphatasia
and Neck
Disorders of the Periodontium/Gingiva
Disorders of the Jaws and Facial Structures
Disorders of the Oral Soft Tissues
Learning Outcomes
At the end of this chapter, the student will be able to:
6.1. Define DNA, chromosome, genes, 6.7. Recognize the principal features of
and alleles. hereditary gingival enlargement.
6.2. Compare autosomal versus 6.8. Recognize and describe the princi-
x-linked modes of genetic disease pal features of the craniosynostosis
transmission. syndromes and Treacher-Collins
6.3. Differentiate between dominant and syndrome.
recessive modes of transmission. 6.9. Explain why early detection in the
6.4. Define genetic mutations and dental office may be crucial in hered-
chromosomal damage. itary hemorrhagic telangiectasia,
6.5. Discuss carrier state, incomplete Gardners syndrome, Cowden syn-
penetrance, and variable expressivity. drome, and Peutz-Jeghers syndrome.
6.6. Define the term syndrome; recognize 6.10. Recognize and describe the princi-
the major features of Papillion- pal features, including mode of
Lefvre syndrome and describe its transmission, for the bone disorders
effects on the periodontium. osteogenesis imperfecta, osteopetrosis,
Continued
193
2577_Ch06_193-226 11/07/14 11:19 AM Page 194
fibrous dysplasia, cherubism, and 6.13. Recognize and describe the principal
cleidocranial dysplasia. features of the three forms of amelo-
6.11. Describe the principal features genesis imperfecta.
of nevoid basal cell carcinoma 6.14. Recognize and describe the two
syndrome. inherited disorders of dentin,
6.12. Recognize and describe the princi- dentinogenesis imperfecta and
pal features of disorders of the oral dentin dysplasia.
soft tissues, including white sponge 6.15. Describe the dental problems
nevus and hereditary benign in- experienced by children with
traepithelial dyskeratosis. odontohypophosphatasia.
Basics of Genetics
Deoxyribonucleic acid (DNA) is the material in
most organisms that transmits inheritance. All cells
of a given individual have the same DNA, which is
located in the cell nucleus. DNA can replicate, or
make copies of itself. Strands of DNA are arranged
in a double-helix formation that serves as a pattern DNA
for duplication, so inherited characteristics can be p arm
passed on to new generations of cells (Fig. 6-1).
Newly formed cells should contain an exact copy of Centromere
DNA from the parent cell.
Chromosomes q arm
1 2 3 4 5
6 7 8 9 10 11 12
13 14 15 16 17 18
gene is expressed, or becomes operational. Several may be the same (homozygous) or different (heterozy-
modes of inheritance are discussed here. gous). If one parent has blue eyes and the other has
green eyes, and the childs eyes are blue, then the allele
Autosomal Versus X-Linked Disorders on the gene from the parent with the blue eyes was ex-
Twenty-two pairs of chromosomes, out of the normal pressed. Another term for this is dominant. The allele
23 pairs, are called autosomes. They appear the same that was not expressed, the green eye allele, was re-
in both males and females. The twenty-third pair, the cessive. When both parents pass on a recessive allele,
sex chromosomes, differs between males and females. then the offspring has that allele. For example, if both
Females have a pair of X chromosomes, whereas males of your parents have green eyes, then most likely your
have one X and one Y chromosome. When an allele for eyes are also green because there is no blue allele to
a disorder is carried on an autosomal chromosome, it is dominate the green allele and block it from expressing
referred to as an autosomal disorder. If the allele is carried itself. The term express in genetics indicates a process
on a sex chromosome, it is referred to as an X-linked or that takes inherited information in genes to make a
Y-linked disorder. Y-linked disorders are exceptionally specific functional product. Alleles rather than genes
rare, whereas X-linked are more common. are the main reason each of us is unique.
In disease, only one copy of a gene is required for a
Dominant Versus Recessive person to be affected by an autosomal dominant disorder.
Genes come in pairs. A pair of genes from each parent Each affected person usually has one affected parent;
participates in determining a trait such as eye color. therefore, there is a 50 percent chance that a child will
There are two alleles for each inherited trait. These inherit the mutated gene (Fig. 6-4).
Autosomal dominant
Chromosome with
allele highlighted
Affected
Unaffected
Autosomal recessive disorders require two recessive disorder has a 50 percent chance of having
copies of the disease allele for a person to be affected. sons who are affected and a 50 percent chance of hav-
Affected individuals usually have unaffected parents ing daughters who carry one copy of the mutated al-
who each carry a single copy of the disease allele. lele, and are therefore carriers (Fig. 6-6).
The parents are referred to as carriers. Two unaffected Carriers are seen mainly in recessive disorders.
people who each carry one copy of the disease (mu- These individuals have inherited a genetic trait or
tated) allele have a 25 percent chance with each preg- mutation but do not show symptoms of the disorder.
nancy of having a child affected by the disorder. They are able to pass the gene on to their offspring,
At conception, each sibling of an affected individual who may then express the gene (have the disorder).
has a 25 percent chance of being affected, a 50 percent The disease appears only when two carriers have
chance of being an asymptomatic carrier, and a children. Each child has a 25 percent chance of having
25 percent chance of being unaffected and not a the disease. Examples of disorders involving carriers
carrier (Fig. 6-5). are hemophilia and sickle cell disease.
X-linked recessive disorders are caused by muta- X-linked dominant disorders are very rare and are
tions in genes on the X chromosome. Males are more caused by mutations in genes on the X chromosome.
frequently affected than females. The sons of a man Males and females are both affected in these disorders,
with an X-linked recessive disorder will not be affected with males typically being more severely affected than
because their X chromosome comes from their females. Some X-linked dominant conditions are fatal
mother. All daughters will carry one copy of the mu- in males either in utero or shortly after birth, and are
tated allele. A woman who is a carrier of an X-linked therefore predominantly seen in females.
Autosomal recessive
Affected
Unaffected
Carrier
Unaffected son Carrier Carrier son Affected Figure 65 Pattern of autosomal recessive inheritance
daughter daughter with identification of carriers.
2577_Ch06_193-226 11/07/14 11:19 AM Page 198
X Y X X
Affected
Unaffected
Carrier
X Y X X X X X Y
Unaffected son Unaffected Carrier Affected son Figure 66 X-linked recessive disorder pattern of
daughter daughter inheritance with identification of carrier.
words, genotype is an individuals genetic composi- with short fingers; white spots on the colored part of
tion. An individuals phenotype is all of its visibly the eye (Brushfield spots); impulsive behavior; short
observable characteristics that are created by the attention span; and slow learning. One must wonder
genes codes. In other words, phenotype is the ob- how such a widely varied and seemingly unrelated
servable appearance. For example, when the genotype group of symptoms all arise together from one altered
melanocortin-1 receptor (MC1R) is found on chro- chromosome.
mosome 16, the phenotype is red hair. There are hundreds of syndromes, only some of
which will be discussed here. Many syndromes are
Mutations eponymous, which means they were named after the
A mutation is a permanent change in the DNA person who first described them. For example, Gorlin
sequence that makes up a gene. Mutations may occur syndrome is named after Dr. Robert Gorlin, who
before birth or after birth. Before birth, there are helped describe this and many syndromes involving
two ways mutations can occur. They may be inher- the head and orofacial structures.
ited from a parent at the time of fertilization of egg Genetic disorders rarely have effective treatments,
and sperm. These are called germline mutations and though gene therapy is being tested as a possible treat-
are present throughout a persons life in all cells. ment. Gene therapy is a technique for correcting
Mutations may occur just after fertilization, often as defective genes responsible for disease development.
a result of exposure to a teratogen. Teratogens are Researchers may use one of several approaches for
agents that may disturb the development of an em- correcting faulty genes, such as inserting a normal
bryo or fetus. Examples of teratogens include radia- gene to replace a nonfunctional gene, swapping the
tion, maternal infections, chemicals, and drugs. abnormal gene for a normal gene, repairing the abnor-
These may produce what are called new (de novo) mal gene, or turning the abnormal gene off. Gene
mutations. De novo mutations may explain genetic therapy is currently not standard of care, but is still
disorders in which an affected child has a mutation in experimental stages.
but has no family history of the disorder because it
was not carried in either the sperm or the ovum.
Another type of mutation that occurs after birth is GENETIC AND INHERITED DISORDERS
called acquired or somatic. Environmental factors, such OF THE HEAD AND NECK
as ultraviolet radiation from the sun, chemical expo- Genetic and inherited malformations of the head and
sure, or radiation exposure, cause damage to the DNA neck are common, with approximately 2 percent of in-
that is then carried on in each subsequent cell division. fants being born with abnormalities that usually arise
Also, if a mistake is made as DNA copies itself during during fetal life. Many of these malformations can be
cell division, an acquired mutation may occur. Cells attributed to defects in genes that regulate the forma-
carry a toolkit of DNA repair enzymes, but if the tion of the head, neck, and oral structures. Several
damage overwhelms the cells ability to repair itself, hundred of these conditions exist. In this section, the
the mutation results. Acquired mutations cannot be focus will be on some disorders of periodontium, jaws,
passed on to offspring. facial structures, and oral soft tissues.
A B
Figure 68 Hereditary gingival overgrowth. A. Child with massive overgrowth of the gingiva. B. The tissue
regrew following gingivectomy.
hereditary gingival enlargement. The clinical condition It is postulated that the gingival lesions result from dam-
generally requires repeated periodontal surgical proce- age to tissue from hydrogen peroxide generated by or-
dures throughout the patients lifetime to manage ganisms in gingival plaque. The hydrogen peroxide
continued gingival enlargement. produced by plaque microorganisms cannot be degraded
by leukocytes genetically lacking the enzyme catalase.
Acatalasia Early tooth loss results from periodontal destruction.
Acatalasia is a rare hereditary metabolic autosomal
recessive disorder caused by the lack of an enzyme called Clinical Implications
catalase. This enzymes job is to assist in the decompo-
Patients with acatalasia often have extensive oral
sition of hydrogen peroxide, a harmful byproduct of
ulcerations. Because these occur in childhood, it
many normal metabolic processes. Catalase converts
often is interpreted as a form of major aphthous
hydrogen peroxide into harmless water and oxygen.
ulcers (Chapter 4) or other immune deciency.
Without this enzyme, hydrogen peroxide builds up and
Loss of periodontal bone and tooth support is not
destroys tissues. Though usually mild and asymptomatic,
a feature of major aphthous ulcers, so this can be
about half of the affected persons have recurrent infec-
used to help dierentiate between the two.
tions of the gingiva that may lead to gangrenous lesions.
2577_Ch06_193-226 11/07/14 11:19 AM Page 202
*More than 200 recognized syndromes may include cleft palate as a manifestation.
Clinical Implications
The clinical alterations of cherubism typically
progress until puberty, after which they stabilize
and in some patients even show regression.
For this reason, surgical treatment is typically
delayed until puberty. Surgical curettage of the
lesions is usually the preferred treatment. By age
30, facial abnormalities are no longer apparent;
residual jaw deformity is rare.
Rarely, cherubism occurs as part of another
genetic disorder. For example, cherubism can
occur with Ramon syndrome, which also involves
short stature, intellectual disability, and gingival
brosis. In addition to the features of cherubism,
patients with Noonan syndrome have short
stature and heart defects.
Figure 615 Cherubism. A 3-D reconstruction of
destructive jaw lesions.
Cleidocranial Dysplasia
Cleidocranial dysplasia is an autosomal dominant
condition associated with mutations in the Runx2
gene, which produces a protein required for osteoblast
differentiation. This condition is characterized by
bone defects involving the clavicle (cleido-) and skull
(-cranial). Clavicles are absent or underdeveloped and
barely functional. The ability of the patient to touch
the shoulders together is pathognomonic of cleidocra-
nial dysplasia (Fig. 6-16). Cranial defects in these typ-
ically short patients include a relatively large head,
ocular hypertelorism (wide spacing between eyes),
Figure 613 Cherubism. Multiple unerupted teeth. and widened base of the nose with depressed nasal
bridge. The large head is because of parietal bossing,
causing the head to be wider from ear to ear because
of delayed closure of the sutures of the skull.
Craniosynostosis Syndromes
Craniosynostosis is a group of syndromes that
are characterized by premature closure of cranial
(cranio-) sutures (-synostosis). The two types discussed
here are Crouzon syndrome and Aperts syndrome.
These syndromes are caused by mutations in a specific
gene called the fibroblast growth factor receptor gene
(FGFR). The type and severity of disease is dependent
Figure 617 Cleidocranial dysplasia. Multiple impacted on the mutation within the FGFR gene.
teeth in the anterior mandible.
Head shapes in craniosynostosis include brachy-
cephaly, scaphocephaly, trigonocephaly, ranging
Oral manifestations include high-arched palate to the most severe form of cloverleaf skull
with increased occurrence of clefting and presence (kleeblattschdel) (Fig. 6-18).
of numerous unerupted permanent and supernumer- Crouzon syndrome is an autosomal dominant syn-
ary teeth (Fig. 6-17). The teeth have distorted crown drome affecting 1 in 65,000 births. There is wide vari-
and root shapes and lack secondary cementum, ation in clinical presentation. When cranial sutures
Brachycephaly
Cloverleaf
kleeblattschdel
Normal
B
Treacher-Collins Syndrome
Treacher-Collins syndrome, also called mandibulofacial
dysostosis, is an autosomal dominant disorder that
affects 1 in 25,000 to 50,000 births, with more than
50 percent representing new mutations. This disorder
is caused by mutations in the Treacher-Collins-
Franceschetti syndrome 1 (TCOF1) gene. The gene en-
codes a protein that, when missing, allows for defects
in first and second branchial arches related to improper
migration of neural crest cells. Patients have a nar-
row face due to hypoplasia of the zygomatic bone
(Fig. 6-21A and B). A downward slant of the palpebral
fissures and a notch in the lateral aspects of the lower
eyelid, called coloboma, are noted (Fig. 6-21C). Most C
patients have external ear defects, including soft tissue Figure 619 A. Patient with mid-face deficiency.
ear tags, as well as hearing loss due to defects in the B. Narrow, high-arched palate. C. Cephalometric image
bones of the middle ear (Fig. 6-21D). Condylar and showing mandibular prognathism. (Courtesy of Rania H.
coronoid hypoplasia cause mandibular deficiency and Younis)
alterations of tooth development. Both cleft palate as
well as lateral facial cleft may be noted in some patients.
2577_Ch06_193-226 11/07/14 11:20 AM Page 208
A B
E
Figure 620 Aperts syndrome. A. Child with mid-face deficiency. B. Syndactyly of hands.
C. Radiograph showing bone fusion. D. Syndactyly of toes. E. Pseudocleft of maxilla with
malpositioned teeth. (A, C, and E from the Gorlin Collection, University of Minnesota; B and D
courtesy of Vladimir Leon Salazar)
Nevoid Basal Cell Carcinoma Syndrome Of interest to dentistry is the development of multiple
(Gorlin Syndrome) odontogenic keratocysts in the jaws. A typical patient
Nevoid basal cell carcinoma syndrome (NBCCS) is an auto- usually develops an average of six to twelve odontogenic
somal dominant disorder with variable expressivity keratocysts during their lifetime (Fig. 6-22C). Cysts have
caused by mutations in the patched gene that is located been reported in both jaws, with the majority in the
on chromosome 9. The most significant feature of the mandible. The median age of occurrence for the first
syndrome is development of up to several hundred basal odontogenic keratocyst in the syndrome is 15 years.
cell carcinomas over a lifetime that occur on both sun- Other lesions characteristic of NBCCS include epider-
exposed and nonsun-exposed skin (Fig. 6-22A). Basal moid cysts of the skin (see Chapter 5), which can become
cell carcinomas in the syndrome initially resemble the quite large. In 65 percent of patients, epithelial pits
common melanocytic nevus or mole, thus the name are frequently seen scattered across the palms and soles.
nevoid basal cell carcinoma, but progress to develop central Although the etiology is not completely understood, the
depressions as other basal cell carcinomas (Fig. 6-22B). most widely accepted explanation is a focal reduction in
They begin to appear around the second decade of life epithelial maturation resulting in a decreased keratin
and show less aggressive behavior than nonsyndromic layer, which leads to a depression in the palm and/or
basal cell carcinomas (see Chapter 7). Basal cell carcino- sole. Very rarely, basal cell carcinomas may develop from
mas tend to be fewer and occur less frequently in African the base of these pits. Other lesions seen in NBCCS are
Americans than Caucasians who have the syndrome. shown in Table 6-3.
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A B
C D
Clinical Implications The prognosis for the NBCCA patients may de-
The basal cell carcinomas in Gorlin syndrome are pend on clinical behavior of the numerous basal cell
similar to those observed in nonsyndromic cases carcinomas. Some basal cell carcinomas behave ag-
and are treated similarly. Syndromic odontogenic gressively, causing localized invasion of underlying
keratocysts are similar to nonsyndromic odonto- structures, including the brain, which may lead to
genic keratocysts. However, odontogenic kerato- death. Both sunlight exposure and radiation ther-
cysts associated with Gorlin syndrome have been apy should be avoided due to the increased propen-
reported to contain increased daughter cysts in sity to develop basal cell carcinomas. Odontogenic
their walls (see Chapter 5), which may be associ- keratocysts are typically enucleated, resulting in
ated with their frequent recurrence. jaw deformities because of frequent surgeries.
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Osteopetrosis (Albers-Schnberg Disease, Narrowing of the cranial nerve foramina leads to cra-
Marble Bone Disease) nial nerve compression with possible resultant compli-
Osteopetrosis is a group of skeletal defects that cations of blindness, headaches, and hearing loss.
are caused by a deficiency in or lack of osteoclast func- Significant dental findings include failure of teeth to
tion, which leads to a defect in bone remodeling and erupt (Fig. 6-24B). Subsequent osteomyelitis is caused
significant increase in bone density (-petrosis = turns to by lack of resistance to normal oral flora.
stone). Both autosomal recessive and autosomal dom- The adult form of osteopetrosis is typically autoso-
inant forms of osteopetrosis originate from mutations mal dominant, less severe, and begins much later in
in genes referred to as the CLCN genes, which are crit- life than the infantile form. Bone marrow failure does
ical for osteoclast function. not occur and the skeleton shows no sclerosis. Bone
Osteopetrosis variants are classified into two gen- pain is the most common symptom. Fractures and cra-
eral subtypes: infantile and adult. nial nerve compression occur in variable intensity.
The infantile type is an example of an autosomal re- Dental and maxillofacial findings include os-
cessive disease. This severe form leads to generalized teomyelitis, with fracture of the mandible a rare event.
dense skeletal bones caused by replacement of the bone Adult osteopetrosis patients typically live relatively
marrow by abnormal bone. Patients experience anemia long lives due to the milder form of the disease,
and granulocytopenia that increases susceptibility to whereas infantile patients may die early in the course
infections. The spleen can be recruited to compensate of the disease. Bone marrow transplantation, parathy-
for the need for more red and white blood cells, which roid hormone, erythropoietin, corticosteroid, inter-
results in hepatosplenomegaly, a key clinical finding. feron gamma, and calcitriol are all treatments that lead
Although the bones seem dense (Fig. 6-24A), they are to varying control of symptoms. In cases that do not
brittle and have an increased susceptibility to fractures. respond, hyperbaric oxygen has also been effective.
2577_Ch06_193-226 11/07/14 11:20 AM Page 212
Clinical Implications
The role of the dental health-care provider
in osteopetrosis may be two-fold: 1) diagnosis
of milder adult forms through routine radi-
ographs, and 2) treatment of osteomyelitis.
Management must be performed swiftly to
avoid unnecessary bone loss. This typically
involves antibiotics, debridement/drainage,
and surgical management.
Fibrous Dysplasia
Fibrous dysplasia (FD) is a developmental disorder
that is characterized by replacement of normal bone
by cellular fibrous connective tissue. Mutations in
guanine nucleotide-binding protein, alpha stimulat-
ing activity polypeptide 1 (GNAS1) gene occur
within the individual and are not passed from the
parents; therefore, the disorder is not hereditary. FD
can be classified clinically as monostotic (affecting
B
one bone), polyostotic (involving multiple bones), or
craniofacial, involving more than one contiguous Figure 625 Fibrous dysplasia. A. Nontender bony
craniofacial bone. expansion of anterior maxilla. B. Ground or frosted glass
Monostotic fibrous dysplasia is the most common appearance to involved bone in maxilla. (From the Gorlin
form and typically involves the jaws of males and fe- Collection, University of Minnesota)
males with equal frequency. Most cases are diagnosed
in the early teenage years, when a slow-growing pain-
less swelling of the affected region, usually the maxilla, Polyostotic fibrous dysplasia is the least common
occurs (Fig. 6-25A). Craniofacial fibrous dysplasia form and is seen as part of a syndrome. Jaffe-
is seen in more than one jaw and/or facial bone Lichtenstein syndrome is characterized by polyostotic
concurrently. fibrous dysplasia and caf-au-lait pigmentation of the
The classic radiographic appearance is referred to skin. Caf-au-lait pigmentation is usually present on
as ground glass or frosted glass radiopacity with the skin of the trunk and upper legs. Caf-au-lait
ill-defined borders (Fig. 6-25B). Early lesions gradu- spots appear as large, light brown macules with
ally become more radiopaque with age. The natural irregular borders and are the color of coffee with
process of the disease correlates with this radiographic cream (Fig. 6-26). McCune-Albright syndrome is
appearance. Early in the disease, normal bone is re- characterized by polyostotic fibrous dysplasia, caf-
placed with dense fibrous connective tissue that ex- au-lait pigmentation of the skin, and endocrine dis-
pands the bony area. Gradually, this fibrous connective orders such as hyperthyroidism, pituitary gland
tissue begins to ossify; however, the newly formed dysfunction, and precocious (early) puberty. Polyos-
bone is abnormal with few or tiny marrow spaces. The totic fibrous dysplasia affects the long bones more
lesion is not encapsulated and is directly fused to the often than the craniofacial bones.
surrounding normal bone, giving fibrous dysplasia its Clinical management of fibrous dysplasia can be
characteristic diffuse ill-defined pattern. challenging. Monostotic lesions may be surgically
Narrowing of the periodontal ligament space excised and bone malformations recontoured; how-
with thinning of the lamina dura may be observed ever, larger lesions, especially in the maxilla, require
in periapical radiographs. Obliteration of the maxil- more extensive and multiple surgeries. Surgical man-
lary sinus may be seen with involvement of the agement typically is postponed until after puberty
maxilla. because fibrous dysplasia may stabilize and even
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Gardners Syndrome
Gardners syndrome is a rare autosomal dominant dis-
order, with occasional spontaneous mutations re-
ported. It is generally considered as part of the
spectrum of familial polyposis syndromes, a group
of syndromes in which the colon is affected by the
overgrowth of colon polyps, some of which have ma-
lignant potential. Gardners syndrome patients have
mutations in the adenomatous polyposis coli (APC)
gene. Several clinical features of the skeleton and teeth
are found in this syndrome. However, the most signif-
icant manifestation is the colon polyps that develop in
100 percent of cases in the second decade of life, most
commonly before age 35. The polyps almost always
undergo malignant transformation to adenocarci-
noma. Patients with a known family history of Gard-
ners syndrome often receive prophylactic colectomy
Figure 626 Fibrous dysplasia-caf au lait spot. Large
brown macules in patient with polyostotic fibrous dysplasia.
upon recognition of the polyps to prevent cancer from
developing.
Recognition of Gardners syndrome in the dental of-
regress after skeletal maturation is complete. How- fice may help save a patients life. Classic skeletal defects
ever, if rapid expansion occurs, surgical intervention may affect the skull and maxillofacial bones (Fig. 6-27A).
may occur at an earlier age. Approximately 50 percent Multiple benign osteomas (bone growths) cause signifi-
of lesions regrow and require further surgical man- cant facial deformity (Fig. 6-27B). Dental defects include
agement. Both intravenous and oral bisphosphonates supernumerary teeth, odontomas, and impacted teeth.
Klinefelter Syndrome
Klinefelter syndrome, also called XXY syndrome, is a
disorder in which males have an extra X chromo-
some. The extra X chromosome is retained because
of a nondisjunction event during division. Nondisjunc-
tion occurs when homologous chromosomes, in
this case the X and Y sex chromosomes, fail to sepa-
rate, producing a sperm with an X and a Y chromo-
some. Affected individuals experience hypogonadism, Figure 628 White sponge nevus. Thick white plaques
gynecomastia, and reduced fertility. In childhood, that do not wipe off in areas not readily traumatized.
XXY males exhibit weak muscles and reduced
strength. Low levels of testosterone are present at much larger and more widely distributed than morsi-
puberty, leading to a taller and less muscular body, catio buccarum (see Chapter 2). The soft palate, labial
less facial and body hair, and broader hips compared mucosa, ventral tongue, and floor of the mouth are
to unaffected males. By adulthood, XXY males look occasionally affected. Lesions may also affect other
similar to males without the condition, although mucosal surfaces, including the larynx, esophageal, and
they are often taller. They are also more likely to genital mucosa. The severity varies from patient to
develop autoimmune disorders, breast cancer, vein patient. Once the diagnosis is confirmed with a biopsy,
diseases, and osteoporosis. treatment is unnecessary. Any surgical or chemical
treatment is typically followed by regrowth of the ep-
Clinical Implications ithelium due to the genetic basis for the condition.
Malignant transformation is not reported.
Approximately 20 percent of patients who have
Klinefelter syndrome develop taurodontism (see Hereditary Benign Intraepithelial
Chapter 5). Some other syndromes that feature Dyskeratosis
taurodontism include: Hereditary benign intraepithelial dyskeratosis (HBID) is an
Amelogenesis imperfecta autosomal dominant disorder that shares the clinical
Tricho-dento-osseous syndrome appearance of white sponge nevus (Fig. 6-29A). The
Down syndrome condition affects an isolate of families of African
Williams syndrome American/Native American/Caucasian descent that
McCune-Albright syndrome originated in northeastern North Carolina. In addition
Van der Woude syndrome to the oral mucosal lesions, the eyes develop lesions on
the conjunctiva early in life that feature thick, white,
jelly-like plaques sometimes affecting the cornea
Disorders of the Oral Soft Tissues (Fig. 6-29B). The appearance of lesions varies with
Oral soft tissues are affected by genetic abnormalities. weather conditions, with active lesions present imme-
These are typically subtle and may escape notice during diately after winter and during spring. Rarely, blindness
the oral examination. Selected disorders are presented. may result because of repeated episodes. No treatment
is necessary after establishment of diagnosis for oral le-
White Sponge Nevus sions other than management for candidiasis, if present.
White sponge nevus is an autosomal dominant mucosal Eye lesions that affect vision are surgically removed.
disorder that shows an alteration in keratinization due Referral to an ophthalmologist is critical.
to mutations in keratin 4 and 13 genes. Lesions usually
develop at or immediately after birth and appear as Peutz-Jeghers Syndrome
thick, bilateral, white, diffuse, irregular corrugated Peutz-Jeghers syndrome is an autosomal dominant dis-
patches on the buccal mucosa (Fig. 6-28) that are order characterized by freckle-like pigmentations on
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Table 6.4 Major Types of Amelogenesis newly formed enamel. Failure of this process results in
Imperfecta the incomplete maturation of the enamel crystals and
the eruption of malformed enamel that is soft and
Type Clinical Appearance of Teeth porous. Teeth may appear mottled and opaque because
the poorly formed crystals fail to reflect light as normal
Hypoplastic Normal to small teeth; normal
enamel. Radiographs show enamel is the same radio-
to opaque white-yellow brown
color
density as dentin.
Thin enamel readily subject to Hypocalcified types (Fig. 6-32D) show normal tooth
attrition morphology upon eruption but this becomes rapidly
Smooth enamel with grooves, fur- stained. It is soft and undercalcified, giving it an
rows, and/or pits opaque appearance. These chalky-appearing teeth
Hypomaturation Opaque to yellow/brown; enamel wear down rapidly and are more susceptible to caries.
soft and rough; dentin sensitivity As the underlying dentin is exposed, the teeth take on
Enamel is the same radiodensity as a yellowish-brown stain.
dentin Hypomaturation/hypoplasia/taurodontism, a fourth
Normal enamel thickness; chips easily and less common type, shows enamel that is both
Hypocalcified Opaque chalky white to yellow- poorly formed and soft and porous. The molars also
brown; rough surface, dentin sensi- show taurodontism.
tivity; open bite; heavy calculus Amelogenesis imperfecta can be a severe disability
Normal enamel thickness; chips to the growing individual and dental management can
easily be very difficult. The main clinical problems are
Hypomaturation/ White/yellow-brown mottled, small aesthetics, dental sensitivity, and loss of tooth struc-
hypoplasia/ teeth; lack proximal contact ture, which may lead to occlusal disharmony. Treat-
taurodontism Reduced thickness; hypomineral- ment depends on the severity of the problem. Early
ized pits placement of full crowns will improve the appearance
Molars demonstrate taurodontism of the teeth and protect them from damage. The
B Hypoplastic type
C Hypomaturation type
A
D Hypocalcified type
Figure 632 Amelogenesis imperfecta. A. Radiographic loss of enamel. Three basic defects in
amelogenesis imperfecta. The specific types feature one or more of these patterns. B. Hypoplastic type.
C. Hypomaturation type. D. Hypocalcified type.
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primary dentition is protected by the use of preformed feature is an abnormal discoloration of the teeth due
metal crowns on posterior teeth and composite to the involved dentin showing through the normal
restorations on anterior teeth. As permanent teeth unaffected enamel (Fig. 6-33A). The dentin lacks its
erupt, they can be preserved with crowns as well. normal tubular structure and the teeth have a variable
blue-purple or gray-to-yellow-brown discoloration
that causes the teeth to appear opalescent (Fig. 6-33B).
Clinical Implications
Amelogenesis imperfecta often has similar clini-
cal features as uorosis. To distinguish amelogen-
esis imperfecta from uorosis, one needs a
detailed medical, dental, and family history. This
should include the condition of family members
teeth as well as their history of uoride exposure.
Dentinogenesis Imperfecta
Dentinogenesis imperfecta is an autosomal dominant
disorder characterized by abnormal tissues formed
from the dental papilla. The dental papilla is the por-
tion of the tooth germ responsible for dentin and pulp
formation. Therefore, the disorder is characterized by
abnormal pulp formation and defective mineralization
of dentin. Enamel and cementum are unaffected. Both
the primary and permanent dentitions may be affected,
but commonly both are involved. B
Dentinogenesis imperfecta occurs alone or with os-
teogenesis imperfecta (see earlier discussion of OI in Figure 633 Dentinogenesis imperfecta. A. Child with
dentinogenesis imperfecta with opalescent discoloration
this chapter). These are often referred to as type I and due to altered dentin. B. Her mother with similar-
type II, respectively. Both types have the same dental fea- appearing teeth, some of which have been treated
tures that appear upon tooth eruption. The principal cosmetically.
Dentinogenesis Autosomal dominant Opalescent crowns; severe attrition Obliterated pulp canal; con-
imperfecta stricted cementoenamel
junction; thistle-shaped root
Dentin dysplasia Autosomal dominant Normal crown color; severe hyper- Short roots; crescent-shaped
mobility; premature tooth loss; horizontal pulps; multiple
easy fracture on extraction periapical dental abscesses
or cysts
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Excessive amounts of this poorly formed dentin cause in several important ways. Genetic evaluation shows
abnormal root shape and bulbous crowns with cervical that dentin dysplasia arises from a different mutation
constriction, giving the tooth a thistle-shape. Excess of the gene responsible for dentinogenesis imperfecta
abnormal dentin formation leads to alteration or oblit- (DSPP gene). It is characterized by clinically normal
eration of the pulp chamber (Fig. 6-34). The blood appearing crowns, malformed roots, severe hypermo-
vessels and nerves of the pulp are present and the bility, and spontaneous dental abscesses or cysts with-
teeth are vital, but the pulp chamber is not visible out obvious cause. Root dentin is weak, often leading
radiographically. to fracture during extraction. The pathogenesis of
Because the dentin is defective, the overlying nor- dentin dysplasia is unknown, but it has been suggested
mal enamel is poorly supported and eventually frac- that the dental pulp becomes calcified, leading to
tures away, leading to rapid wear and attrition of the reduced growth and final obliteration of the pulpal
teeth. The severity of discoloration and enamel frac- space.
turing is highly variable even within the same family. Because teeth in dentin dysplasia erupt with a normal
Treatment is restoration of the clinical crowns. With clinical appearance of the crown (Fig. 6-35A), the diag-
the advent of dentin bonding systems, these treatments nosis is based on radiographic findings of the roots.
are more successful than in the past. If left untreated, Both dentitions are affected. Roots are short, blunt, and
it is not uncommon to see the entire dentition worn conical. The teeth lack pulp chambers or have pulp
down level with the gingiva at an early age. chambers that are horizontally oriented with a crescent
shape and roots that are only a few millimeters in
length. Frequently, periapical radiolucencies are ob-
Clinical Implications served (Fig. 6-35B). The extremely short roots give
Not everyone with dentinogenesis imperfecta dentin dysplasia the name rootless teeth. Premature
may have a family member with the condition. loss of teeth may occur. Occasionally, there is a visible
The condition may present as a spontaneous mu- normal pulp with large pulp stones that creates localized
tation. Detailed family history along with com- obstructions within the pulp chambers (Fig. 6-35C).
plete documentation of all clinical dental features Management of patients with dentin dysplasia in-
is essential in arriving at a diagnosis. cludes extraction of teeth with necrotic pulp and apical
abscess. Because these patients usually have early tooth
exfoliation and alveolar bone atrophy, treatment with
Dentin Dysplasia a combination of bone grafting and/or sinus lift may
Dentin dysplasia is an autosomal dominant hereditary be needed for implant placement. Alternately, apical
disorder that also affects tissues derived from the den- surgery with root end resection/root end fill may save
tal papilla, but it varies from dentinogenesis imperfecta teeth if they have long roots.
Case 6-3: Your 6-year-old patient presents Case 6-4: A new family presents to your practice
with several loose teeth. He has experienced early for routine dental care. You have the opportunity to
exfoliation of all his primary teeth and now his few examine four of the six family members. Three of them,
permanent teeth are mobile. including one of the parents, have mottled enamel and
severe attrition. X-rays show thinning of the enamel and
numerous open contacts. When questioned, the parents
List some possible causes of premature tooth loss in a
report that they believe their well water is the source of
child.
the problem. All the children have lived in the same
In addition to a complete medical history and family
location since birth: however, only two of them show
history, what supplementary information should be
the tooth abnormality.
obtained to help differentiate among these causes?
What data can be obtained during the dental
appointment? Do you believe that the well water is the source of the
problem?
If not, what condition do you believe this family has?
What is your evidence?
Review Questions
1. All of the following are true about DNA 4. Which one of the following is an address of
EXCEPT: a gene along a chromosome that allows geneti-
A. It is present in the cytoplasm of every cists to locate a defective gene?
cell. A. 10p12
B. Strands of DNA are arranged in a double B. 23n-33m
helix. C. 1600der
C. Chromosomes are tiny units of organization. D. 46j38
D. It can replicate or make copies of itself.
5. Which one of the following describes when a
2. Segments or sequences of DNA that determine chromosome breaks and is reinserted in the
specific traits or characteristics are called wrong location?
A. euploid. A. Deletion
B. aneuploid. B. Euploid
C. genes. C. Aneuploid
D. centromeres. D. Inversion
3. If a cell has a missing chromosome, that cell is 6. If a gene is expressed, that means the trait
referred to as carried by that gene will
A. autosomic. A. be seen in the individual.
B. euploid. B. lie dormant until the next generation.
C. aneuploid. C. be passed on to the next generation.
D. haploid. D. cause the person to be outgoing and eloquent.
2577_Ch06_193-226 11/07/14 11:20 AM Page 223
7. Many genetic disorders demonstrate signs 12. Patients with ectodermal dysplasia
and symptoms that differ among affected A. have scant eyebrows and sparse fine hair.
individuals. This is known as B. develop fevers of unknown origin.
A. penetrance. C. have conical shaped and numerous missing
B. syndromism. teeth.
C. mutation. D. All of the above
D. variable expressivity.
13. Which one of the following is associated with
8. All of the following are true about autosomal paramedian lip pits?
recessive disorders EXCEPT: A. Cherubism
A. There must be two copies of the disease B. Papillon-Lefvre syndrome
allele for the person to be affected. C. Hypohydrotic ectodermal dysplasia
B. Affected individuals usually have one un- D. van der Woude syndrome
affected parent.
C. There is a 25 percent chance that a child 14. How is cherubism transmitted?
will inherit the mutated gene. A. Autosomal dominant
D. There is a 50 percent chance that a child B. Autosomal recessive
will be an asymptomatic carrier. C. X-linked recessive
D. Mutation only
9. What term is used to describe the visible
characteristics that are created by the gene 15. All of the following are true about Cleidocra-
codes? nial dysplasia EXCEPT:
A. Phenotype A. It is also known as Crouzons syndrome.
B. Genotype B. There is delayed closure of the sutures of
C. Penetrance the skull.
D. Carrier C. Individuals are usually short with large
heads.
10. A collection of signs and symptoms that occur D. Ability of the patient to touch the shoulders
together and when concurrent lead to the di- together is pathognomonic.
agnosis of the condition is called a(n)
A. mutation. 16. Craniosynostoses
B. epinonymous. A. involve premature closure of cranial sutures.
C. syndrome. B. are characterized by accumulations of mult-
D. malformation. inucleated giant cells.
C. are characterized by delayed closure of
11. All of the following are characteristics of cranial sutures.
Papillon-Lefvre syndrome EXCEPT: D. patients have a reduced ability to sweat.
A. It is characterized by the lack of an enzyme
called catalase. 17. All of the following characterize Treacher-
B. There are both dermatological and oral Collins syndrome EXCEPT:
manifestations. A. It is an autosomal dominant disorder.
C. Mutation occurs in the cathepsin C gene. B. There is hyperplasia of the zygomatic bone.
D. Palmoplantar keratosis and advanced peri- C. Colobomas are a feature.
odontitis are features. D. Most patients have hearing loss due to de-
fects in the bones of the middle ear.
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18. Which of the following is not a feature of 24. A benign tumor-like growth made up of nor-
nevoid basal cell carcinoma syndrome? mal mature cells in an abnormal number or
A. Basal cell carcinomas distribution is known as a(n)
B. Bifid ribs A. granulocytopenia.
C. Odontogenic keratocysts B. kleeblattschdel.
D. Coronoid hyperplasia C. hamartoma.
D. nondisjunction.
19. Which one of the following is false regarding
osteogenesis imperfecta? 25. Which of the following statements concern-
A. Dental manifestations include supernumer- ing amelogenesis imperfecta is incorrect?
ary and conical teeth. A. It is an exclusively ectodermal disorder.
B. Teeth develop an opalescent appearance B. This condition results from a defective for-
because of defective dentin. mation, mineralization, or maturation of
C. It is the most common inherited bone enamel.
disease. C. Dentin develops abnormally as part of this
D. Blue sclera of the eyes and hearing loss condition.
can occur. D. The enamel of these teeth is very soft and
abrades away or chips off easily.
20. What group of inherited skeletal defects,
caused by a deficiency in osteoclast function, 26. Which condition is characterized by gray to
results in defective bone remodeling and a violet opalescent teeth and obliterated pulp
significant increase in bone density? chambers and canals, and may occur in
A. van der Woude syndrome conjunction with osteogenesis imperfecta?
B. Cleidocranial dysplasia A. Dentin dysplasia
C. Osteopetrosis B. Dentinogenesis imperfecta
D. Cherubism C. Hypophosphatasia
D. Amelogenesis imperfecta
21. Caf-au-lait pigmentation (spots) is
A. a feature of cherubism. 27. Which statement about dentin dysplasia is
B. present in McCune-Albright syndrome. incorrect?
C. associated with mutation of the TCOF1 gene. A. This condition is also called rootless
D. caused by mutations in the patched gene teeth.
B. It is sex-linked recessive.
22. The most significant manifestation of Gardners C. The roots of these teeth appear short,
syndrome is blunted, or conical.
A. colon polyps that develop in the second D. Premature loss of teeth may occur.
decade of life.
B. high arched palate with clefting. 28. Which one of the following is a metabolic
C. dentinogenesis imperfecta. bone disorder that affects the development of
D. tendency to develop malignant bone tumors. bones, cementum, and periodontal ligament?
A. Peutz-Jeghers syndrome
23. Peutz-Jeghers syndrome is characterized by B. Amelogenesis imperfecta
all of the following EXCEPT: C. Cherubism
A. Adenocarcinoma of the GI tract develops in D. Hypophosphatasia
close to 30 percent of patients.
B. Freckle-like pigmentations occur on the
hands, perioral skin, and intraoral mucosa.
C. Intestinal obstruction due to intussuscep-
tion is a common complication.
D. There is mutation of the keratin 4 and
13 genes.
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C h a p t e r
7
Neoplasms of the Oral Soft Tissues
and Facial Skin
Learning Outcomes
At the end of this chapter, the student will be able to:
7.1. Define all key terms in the chapter. 7.5. List known and suspected risk
7.2. Explain how neoplasms differ from factors for oral squamous cell
reactive lesions. carcinoma.
7.3. Describe the characteristics that 7.6. Explain the significance of dysplasia
distinguish benign neoplasms from of oral epithelium.
malignant neoplasms. 7.7. List the most common locations for
7.4. Describe the standard method of intraoral squamous cell carcinoma.
tumor nomenclature and list excep- 7.8. Explain the use of the TNM Classifi-
tions to the method. cation of Malignant Tumors.
Continued
227
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228 Chapter 7 Neoplasms of the Oral Soft Tissues and Facial Skin
7.9. Describe the treatment for oral 7.11. Describe the clinical features as well
squamous cell carcinoma and its as prognosis for neoplasms affecting
side effects. the oral cavity.
7.10. Give examples of the most common 7.12. Describe epithelial neoplasms com-
benign and malignant salivary gland monly seen on the facial skin.
tumors.
BASIC PRINCIPLES OF NEOPLASIA it may recover and/or adapt to its new environment.
Neoplasia comes from the Greek word meaning new Alternately, its DNA may be damaged to the extent
growth. In neoplasia, the growth of cells is not coor- that the cell can no longer recover sufficiently to carry
dinated with growth of adjacent normal tissues. This out its normal functions.
typically causes a lump referred to as a tumor. The DNA contains genes that regulate the growth and
word tumor literally means swelling but has become functions of the body. When DNA is damaged, one of
accepted to be synonymous with neoplasm. However, several outcomes occurs: the cell dies; the cell recovers
contrary to common belief, tumor and neoplasm are with the help of specialized repair genes; portions of
not synonymous with cancer. This chapter reviews a the DNA that regulate the life cycle of the cell are per-
variety of tumors, some benign (noncancerous) and manently damaged. Neoplasia results from damage to
some malignant (cancerous), that are encountered in the cells DNA. When cells undergo change as a result
dental hygiene practice. of DNA damage, they are known as neoplastic. These
In the 1960s, R. A. Willis defined a neoplasm as an cells form a neoplasm, or tumor. A unique feature of
abnormal mass of tissue, the growth of which exceeds neoplastic cells is that if the injurious agent is removed,
and is uncoordinated with that of normal tissues and the cells continue to grow on their own without
persists after cessation of the stimuli that evoked the further provocation or stimulation. This is one of the
change. The resulting mass is purposeless. For exam- major differences between a neoplasm and a reactive
ple, a neoplastic osteoblast (bone-forming cell) may process: reactive lesions generally resolve when the orig-
continue to lay down bone, but the bone is abnormal inating stimulus is removed; neoplasms do not.
in form and function. Tumors grow autonomously Damage to a cells genes, within the DNA, may be
(independently) and may be life-threatening, invading caused by both known and unknown etiologic agents.
vital structures. The etiology of most neoplasms is unknown. Damage
All benign or malignant tumors originate from a that results in neoplasia includes but is not limited to en-
single cell that was injured. If injury does not kill a cell, vironmental agents and oncogenic viruses (Table 7-1).
Chapter 7 Neoplasms of the Oral Soft Tissues and Facial Skin 229
An altered gene capable of causing a neoplasm is called to eliminate abnormal or damaged cells, a process
an oncogene. The prefix onco- means neoplasm or called apoptosis. If the gene that regulates apoptosis is
tumor. Cells have the ability to repair this damage damaged, cell death may not occur. Affected cells then
through the action of DNA repair genes. If damage oc- continue to live and transmit defective genetic material
curs rapidly and repair genes are overwhelmed, the cells to future generations of cells. This type of transforma-
may continue to divide and transfer genetic damage to tion is the first step in tumorigenesis (tumor develop-
future generations of cells. Cells also have a mechanism ment) (Fig. 7-1).
Healthy cell
Injury to cell*
Genetic errors
Cell returns
result from injury
to health
All errors
Cell attempts repaired
to repair errors
Some
errors remain
Additional
injury to cell
Cell with damaged
DNA continues The killer T cells
Cell unable to to divide recognize and
repair errors destroy the bodys
damaged cells
Cell does not No tumor forms
undergo apoptosis
Cells proliferate
More damage
accumulates
Eventually a
Cell programs malignant
itself to die via tumor forms
apoptosis
* See Table 7-1 for injurious agents.
Figure 71 Carcinogenesis. When the DNA of cells is exposed to a carcinogen, the cells either die (apoptosis) or carry
on the defect to form a tumor mass.
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230 Chapter 7 Neoplasms of the Oral Soft Tissues and Facial Skin
*By convention, these malignancies are not termed melanocarcinoma, lymphosarcoma, or schwannosarcoma.
2577_Ch07_227-268 09/07/14 3:31 PM Page 231
Chapter 7 Neoplasms of the Oral Soft Tissues and Facial Skin 231
The most common defining characteristic is cell Increased or bizarre mitosis: mitotic activity that
appearance. Cells from benign tumors resemble the is increased and abnormal (Cancer cells often divide
cells from which the tumor arose, but they comprise at a faster rate so more dividing cells than normal
a disorganized, purposeless mass. For example, be- may be seen; because their division is out of control,
nign smooth muscle tumors called leiomyomas are the dividing cells may show bizarre shapes, as
made up of cells that look the same as normal shown in Figure 7-2.)
smooth muscle cells. Disordered maturation: normal growth and devel-
Cells change appearance due to damage to their opment of cells is altered; they do not mature prop-
DNA. The term used to describe the degree to which erly (Normal cells form layers or exhibit patterns
they appear different from their normal counterparts necessary for the functions of a specific tissue. An ex-
is differentiation. The range of differentiation is from ample of altered development is epithelium that is
well-differentiated (closest to normal appearance) to undergoing malignant change. Basal cells normally
poorly differentiated (harder to recognize normal appear- located along the basement membrane may be seen
ance) to undifferentiated (change so great that origin of near the surface; Keratin normally located on the
cells cannot be determined by visual inspection). Cells surface may be seen forming in the basal layer.)
that do not appear like their normal counterparts are Besides alteration in individual cells, the tumor as a
termed anaplastic. Anaplastic cells with DNA damage whole may display features that can predict whether it
display one or more of the following features: is benign or malignant:
Pleomorphism: wide range in cell size and shape 1. Rate of growth: Most, but not all, benign tumors
(cells are dissimilar to the size and shape of normal grow slowly over several years; malignant tumors
cells; pleo = many; morphism = shape) tend to grow more quickly, often with sporadic
Hyperchromatism (hyperchromasia): intense growth spurts.
deep blue coloration to nucleus 2. Encapsulation: Because benign tumors grow
Altered nuclear/cytoplasm ratio: nucleus may slowly, they have time to form an outer fibrous
appear extra large or extra small capsule or shell (Fig. 7-3). This keeps the cells
Pleomorphism
Atypical mitosis
Cytoplasm
Nucleolus
Normal
Enlarged bizzare
mitosis
Prominent nucleoli
Figure 72 Anaplasia. Cells
undergoing malignant change
Keratin pearl formation Altered nuclear/ show a variety of atypical cellular
cytoplasmic ratio features.
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232 Chapter 7 Neoplasms of the Oral Soft Tissues and Facial Skin
Chapter 7 Neoplasms of the Oral Soft Tissues and Facial Skin 233
Lymph
Invasion of the tumor border
node
In situ cancer
Lymphatic
spread
Figure 74 Metastasis. A. Cancer cells leave tumor and enter blood stream or lymphatics,
eventually spread to the lungs. B. Patient with indurated lymph node of left neck indicating
metastasis of oral cancer.
234 Chapter 7 Neoplasms of the Oral Soft Tissues and Facial Skin
exposed to tobacco smoke comprise a eld of section explores what these precancerous and cancer-
exposure. One or more sites in this eld can ous changes look like.
develop oral cancer over time. Patients with one
Precancerous Changes in the Oral Cavity
tumor may go on to develop additional tumors in
dierent parts of the mouth in the future. The development of oral cancer is often progressive.
Heredity has not been shown to be a strong Under the influence of a carcinogen, the basal cells
risk factor in oral cancer development; oral can- of the epithelium produce abnormal cells that divide
cer tends not to run in families. There is also a at a rapid rate. This can vastly increase the numbers
misconception that chronic irritation is a cause of cells within the epithelial layer, resulting in excess
of oral cancer. Research has not proven a causal keratin production at the surface. The excess keratin
relationship between chronic irritation, such as a appears clinically as a white plaque. In some cases,
rough tooth, denture, or cheek-chewing habit, the rapidly produced abnormal cells lose their ability
and the development of oral cancer. However, to produce keratin and surface breakdown occurs.
some researchers have observed an association The lack of keratin appears clinically as ulceration.
between chronic inammation and oral cancer. A Therefore, precancerous changes may appear clini-
new area of research is the association between cally with excessive keratin, lack of keratin produc-
mediators of inammation and cellular changes tion, or a combination of both.
that may predispose a person to cancer. Many of
these factors are yet unknown.
Clinical Implications
Knowledge of oral cancer is important in
understanding the: Many white lesions occur in the oral cavity, the
Known risk factors: This is useful in counseling most common of which is frictional keratosis, a
patients on social habits such as alcohol use benign condition caused by chronic irritation
and smoking. (see Chapter 2). Often, it is impossible to tell by
Concept that association does not imply causa- appearance alone which white lesions are pre-
tion. For example, it is incorrect to tell a pa- cancerous and which ones are not. All white
tient that smoking will cause him or her to lesions are considered suspicious until the diag-
develop oral cancer. However, it is correct to nosis is conrmed. Eighty to ninety percent of
tell the patient that smoking will increase his white lesions in the oral cavity are not precancer-
or her risk for developing oral cancer. ous or cancerous. Biopsy is often the only way to
Importance of a comprehensive head and dierentiate between nonmalignant, premalig-
neck examination for all patients, regardless nant (precancer), or malignant (cancer) changes.
of the presence or absence of known risk Look at Figure 7-5A and B. Can you identify
factors. which one is oral cancer?
Not all risk factors for oral cancer are known.
Early lesions in patients may escape detection if
thorough extraoral and intraoral exams are not Dysplasia
performed, simply because a patient does not Dysplasia means loss of normal maturation. Normal
appear to be at risk. oral squamous epithelium is composed of a cuboidal
basal layer, a spinous layer that flattens near the sur-
face, and a keratin layer. Dysplasia means that the
cells have an altered ability to mature from basal
Clinical Features of Oral Cancer cells into keratinocytes that produce keratin. This
Cancer may develop in all oral tissues, including lining may result in loss of the stratified (layered) structure.
and masticatory mucosa, salivary glands, blood vessels, Dysplastic oral epithelium is considered precancerous;
or tonsils. In addition, cancers from other areas in the meaning there is a risk that it will develop into oral
body may metastasize to the oral cavity. However, the cancer. Dysplasia may be mild, moderate, or severe
term oral cancer is reserved in this text to describe ma- depending on the extent of altered maturation. Mild
lignancies that develop within the squamous epithe- dysplasia is cellular change confined to the basal
lium of the oral mucosa. This results in visible and cell layer. This can revert to normal epithelium if the
detectable changes to the surface appearance. The next suspected carcinogenic agent, such as tobacco, is
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Chapter 7 Neoplasms of the Oral Soft Tissues and Facial Skin 235
A B
Figure 75 Comparison of two white plaque of unknown diagnosis. Which of these white lesions is cancerous and
which one is benign? Can you tell?
removed or eliminated. Moderate and severe dyspla- Greek). Often, there is a mixture of red and white in
sia affect the basal, spinous, and keratin layers. Here dysplastic lesions. Erythroleukoplakia is a patch with
the cells cannot revert to normal even if the suspected both red and white features (Fig. 7-7B). These are
carcinogenic agent is removed. Mild, moderate, or clinical descriptive terms and do not imply any specific
severe dysplasia can only be determined by a biopsy diagnosis. Diagnosis is determined microscopically
and examination under the microscope (Fig. 7-6). with a biopsy.
Clinical appearance of oral epithelial dysplasia
varies depending on the extent of cellular changes. If Progression to Oral Cancer
abnormal cells pile up and produce a thick layer of Carcinoma-in-situ (CIS) is the earliest form of oral
keratin, the lesion will appear as a white plaque that cancer. In-situ means in the original placenot
does not wipe off. White lesions that do not wipe off are having been moved or transferred to another location.
called leukoplakia (white plaque in Greek) (Fig. 7-7A). In CIS, all layers of the squamous epithelium demon-
If the cells fail to produce keratin and the surface ep- strate lack of proper maturation. There is no invasion
ithelium becomes thinned (atrophic) or is lost, then into the underlying connective tissue (Fig. 7-8).
the lesion will appear erythematous (red). Erythema- When oral cancer cells invade the underlying con-
tous lesions are called erythroplakia (red plaque in nective tissue, the lesion may become indurated or
Epithelium
Connective
tissue
Atypical cells
Figure 76 Grades of dysplasia to SCC. Epithelial dysplasia is considered a precancerous condition that can develop
through stages from mild to severe, leading to carcinoma.
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236 Chapter 7 Neoplasms of the Oral Soft Tissues and Facial Skin
A
Figure 79 White plaque. Flat white plaque.
Chapter 7 Neoplasms of the Oral Soft Tissues and Facial Skin 237
High-Risk Locations for Oral Cancer Figure 714 Actinic cheilitis. Dry crusted change to
lower lip indicating premalignant lesion.
Oral cancer may occur in any intraoral anatomic loca-
tion but some locations are at higher risk. The follow-
ing list highlights areas of highest to lowest risk: border of the lip is characterized by loss of normal
Vermillion of the lip. SCC of the lip is the most fine wrinkling and loss of a well-defined vermillion-
common form of oral cancer. Fair-skinned individ- skin interface. Individuals who smoke a pipe or
uals who have outdoor occupations or hobbies are cigar may experience similar localized alterations of
at increased risk of excessive sun exposure and dam- the lip. These changes predispose for development
age to the lips. Actinic damage (change produced of SCC. SCC of the lip initially presents as a
by radiant energy, i.e., sun exposure) can predispose crusted, nontender, indurated area. This often
the lower lip to dysplastic and cancerous alterations mimics chapped lips and lesions may grow unat-
(Fig. 7-14). Atrophy (thinning) of the vermillion tended for many months. SCC of the lower lip can
be destructive, resulting in significant lip deformity.
Metastasis occurs in advanced lesions.
Clinical Implications
Individuals spending a great deal of time out-
doors should be counseled on how to avoid
sun damage to the lips, especially the lower
Figure 712 Exophytic mass. Papillary exophytic mass lip. Sun-blocking lip balm is recommended,
(red and white mass). along with wearing a hat. Hats with a brim
shade and protect the face and lower lip from
sun exposure. Close surveillance is indicated
for sun-damaged lips with a mottled chapped
appearance and indistinct vermillion-epithelial
interface.
238 Chapter 7 Neoplasms of the Oral Soft Tissues and Facial Skin
the tongue must be extended completely and turned mouth and ventral tongue difficult to determine
from side to side so that the posterior borders may and compare. However, approximately 35 percent
be fully inspected. The dorsum of the tongue is of all oral cancer is reported to occur in the floor
rarely affected (Fig. 7-15E). of mouth (Fig. 7-16). Oral cancers of the lateral
Floor of the mouth. The floor of the mouth is border of the tongue and floor of mouth occur
continuous with the ventral tongue. This makes with greatest frequency in tobacco smokers, more
the rates of occurrence for SCC between floor of than in any other known risk group. SCC in this
A B
C D
E
Figure 715 Squamous cell carcinoma (SCC). A. Lateral tongue cancer with both endophytic and exophytic features.
B. SCC ventral tongue. Indurated thick, white plaque with raised rolled borders. C. SCC latero-ventral tongue. Lateral
tongue cancer with pebbly red and white surface, extending to ventral tongue. D. SCC ventral tongue. Large exophytic
mass with ulcerated surface. E. SCC dorsal tongue. Red and white change with pebbly exophytic mass on dorsal tongue.
2577_Ch07_227-268 09/07/14 3:31 PM Page 239
Chapter 7 Neoplasms of the Oral Soft Tissues and Facial Skin 239
A
Figure 716 Squamous cell carcinoma-oor of mouth.
Fungating red and white indurated mass encroaching upon
the alveolar ridge.
240 Chapter 7 Neoplasms of the Oral Soft Tissues and Facial Skin
A B
C D
Figure 718 Squamous cell carcinoma: Gingiva. A. Exophytic pebbly red and white mass, thought to be lichen planus
until it became indurated. B. Painless exophytic red and white mass thought to be a pyogenic granuloma. C. Granular red
and white change to the gingiva; teeth became mobile over a 3- to 4-month period. D. Radiograph of C. Posteroanterior
film shows dramatic bone loss of short period of time as tumor invades the alveolar bone.
Chapter 7 Neoplasms of the Oral Soft Tissues and Facial Skin 241
carcinoma. Smokeless tobacco products come for developing oral cancer, but also for devel-
in various forms, such as finely ground, shred- oping periodontitis and caries. Occasionally,
ded, or powdered. They often contain more because of social stigma attached to smokeless
than 28 known carcinogens, in addition to sug- tobacco use, users hide the tobacco out of
ared flavoring agents. Users are not only at risk sight, including under dentures.
A B
Figure 719 Squamous cell carcinoma: Alveolar ridge. A. Mobile tooth was extracted; tumor
grew into socket. B. Patients denture became unstable as tumor spread from alveolar ridge to floor
of mouth and lateral tongue.
A B
Figure 720 Squamous cell carcinoma: Buccal mucosa. A. Extensive firm, indurated tumor extends from buccal
mucosa to retromolar pad and gingiva. B. Stippled red and white patch with ill-defined borders.
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242 Chapter 7 Neoplasms of the Oral Soft Tissues and Facial Skin
A B
Figure 721 Squamous cell carcinoma: Hard palate. A. Depressed granular center with raised indurated borders
with white surface alteration. B. Nonpainful exophytic red and white tumor extending to involve soft palate.
A B
Figure 722 Verrucous carcinoma. A. Firm verruco-papillary surface texture. B. On close
inspection, early verrucous carcinoma shows fine verruco-papillary surface.
Chapter 7 Neoplasms of the Oral Soft Tissues and Facial Skin 243
Table 7.3 TNM System for Staging of Oral a relatively good prognosis. See Table 7-4 for current
and Oropharyngeal Cancer 5-year-survival data on oral cancer.
TNM System
244 Chapter 7 Neoplasms of the Oral Soft Tissues and Facial Skin
Chapter 7 Neoplasms of the Oral Soft Tissues and Facial Skin 245
246 Chapter 7 Neoplasms of the Oral Soft Tissues and Facial Skin
and loss of the overlying oral mucosa. The soft tissue Chemotherapy and Side Eects
sloughs, exposing areas of dead bone to the oral cavity Chemotherapy involves the use of drugs that interfere
(Fig. 7-26). The threat of developing osteoradionecrosis with cancer cells ability to divide or grow. The types
is lifelong and preventive measures must continue of drugs used depend on what type of tumor is being
throughout life. treated. Not all tumors are sensitive to chemotherapy
so not all patients receive it.
Clinical Implications The severity of chemotherapy side effects differs
from person to person. Typical side effects include
Figure 7-27 shows a young patient who received
gastrointestinal tract irritation leading to nausea and
radiation therapy to treat cancer during child-
vomiting, alopecia (hair loss), anemia and leukopenia,
hood to the left side of the head. Tooth follicles
thrombocytopenia, dermatitis, mucositis, fatigue, and de-
developing at that time received radiation
layed wound healing. During chemotherapy, patients
damage, resulting in dental hypoplasia.
may be unable to mount a normal immune response
to common injuries and infections. Mucositis
may affect eating and maintaining good nutrition.
Erosion of the teeth may result from prolonged
periods of nausea and vomiting.
Chemotherapy-induced mucositis is typically
less severe and of shorter duration than radiation-
induced mucositis. However, patients receiving con-
current radiation and chemotherapy treatment have
increased severity and duration of mucositis. Mucositis
has recently become more problematic as the combi-
nation of treatment with chemotherapy and radiation
have become more common.
Oral mucositis is painful, making eating and swal-
lowing difficult. Oral ulcerations commonly develop
(Fig. 7-28) and when they are extensive, there is an
increased risk of a developing septicemia. Prophylac-
tic antibiotics may be indicated. Oral care is difficult
during an episode of oral mucositis. Severe pain may
make brushing and flossing difficult and unpleasant,
Figure 726 Osteoradionecrosis. Necrotic bone in leading to compromised oral hygiene.
patient who received radiation therapy for tumor in
posterior tongue.
Figure 727 Dental hypoplasia in patient irradiated for Figure 728 Chemotherapy mucositis. Severe
cancer as a child; the developing tooth buds in the path of inflammation of palate, tongue, and gingiva causing
radiation were damaged. problems with proper nutrition and hydration.
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Chapter 7 Neoplasms of the Oral Soft Tissues and Facial Skin 247
Spontaneous healing of chemotherapy-induced hair may be observed growing in the center. Many
mucositis occurs within 2 to 3 weeks after completion regress with age, so few may be seen in older adults.
of treatment. Healing of ulcers usually takes 7 to No treatment is needed unless the patient elects to have
10 days. Because oral mucositis is self-limiting, man- the lesions removed for cosmetic reasons or they
agement of lesions should include oral debridement, become irritated. Few ever progress to cancer.
antifungal and antibacterial rinses, pain management,
and control of bleeding. Palliative care with topical Intraoral Melanocytic Nevus
bio-adherent oral gels may help the patient for the du- Intraoral melanocytic nevi may be seen on any
ration of the therapy. A protocol consisting of regular mucosal surface. They tend to be less pigmented than
rinsing with bland agents, brushing with a soft tooth- nevi of the skin (Fig. 7-30); however, when they are
brush, using water-based moisturizers, and flossing, if pigmented they can mimic the rare intraoral melanoma
tolerable, is indicated. Cryotherapy (sucking on ice (discussed below). Unlike the skin, there are few distin-
chips) and topical anesthetics may be helpful. guishing clinical features to differentiate between intra-
oral nevus and intraoral melanoma, so surgical excision
is often undertaken.
EPITHELIAL NEOPLASMS OF THE ORAL The unusual blue nevus has been reported in the
MUCOUS MEMBRANES AND FACIAL SKIN oral cavity. It most often occurs as a macular lesion on
Oral cancer is the epithelial neoplasm of most concern the palate. Blue nevi have a characteristic blue to blue-
in the oral cavity; however, additional benign neoplasms black color produced by abundant melanin particles
do occur in the oral cavity and on the facial skin. Some located deep in the submucosa. The overlying tissues
of these are presented in the next section. cause a change in light reflectance, causing these nevi
to appear blue rather than brown.
Benign Tumors
Acquired Melanocytic Nevus (Common Mole) Clinical Implications
Nevus (plural nevi) refers to a congenital (present at
With the exception of an x-rayconrmed amal-
or shortly after birth) circumscribed malformation of
gam tattoo (Chapter 2), it is not possible to dif-
the skin composed of nevus cells. The acquired
ferentiate among most intraoral pigmented
melanocytic nevus (common mole) is a benign collec-
lesions by visual inspection alone. All pigmented
tion of nevus cells that have the ability to produce
lesions in the oral cavity that cannot be clearly
melanin pigment. Nevus cells have an embryonic
identied should be considered for biopsy.
origin similar to melanocytes cells of the epidermis.
Nevi develop early in childhood, most commonly on
skin of the head and neck region. They begin as a Seborrheic Keratosis
sharply delineated brown macule that gradually evolves Seborrheic keratosis (SK) is a benign proliferation of
into a smooth exophytic papule as cells proliferate in basal cells, which is common on sun-exposed areas of
the dermis (Fig. 7-29). Lesions rarely exceed 6 mm in
diameter. As the lesion ages, pigment tends to fade.
The surface becomes less smooth and occasionally a
Figure 729 Dermal nevus. Well-defined pigmented Figure 730 Intramucosal nevus. Exophytic pigmented
nodule of the lower lip. mass of the gingiva.
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248 Chapter 7 Neoplasms of the Oral Soft Tissues and Facial Skin
the skin as an individual ages. SK is not seen inside the of keratin seen against a mottled background of
oral cavity. Lesions are caused by a proliferation of sun-damaged skin. Men are affected more often than
normal basal cells of the epithelium in response to sun- women, probably because of the protection provided
light or because of a genetic mutation in one of the by lipstick. The presence of actinic keratosis is
growth factor genes. Because melanocytes reside considered a risk factor for developing skin cancer
among the basal cells, SKs tend to be pigmented. They (squamous cell carcinoma of the skin). Rate of trans-
appear as pigmented soft tissue masses that may occur formation to squamous cell carcinoma is estimated
singly but often occur in clusters. SKs rarely exceed to be 10% to 20% over 10 years. Removal of actinic
2 mm in diameter though they may gradually increase keratosis helps reduce the risk of developing skin
in size and eventually flatten. The surface can vary cancer in that area.
from rough to smooth. The lesions have been de-
scribed as having a stuck-on raisin-like appearance Proliferative Verrucous Leukoplakia
in the early stages. Occasionally, patients report they Proliferative verrucous leukoplakia (PVL) is a condition
are pruritic (itchy). characterized by thick white plaques. It is chronic, per-
A variant of seborrheic keratosis is seen in patients sistent, and recurrent. Unlike other leukoplakias, PVL
of African descent. Dermatosis papulosa nigra is seen as has a high rate of malignant transformation to either
multiple small pigmented papules of the face, usually squamous cell carcinoma or verrucous carcinoma. When
in the malar and infraorbital areas (Fig. 7-31). the lesions are initially examined, most have benign
Seborrheic keratosis is rarely of concern because features. Changes toward premalignant dysplasia and
the lesions have no potential to become malignant. eventually carcinoma are seen over time. An association
Lesions are often removed for cosmetic purposes; with HPV infection, particularly strains 16 and 18, has
however, concern arises when there is a sudden erup- been implicated in some cases, but many cases are HPV
tion of numerous SK lesions over a short period negative. Only 30% of patients with PVL report a his-
of time. This is called the sign of Leser-Trlat and may tory of smoking, much lower than in populations
signal the patient is developing an internal malignancy. affected by hyperkeratosis (Chapter 2), epithelial dyspla-
sia, and oral cancer. Most cases occur in females, with a
Epithelial Disorders With Malignant female-to-male ratio of approximately 4:1 in adults older
Potential than 40 years. The peak incidence occurs in women aged
Some benign conditions have malignant potential in all 60 to 70 years.
patients afflicted by them. Three will be discussed here. PVL begins as a flat white plaque, much like any
other keratotic plaque, such as frictional keratosis
Actinic Keratosis (Fig. 7-32A). Progressively, dramatic multiple keratotic
Actinic keratosis is a premalignant condition of sun- plaques with rough corrugated surfaces and papilloma-
exposed skin. The term actinic refers to exposure to like projections or thick white fissuring develop
the sun. Keratosis refers to rough, raised area of (Fig. 7-32B). The plaques slowly spread; multiple
keratinized epithelium. These skin lesions appear as sites may be involved at one time. The gingiva and
irregular crusty plaques producing excessive amounts buccal mucosa are typically included. Due to its pro-
gressive behavior, multiple serial biopsies are needed
to establish the diagnosis of PVL.
Surgical excision is the current treatment for PVL.
Because of the high recurrence rate, multiple surgeries
may be necessary. Carbon dioxide laser, radiation, top-
ical bleomycin solution, oral retinoids, beta-carotene,
and systemic chemotherapy have all failed to reduce
recurrence rates. Laser ablation and topical photody-
namic therapy hold some promise in research trials.
Chapter 7 Neoplasms of the Oral Soft Tissues and Facial Skin 249
A A
B B
Figure 732 Proliferative verrucous leukoplakia.
A. Thick, white plaques with no obvious source of friction
showed early stages of dysplasia on biopsy. B. Heavy
corrugated premalignant lesions of the gingiva and
vestibule.
250 Chapter 7 Neoplasms of the Oral Soft Tissues and Facial Skin
Chapter 7 Neoplasms of the Oral Soft Tissues and Facial Skin 251
A B
Figure 736 Melanoma of facial skin. A. Large,
asymmetric variegated lesion with irregular borders of
sun-exposed skin. B. Melanoma of ear in a young patient
who spent long hours in the sun. Figure 737 Intraoral melanoma. Gingival melanoma.
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252 Chapter 7 Neoplasms of the Oral Soft Tissues and Facial Skin
the need for early detection. The prognosis for intra- The most common location for pleomorphic ade-
oral melanoma is very poor, with only 10% to 15% of noma is the parotid gland, where it produces a very
patients surviving beyond 5 years. slow-growing firm and painless mass (Fig. 7-38) that
may sometimes be superimposed over the angle of the
mandible (Fig. 7-39). Upon palpation, these tumors
SALIVARY GLAND NEOPLASIA
have the consistency of a raw potato. They are usually
Salivary gland neoplasia is exclusively found in the well encapsulated; however, the facial nerve is often
oral cavity and tissues of the head and neck. Under-
standing their clinical features is essential because
many salivary gland tumors are discovered during a
dental visit. Most salivary gland tumors are benign;
however, some can be malignant. Therefore, early
diagnosis may help save a patients life.
Salivary gland neoplasms can occur wherever there
is salivary gland tissue. Considering the large number
of minor salivary glands within the oral mucosa, this
includes almost the entire oral cavity, with the excep-
tion of the gingiva and anterior hard palate. Normal
glands are composed of a variety of duct cells, saliva-
producing acinar cells, and specialized myoepithelial cells,
which contract and help expel saliva from the gland. Figure 738 Pleomorphic adenoma. Mass of lower lobe
This variety of cells helps explain the large number of of parotid gland.
both benign and malignant salivary gland tumors that
arise from them. More than 34 different types of
salivary gland tumors exist but only the most common
are discussed here (Table 7-5).
Chapter 7 Neoplasms of the Oral Soft Tissues and Facial Skin 253
Warthin Tumor
Warthin tumor is almost exclusively found in the
parotid gland, where it shares many clinical features Figure 741 Pleomorphic adenoma: submandibular
with pleomorphic adenoma. However, a distinguishing gland. Firm mass of right submandibular gland causing
cervical swelling.
clinical feature is that Warthin tumor is seen almost
exclusively in smokers. Historically, there was a male-
to-female ratio of 10:1; however, as the number of times (metachronous rather than synchronous).
women smokers has increased, the male-to-female Treatment is conservative surgical excision with care
ratio has dropped to 2:1. Another interesting feature to spare the facial nerve.
of this tumor is that it can occur bilaterally at different
Canalicular Adenoma
Canalicular adenoma is seen almost exclusively in minor
salivary glands, strongly favoring the upper lip of older
adults. The tumor assumes its name from its micro-
scopic appearance, which shows ductal structures that
mimic a network of canals. It appears as a slow-growing
painless mass that can mimic a mucocele, which is rare
on the upper lip. Like Warthin tumor, they can occur
at multiple sites. Treatment is conservative surgical
excision and there is a low rate of recurrence.
Surgery is the treatment of choice for benign
salivary gland tumors. Most develop a capsule, which
helps with their surgical removal; however, risk
of damage to the facial nerve is increased when the
A
surgery occurs in the parotid gland.
B Mucoepidermoid Carcinoma
Figure 740 Pleomorphic adenoma of palate. A. Firm Mucoepidermoid carcinoma is the most common
exophytic mass of the posterior palate. B. Close-up view malignant salivary gland neoplasm. It derives its name
shows small ulcerations. from the mixture of neoplastic mucous acinar cells and
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254 Chapter 7 Neoplasms of the Oral Soft Tissues and Facial Skin
epidermoid ductal cells. The most common type is the glands. It is most frequently seen in the minor salivary
low-grade variant that is slow growing and follows a glands, predominantly in the palate where it causes a
relatively benign clinical course, rarely causing death. painless swelling often near the junction of the hard
High-grade mucoepidermoid carcinomas tend to grow and soft palate (Fig. 7-43). It may be mucosal-colored
more quickly and metastasize, reducing the chance of or have a bluish tint. As with other palatal masses, it
survival. may become ulcerated because of local trauma. It is
Mucoepidermoid carcinoma may occur at any age rarely seen in the major glands. As the name implies
and is the most common salivary gland malignancy (low-grade), the tumor has a favorable prognosis.
among children. The most frequent site is the parotid,
where the low-grade type shares clinical characteristics Adenoid Cystic Carcinoma
with pleomorphic adenoma and Warthin tumor. How- Adenoid cystic carcinoma occurs in both the minor
ever, high-grade tumors may invade the facial nerve and salivary glands of the posterior hard palate and major
cause facial nerve pain and paralysis. When the tumor salivary glands (Fig. 7-44). It is unusual among
develops in minor glands, it may resemble a mucocele. salivary gland neoplasms in that pain is an early sign.
The abundance of neoplastic mucous cells produce This is because this tumor is neurotropic, meaning
mucin that gives the tumor a fluctuant, bluish appear- it tends to invade peripheral nerve. It rarely occurs
ance (Fig. 7-42). If it is ignored, the tumor may enlarge, in individuals younger than age 20 and tends to pre-
necessitating a more extensive surgery to remove it. dominate among females. The tumors tend to recur
Treatment depends on tumor grade. Low-grade tumors later in life rather than sooner, unlike other malig-
are conservatively excised and have low risk of recur- nancies. Unlike other salivary gland malignancies,
rence. High-grade tumors may require presurgical and the 5-year survival is relatively high and the 10-year
postsurgical radiation and neck dissection to remove any survival rate is very low, with few patients surviving
lymph nodes that contain spreading tumor cells. beyond 20 years due to widespread metastasis.
On rare occasions, mucoepidermoid carcinoma Adenoid cystic carcinoma has a tendency to metas-
may develop within the body and ramus of the tasize to the brain.
mandible, causing signs that mimic an odontogenic
tumor (Chapter 8). Some believe they develop from Acinic Cell Carcinoma
salivary gland tissue that became entrapped during em- Acinic cell carcinoma is derived from the saliva-
bryogenesis. Others propose that the neoplastic mucous producing serous acinar cells of the parotid gland.
cells form from remnants of odontogenic epithelium. Along with mucoepidermoid carcinoma, it is one of
The true answer is unknown. the few salivary gland tumors seen in children. It pres-
ents as an asymptomatic slow-growing mass that may
Polymorphous Low-Grade Adenocarcinoma be present for many months before the patient seeks
Polymorphous low-grade adenocarcinoma (PLGA) is treatment. It is considered to be a low-grade malig-
the second most common malignancy of salivary nancy with a favorable prognosis.
Figure 742 Mucoepidermoid carcinoma. Ulcerated Figure 743 Polymorphous low-grade adenocarcinoma.
exophytic mass of palate; patient reports denture no longer Ulcerated exophytic mass of palate; patient reports denture
fits. no longer fits.
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Chapter 7 Neoplasms of the Oral Soft Tissues and Facial Skin 255
Figure 744 Adenoid cystic carcinoma. Painful swelling Figure 745 Lipoma. Well-encapsulated exophytic soft
of palatal mucosa extending to alveolar ridge. mucosal colored-to-yellow mass of floor of mouth.
Carcinoma Ex-Pleomorphic Adenoma location is the buccal mucosa. Others occur as mobile
Carcinoma ex-pleomorphic adenoma is the malignant submucosal masses on the tongue, floor of mouth,
form of pleomorphic adenoma. It forms in an existing and lips. As is typical of benign tumors, they are well-
pleomorphic adenoma or in a patient whose pleomor- circumscribed, often encapsulated, and when removed
phic adenoma was previously removed. In either case, surgically they tend to come out in a solid mass. They
sudden rapid growth of a previously stable salivary rarely recur.
gland mass presents the clinical clue that the tumor is Liposarcomas of the head and neck area are typi-
malignant. cally well-differentiated and carry an excellent 5-year
survival rate, unlike liposarcomas that occur in deep
SOFT TISSUE NEOPLASIA body cavities that have a low survival rate. Once re-
Soft tissue has a specific connotation in pathology. moved, liposarcomas of the head area must be closely
Although many tissues are soft, including epithe- followed for recurrence.
lium, glands, and muscle, this term is reserved for
nonepithelial tissue derived embryologically from Connective Tissue Proper
mesoderm and neuroectoderm. This includes fibrous Myofibromas arise from myofibroblasts, a common con-
connective tissue, blood and lymphatic vessels, adi- nective tissue cell. This cell has features of both fibrob-
pose tissue, skeletal and smooth muscle, peripheral lasts and smooth muscle cells. Myofibromas are most
nerves and their supporting tissues. Benign tumors of common in the tongue, lips, and buccal mucosa. Typi-
soft tissues are identified by using the tissue name and cally, they are firm, slow-growing submucosal nodules
adding -oma. Malignancies of the nonepithelial soft or exophytic masses. Although patients are frequently
tissues are termed sarcomas. asymptomatic, the lesions may be tender or painful.
Treatment for oral myofibromas is conservative excision.
Adipose Tissue The recurrence rate is low and spontaneous regression
Lipoma is benign tumor of fat, or adipose, tissue and (lesion resolves on its own) has been reported.
one of the most common tumors in the body. Most Fibrosarcoma in the head and oral cavity accounts for
occur in the arms, legs, and trunk of adults but rarely less than 10 percent of all fibrosarcoma cases in the
in children. Intraoral lipoma is far less common than body. They tend to be slow-growing nasal and sinus
extraoral lipoma. The etiology is unknown and they masses in children and young adults. Obstructive
are not associated with any syndromes or genetic symptoms and pain are frequent complaints. After sur-
abnormalities. gical therapy, tumors often recur. The 5-year survival
Intraoral lipoma is often yellow in color, reflecting rate is approximately 50 percent.
the natural yellow color of mature fat. However, if a
layer of fibrous connective tissue lies between the Nerve and Nerve Sheath Tumors
tumor and the surface mucosa it may appear the color Tissue that surrounds and supports peripheral nerves
of normal mucosa (Fig. 7-45). The most common (nerve sheath) can produce a number of neoplasms
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256 Chapter 7 Neoplasms of the Oral Soft Tissues and Facial Skin
such as schwannoma, neurofibroma, and granular surfaces that appear in young adulthood. On the skin,
cell tumor. their texture is soft and spongy but in the oral cavity
Schwannoma, also called neurilemoma, is a benign they may be firm, similar to a pencil eraser. They tend
neoplasm arising from the myelin-producing Schwann to be small (Fig. 7-47A), but lesions greater than 1 cm
cells that surround and support peripheral nerves. have been reported. Neurofibromas are treated by
Myelin is an insulating material that forms the myelin simple excision and rarely recur. Lesions that develop
sheath to protect the nerve axon and help with nerve along the inferior alveolar nerve are detected
impulse conduction. Up to 50 percent of all schwan- radiographically (Fig. 7-47B). Neurofibromatosis
nomas occur in the head, neck, and oral cavity, with Types 1 and 2 are two hereditary conditions caused
the tongue being the most common location. They by genetic mutations that produce hundreds of
tend to occur as a single tumor that grows slowly to neurofibromas.
reach up to 1 cm. Schwannomas are not painful be- Neurofibrosarcoma, also called malignant peripheral
cause they do not affect the nerve axon; however, if nerve sheath tumor (MPNST), is a malignancy that
they surround a nerve, they may push the nerve out of arises in a neurofibroma and has only a 15% to 20%
its normal position, causing tenderness. Most schwan- 5-year survival rate. Patients typically develop
nomas are easily removed without damaging the MPNST during their 50s. When lesions occur, they
nearby nerve. appear as asymptomatic masses that grow quickly.
Granular cell tumor tends to favor the oral cavity. They cause pain only after they have invaded adjacent
Its name is derived from the unusual microscopic
appearance of large pink cells with grainy-looking
cytoplasm. The origin of these granular cells was the
subject of much debate over the years until special
tests became available that showed similarities
between the granular cells and Schwann cells. The
most common location is the dorsal tongue, where
it appears as a single asymptomatic sessile submu-
cosal mass that averages less than 1 cm in size
(Fig. 7-46). Other locations include the buccal mu-
cosa, labial mucosa, and the skin. Surgical excision
is the treatment and they rarely recur.
Neurofibroma is the most common benign neo-
A
plasm of the peripheral nervous system. They arise
from both Schwann cells and neurofibroblasts, cells
that produce collagen to support the nerve. They are
slow-growing, painless lesions of the skin and mucosal
B
Figure 747 Neurobroma. A. Neurofibroma of the
palate. Painless exophytic soft mucosal-colored mass of palatal
Figure 746 Granular cell tumor of tongue. Well-defined mucosa. B. X-ray of the mandible. Tender enlargement of
painless soft yellow mass of left dorsolateral tongue. mandibular canal.
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Chapter 7 Neoplasms of the Oral Soft Tissues and Facial Skin 257
258 Chapter 7 Neoplasms of the Oral Soft Tissues and Facial Skin
Clinical Implications
Another condition called vascular anomaly or
vascular malformation is often confused clini-
cally with hemangioma. Unlike hemangiomas,
they are caused by abnormalities in the structure
of the vessels and are not due to a proliferation
of endothelial cells. Port wine stain is a form of
vascular malformation that develops along the
path of the trigeminal nerve. If the rst (ocular)
division of the trigeminal nerve is aected, the
patient may have a condition called Sturge-
Weber syndrome. If the second or third divisions
of the trigeminal nerve are aected, the patient
may exhibit raised red/blue gingival lesions. C
Figure 749 Hemangioma. A. Adult male with lifelong
soft blue mass that blanches upon gentle pressure.
B. Extensive diffuse congenital blue lesion in child.
Angiosarcoma C. Well-defined radiolucency associated with inferior
Angiosarcoma is a malignancy of blood vessels, alveolar canal; blood was aspirated.
specifically of the endothelial cells that line blood and
lymphatic vessels. The skin of the head and neck area
is one of the favored sites. Patients tend to be elderly
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Chapter 7 Neoplasms of the Oral Soft Tissues and Facial Skin 259
and present with a bruise of the scalp or forehead declined. However, KS unrelated to HIV/AIDS
that does not heal, and eventually enlarges into a is not rare among organ transplant patients who
tumor mass. Angiosarcoma has a very poor prognosis, receive drugs that cause immunosuppression to pre-
with a less than 20 percent 5-year survival rate. vent organ rejection. Lesions may develop soon
after the transplant or later at any time, since im-
Kaposis Sarcoma munosuppression is long term.
Kaposis sarcoma (KS) is a type of angiosarcoma that is
associated with an oncogenic virus called human her- Tumors of Muscle
pes virus 8 (HHV8). This virus infects endothelial cells Two types of muscle are found in the head and neck:
and causes them to proliferate, forming tiny vascular 1) voluntary striated skeletal muscle, and 2) involun-
channels. The vessels are just large enough for red tary smooth muscle, typically found around arterioles
blood cells to pass through single-file, giving the skin and in the erector pili muscles of the skin. The prefix
a red/blue appearance. Tumors begin as flat, painless used to indicate tumors of striated skeletal muscle is
macular lesions that grow slowly into nodules, seen rhabdomyo-; the prefix used to indicate tumors of
mainly on the skin. Intraorally, they appear in areas ex- smooth muscle is leiomyo-.
posed to trauma, such as the hard palate (Fig. 7-50) Rhabdomyoma is a benign tumor of mature striated
and gingiva. KS differs significantly from other an- skeletal muscle cell origin. Intraorally, these are found
giosarcomas in that it is very low-grade and is rarely in or near normal skeletal muscles in adults, mainly in
life-threatening. Once diagnosed, many lesions are not the posterior oropharynx where airway obstruction
treated. can be the presenting feature. There are no clinical
Kaposis sarcoma was virtually unknown before features that are unique to this tumor, and its diagnosis
the onset of HIV/AIDS in the 1980s. Before then, the is usually a surprise to the surgeon and the pathologist.
so-called classical form of KS was seen in elderly men Treatment is surgical excision.
in Mediterranean and Slavic countries. Endemic Rhabdomyosarcoma is a malignant tumor of striated
KS affecting internal organs was seen in children in skeletal muscle. Although a rare form can occur in
Africa. Some forms of endemic KS are indolent (cause adults older than age 45, this is primarily a childhood
few or no symptoms), but others can cause significant tumor generally seen in children younger than age 10.
morbidity (illness) and even mortality (death) when The head and neck are the most common sites. Many
vital organs are affected. tumors appear as painless asymmetrical swellings
Kaposis sarcoma typically seen in the United in the maxillary sinus or nasal cavity that often arise
States and other developed countries is associated rapidly over a short period of time. Tumors may
with immune suppression. Since the advent of invade adjacent bone readily and cause dramatic facial
highly active antiretroviral therapy (HAART), the asymmetry. Recent advances in chemotherapy have
incidence of KS among HIV-positive patients has improved long-term survival, but rhabdomyosarcoma
continues to have a poor prognosis.
Leiomyoma is a benign tumor of smooth muscle cells
that is most common in the uterus, where it is com-
monly called fibroids. Other common locations are
the erector pili muscles of the skin and the smooth
muscle layer of the GI tract. Oral leiomyomas may be
seen in association with the smooth muscle of blood
vessel walls, in which case they are termed angioleiomy-
oma or vascular leiomyoma. They present as a painful
or tender red/blue submucosal nodule anywhere in
the oral cavity. Leiomyomas are treated by surgical
excision and rarely recur.
Leiomyosarcoma is a malignant tumor of smooth mus-
cle found in locations where smooth muscle is abun-
dant. In the head and neck, they occur in association
Figure 750 Kaposis sarcoma. Slightly raised blue with the smooth muscle of blood vessel walls. There
lesion with ill-defined border on right palate. are so few cases in the oral cavity that generalizations
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260 Chapter 7 Neoplasms of the Oral Soft Tissues and Facial Skin
about age, location, and clinical features cannot be Table 7.7 Origin of Metastatic Tumors
made. Treatment is surgery, but recurrence and metas- Found in the Oral Soft Tissues
tasis are not rare. The 5-year survival rate is about
50 percent. Males Females
Chapter 7 Neoplasms of the Oral Soft Tissues and Facial Skin 261
262 Chapter 7 Neoplasms of the Oral Soft Tissues and Facial Skin
A
Figure 753 Hodgkins lymphoma. Slight enlargement
of right neck is firm and fixed to underlying musculature.
Chapter 7 Neoplasms of the Oral Soft Tissues and Facial Skin 263
A B
C D
E
Figure 754 Lymphoma. A. Pyogenic granuloma-like mass in adult patient with lymphoma. B. Lymphoma causing
bilateral enlargement of the hard palate with surface ulceration. C. Lymphoma arising in lingual tonsil. D. Lymphoma
arising within bone causing resorption of molar roots. E. Tooth in D was extracted; mass grew out of extraction site.
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264 Chapter 7 Neoplasms of the Oral Soft Tissues and Facial Skin
Burkitts lymphoma. Burkitts lymphoma primarily expansion. Occasionally, the tumor is found outside
affects young children and adolescents in two main the marrow spaces (extramedullary). Treatment con-
forms: endemic and sporadic. Endemic Burkitts is be- sists of surgical excision and radiation therapy.
lieved to be caused by Epstein-Barr virus. It was first Plasmacytoma patients who are observed over
identified in African children and termed African many years often go on to develop multiple lesions.
Burkitts, though children in other countries have This condition is called multiple myeloma. Multiple
been reported to have the disease. Patients develop myeloma is rarely diagnosed before age 40. Bone pain
relatively painless intraosseous maxillary lesions that and pathologic fracture may be the presenting signs
cause gross facial deformity, tooth mobility, and of the tumor. The classic radiographic appearance
premature exfoliation of deciduous teeth. Sporadic is punched out or coin lesions/radiolucencies
Burkitts is believed to be caused by a chromosomal (Fig. 7-56).
abnormality. It was first identified in American Because normal plasma cells are responsible for an-
children and is therefore termed American Burkitts. tibody production, patients with widespread multiple
These children develop abdominal tumors with only myeloma often suffer from repeated infections due
rare jaw involvement. Immunosuppressed patients to abnormal immunoglobulins (antibodies). These
such as HIV-positive patients or transplantation abnormal immunoglobulins can be detected in the
patients have also been reported to develop Burkitts urine in the form of Bence-Jones proteins. Also, other
lymphoma. Regardless of form, the radiographic abnormal proteins called amyloid are deposited in the
features include ill-defined radiolucent bone de- soft tissues. If the tongue is affected, macroglossia
struction that may be preceded by loss of lamina may result.
dura. Treatment includes aggressive chemotherapy Treatment is mainly aggressive chemotherapy,
and recurrence is possible. but long-term survival is less than 20 percent.
Bisphosphonate therapy has been shown to be help-
Plasmacytoma and Multiple Myeloma ful in strengthening the bone and reducing fractures.
Plasmacytoma and multiple myeloma are two re- Patients being treated with bisphosphonates are
lated malignancies of plasma cells. Plasma cells are at increased risk for developing bisphosphonate
responsible for producing antibodies. Plasmacytoma osteonecrosis (Chapter 2).
is a solitary (single) tumor of malignant plasma cells
that is most commonly seen in adult females older Langerhans Cell Histiocytosis
than age 40. The marrow spaces of the spine are the Langerhans cells are found in the skin and mucous
most common location, though many cases have membranes, lymph nodes, and bone marrow, where
been reported in the jaws (Fig. 7-55). The tumor they act as part of the immune system that helps de-
causes a single unilocular radiolucency with jaw stroy foreign antigens. Langerhans cell histiocytosis is
Figure 755 Plasmacytoma. Enlargement within Figure 756 Multiple myeloma x-ray. Punched-out
maxillary vestibule with loss of underlying bone. radiolucencies associated with bone destruction.
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Chapter 7 Neoplasms of the Oral Soft Tissues and Facial Skin 265
a neoplastic process that is characterized by prolifera- whose diagnosis is made at an older age. Younger pa-
tion of abnormal Langerhans cells in their normal tients tend not to do as well; however, death from this
anatomic locations. disease rarely occurs.
Two basic forms are named based on how wide- The acute disseminated form is called Letterer-Siwe
spread lesions are. The chronic localized form is confined disease and is seen in neonates. The disease is rapidly
to one bone (monostotic) and is referred to as eosinophilic progressive and lethal. Patients generally do not reach
granuloma. This form is seen mainly in adults as local- age 2.
ized bone destruction (Fig. 7-57A and B). Frequently,
the Langerhans cells of the overlying mucosa are also
affected, resulting in severe ulcerative mucositis. Early
lesions resemble localized periodontitis that does not
respond to conventional periodontal therapy. Treat- Critical Thinking Questions
ment of eosinophilic granuloma usually involves curet- Case 7-1: Your 55-year-old patient informs you he
tage of bone lesions, radiation, or local injections of has just been diagnosed with chronic myelogenous
corticosteroids. leukemia (CML). His presenting signs and symptoms
The chronic disseminated form is named Hand- included fatigue, weight loss, and decreased exercise
Schller-Christian disease after the three doctors who tolerance. He is just starting chemotherapy and wants
first described it. This form is seen mainly in children his teeth cleaned.
younger than 15 years of age who develop multiple
lesions that involve the skin, mucosa, bone, deep
visceral organs, and/or lymph nodes. Lesions can be What are some oral manifestations of leukemia you might
found in multiple bones (polyostotic). When the jaws are encounter during his visit?
affected, radiographs show teeth that appear to be How does leukemia affect the patients ability to fight
floating in bone that is destroyed around them. The infection? What impact will this have on his oral
overlying soft tissues will appear ulcerated as in health in terms of caries? Periodontal disease?
eosinophilic granuloma. What is his long-term outlook? How will this impact his
The chronic disseminated form is more difficult to future dental care?
treat than the localized form. Patients may require
chemotherapy. Prognosis is better for individuals
266 Chapter 7 Neoplasms of the Oral Soft Tissues and Facial Skin
Case 7-3: A 64-year-old female comes in for a What are some potential radiation side effects this patient
may experience in the oral cavity?
routine dental examination. She spends a great deal of
time outdoors gardening, sailing, and playing golf. You How might these complications affect the patients quality
observe dryness, white-colored sun damage, and an in- of life? Oral health?
distinct vermillion border to her lower lip. One area Is this patient at risk for developing additional intraoral
appears crusted but it is nontender and not hard or in- cancers? Why?
durated. When you palpate her neck, no firm lymph
nodes are found.
Review Questions
1. Oncogenes cause transformation of normal 3. Metastasis of tumors
cells and A. is synonymous with infiltration.
A. cause cell death. B. occurs regularly with both benign and ma-
B. prevent cells from growing on their own. lignant neoplasms.
C. are capable of repairing damaged DNA. C. with rare exception, is associated with ma-
D. are capable of causing a neoplasm. lignant tumors.
D. results in a neoplasm that does not resemble
2. Neoplastic and reactive processes the original tumor.
A. differ in that reactive processes generally
resolve when the originating stimulus is 4. What percent of all oral cancers are squamous
removed. cell carcinomas?
B. both produce damage to the cells DNA A. 5 percent
that results in gene alterations. B. 25 percent
C. are similar in that they both progress to C. 60 percent
tumorigenesis. D. 90 percent
D. both require ongoing stimulus for contin-
ued cell growth.
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Chapter 7 Neoplasms of the Oral Soft Tissues and Facial Skin 267
5. As a general pathology term, dysplasia means 11. Which one of the following is a benign neo-
A. caused by friction. plasm arising from the myelin-producing
B. loss of normal maturation of cells and tissues. cells that surround and support peripheral
C. precancerous. nerves?
D. caused by sunlight. A. Neurofibrosarcoma
B. Schwannoma
6. Of the locations listed below, which one is the C. Myofibroma
most common intraoral site for squamous cell D. Hemangioma
carcinoma?
A. hard palate. 12. Which one of the following is a malignant
B. floor of the mouth. tumor of striated skeletal muscle?
C. gingiva. A. Leiomyoma
D. lateral tongue. B. Leiomyosarcoma
C. Rhabdomyoma
7. Which intraoral neoplasm is characterized by D. Rhabdomyosarcoma
a thick, wart-like surface texture and is seen
most often in smokeless tobacco users? 13. Leukemia patients may present with bleeding
A. Myofibroma gums and bruising. This is related to
B. Rhabdomyosarcoma A. excess production of red blood cells in the
C. Verrucous carcinoma spleen.
D. Hemangioma B. decreased platelets for clotting (thrombo-
cytopenia).
8. Therapeutic radiation-induced bone death C. onset of fatigue, weight loss, and decreased
A. is known as osteoradionecrosis. exercise tolerance.
B. heals completely over time. D. a relative increase in thrombocytes.
C. is due to radiation-induced xerostomia.
D. can be prevented by the use of sialogogues. 14. Which one of the following is a malignancy of
plasma cells that may show multiple punched
9. Cumulative sun exposure may leave large out radiolucencies in bone?
areas of skin of a person with skin cancer at A. Burkitts lymphoma
risk for developing new skin cancers. This B. Multiple myeloma
phenomenon is known as C. Plasmacytoma
A. programmed cell death. D. Hodgkins lymphoma
B. Mohs phenomenon.
C. micro-invasion potential. 15. Radiographs of a 5-year-old child with
D. field cancerization. Langerhans cell histiocytosis show teeth that
appear to be floating in the jaws. What
10. Which one of the following is the most com- name is given to the chronic disseminated
mon salivary gland tumor? form of this disease?
A. Mucoepidermoid carcinoma A. Eosinophilic granuloma
B. Polymorphous low-grade adenocarcinoma B. Hand-Schller-Christian disease
C. Pleomorphic adenoma C. Letterer-Siwe disease
D. Warthin tumor D. Bence-Jones disease
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268 Chapter 7 Neoplasms of the Oral Soft Tissues and Facial Skin
16. Which one of the following terms is used to Regezi, J, Scuibba, J, Jordan, R: Oral Pathology: Clinical Pathologic
describe abnormal cells that show changes Correlations, ed. 4. Saunders, Philadelphia, 2007.
Robbins, SL, Kumar, V, Abbas, AK, Cotran, RS, et al: Pathologic
that are considered cancerous? Basis of Disease, ed. 10. Saunders Elsevier, Philadelphia, 2010.
A. Anaplastic Rubin, E, Reisner, H: Essential Pathology, ed. 5. Lippincott,
B. Well-differentiated Williams and Wilkins, Philadelphia, 2008.
C. Hyperchromatic
D. Metastatic Journal Articles
Abla, O, Egeler, RM, Weitzman, S: Langerhans cell histiocytosis:
Current concepts and treatments. Cancer Treatment Review
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not be attributed to any specific source or Aziz, SR: Coming to America: Betel nut and oral submucous fibrosis.
cause is known as Journal of the American Dental Association 141:423428, 2010.
Berk, LB, Shivnani, AT, Small, W: Pathophysiology and manage-
A. carcinoma. ment of radiation-induced xerostomia. Journal of Supportive
B. leukoplakia. Oncology 3:191200, 2005.
C. erythroplakia. Ferner, RE: Neurofibromatosis 1 and neurofibromatosis 2: A
D. carcinoma-in-situ. twenty-first century perspective. Lancet Neurology 6:340351,
2007.
Geller, AC, Annas, GD: Epidemiology of melanoma and non-
18. Epithelial disorders with malignant potential melanoma skin cancer. Seminars in Oncology Nursing 19:211,
include all of the following EXCEPT 2003.
A. dermatosis papulosa nigra. Geraminejad, P, Memar, O, Aronson, I, Rady, PL, et al: Kaposis
sarcoma and other manifestations of human herpes virus 8.
B. proliferative verrucous leukoplakia.
Journal of the American Academy of Dermatology 47:641655,
C. actinic keratosis. 2002.
D. oral submucous fibrosis. Jose, BO, Koerner, P, Spanos, WJ, Paris, KJ, et al: Hodgkins lym-
phoma in adults - Clinical features. Journal of the Kentucky
19. Which one of the following is true of basal Medical Association 103:57, 2005.
Philipsen, HP, Reichart, PA: The development and fate of epithelial
cell carcinomas?
residues after completion of human odontogenesis with special
A. They occur 90 percent of the time in the reference to the origins of epithelial odontogenic neoplasms,
oral cavity. hamartomas and cysts. Oral Biosciences and Medicine1:171179,
B. They do not commonly metastasize. 2004.
Pulte, D, Brenner, H: Changes in survival in head and neck can-
C. They do not invade or expand into vital cers in the late 20th and early 21st century: A period analysis.
structures. Oncologist 15(9):9941001, 2010.
D. They are considered to be premalignant. Rosenthal, DI, Trotti, A: Strategies for managing radiation-induced
mucositis in head and neck cancer. Seminars in Radiation Oncology
20. All of the following are considered benign soft 19:2934, 2009.
Simard, EP, Engels, EA: Cancer as a cause of death among people
tissue neoplasms EXCEPT with AIDS in the United States. Clinical Infectious Diseases
A. myofibroma. 51(8):957962, 2010.
B. lipoma. Smith, MA, Seibel, NL, Altekruse, SF, Ries, LA, et al: Outcomes
for children and adolescents with cancer: Challenges for the
C. schwannoma.
twenty-first century. Journal of Clinical Oncology 28:26252634,
D. fibrosarcoma. 2010.
C h a p t e r
8
Neoplasms of the Bones of
the Mandible and Maxilla
Learning Outcomes
At the end of this chapter, the student will be able to:
8.1. Define all key terms in the chapter. 8.6. Explain why compound and complex
8.2. Explain the difference between an odontomas have different radiographic
odontogenic neoplasm and a features.
nonodontogenic neoplasm. 8.7. Explain why peripheral odontogenic
8.3. Give examples of odontogenic tumors tumors have a different prognosis
that occur mainly in adults and those than central odontogenic tumors.
that occur mainly in children. 8.8. Recognize the key radiographic
8.4. Identify on a radiograph the key features of osteoma versus
features of odontogenic neoplasms. osteosarcoma.
Explain why some tumors appear 8.9. Give examples of malignant tumors
multilocular. of the body that are known to
8.5. Describe two odontogenic tumors metastasize to the jaws.
that can mimic periodontitis
radiographically.
269
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NEOPLASMS OF THE BONES OF THE the portion of the developing tooth germ from which
MAXILLA AND MANDIBLE each odontogenic neoplasm is believed to arise.
Many different neoplasms (tumors) arise within the With rare exceptions, odontogenic tumors are found
maxilla and mandible. Primary jaw tumors are those in the tooth-bearing areas of the jaws. Odontogenic
that originate in the jaw bones. They are either odon- tumors closely approximate teeth but they do not al-
togenic (arising from tooth-forming tissues) or non- ways affect the health of adjacent teeth. Teeth remain
odontogenic (not arising from tooth-forming tissues). vital, though they may be displaced. Odontogenic
Many other neoplasms may arise in this location, but tumors are either central or peripheral. Most odonto-
this chapter focuses on odontogenic tumors and genic tumors are central, meaning they develop within bone
tumors of bone and cartilage tissue. Tumors that of the mandible or maxilla. Some odontogenic tumors are
develop in distant sites and metastasize to the jaws will peripheral, meaning they develop outside the bone cortex in
also be briefly discussed. overlying soft tissues. Overall, peripheral odontogenic tumors
Many primary jaw tumors are found incidentally on are less aggressive and more easily treated than central
radiographs or other types of imaging. Early symp- odontogenic tumors.
toms may include pain, bone expansion and swelling, Classification of odontogenic tumors can be based
numbness, drifting of teeth, and changes in occlusion. on tissue of embryologic origin (see Fig. 8-1), age
Most jaw tumors require surgical management. Benign (Table 8-1), or radiographic appearance (see Table 8-1).
tumors tend to be encapsulated, making surgical However, the most clinically useful classification is
removal less of a challenge than those with no capsule. by age and radiographic appearance. Often these are
Often, infiltrating tumors with a high rate of recur- used together in formulating a differential diagnosis
rence may require resection (removal) of a section of or a list of possible tumors. For example, a radiolucent
the jaw followed by reconstruction to replace the lost lesion in a pediatric patient could be cystic ameloblas-
tissue. Occasionally, teeth will need to be removed and toma, ameloblastic fibroma, or keratinizing cystic
replaced with dental prosthetics and/or implants. odontogenic tumor.
There are many odontogenic tumors; only the most
ODONTOGENIC NEOPLASMS common ones are discussed in this chapter. Odonto-
genic tumors seen more commonly in adults are
Odontogenic neoplasms arise from remnants of the de-
discussed first.
veloping tooth germ that undergo neoplastic transfor-
mation. (See Chapter 7 for a discussion of neoplastic
transformation.) A review of the histologic develop- Ameloblastoma
ment of teeth will provide insight into the complex The most common benign odontogenic tumor is
structure of the developing tooth. Figure 8-1 indicates ameloblastoma. It is characterized by the proliferation
Figure 81 Odontogenic tumors are derived from cells of the tooth germ.
2577_Ch08_269-286 09/07/14 3:36 PM Page 271
of cells resembling three of the four layers of is a multilocular soap-bubble appearance (Fig. 8-2A).
cells constituting the enamel organ: outer enamel Early lesions may appear unilocular (Fig. 8-2B).
epithelium, stellate reticulum, and inner enamel Patients present with a painless swelling of the jaw,
epithelium, which forms the ameloblasts. Because which can grow to dramatic proportions if left
no dental hard tissues form, ameloblastomas are untreated (Fig. 8-2C). As the tumor expands, it can
radiolucent. destroy or thin the buccal and/or lingual cortex,
There are three types of ameloblastoma: multicystic producing an egg shell consistency (Fig. 8-2D).
solid, cystic intraosseous, and peripheral. Each has a Ameloblastomas do not form capsules even though
different clinical and radiographic presentation, treat- they are benign tumors. This allows them to insidi-
ment, and prognosis. ously infiltrate bone, complicating surgical removal.
Microscopic remnants of tumor left behind after
Multicystic Solid Ameloblastoma surgery can lead to recurrence years later.
Multicystic solid ameloblastoma, sometimes called Treatment is complete surgical excision. This may
conventional ameloblastoma, is the most worrisome of necessitate removal of large amounts of bone depend-
the three types due to its aggressive and recurrent na- ing on tumor size. Reconstruction with bone grafting
ture. It is rarely seen in patients under age 20 or over may be necessary. Recurrence is a serious risk and
age 40. The pathogenesis of ameloblastomas remains patients must be followed throughout their lives.
controversial. Ameloblastomas may occur in any loca- When malignant transformation of ameloblastoma
tion within the jaws but are most common in the pos- occurs, the malignant tumor is called ameloblastic
terior mandible. The classic radiographic appearance carcinoma. Most patients who experience ameloblastic
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A B
C D
Figure 82 Multicystic ameloblastoma. A. Multilocular radiolucency in the posterior mandible that extends into the
ramus and TMJ. B. Early ameloblastoma with unilocular appearance. C. Clinical appearance of painless enlargement
of the left mandible and malpositioning of teeth. D. Ameloblastoma: Gross specimen. Ameloblastoma of the mandible
with lingual bone removed; bony walls produce multilocular radiographic appearance.
carcinoma have had a previous long-term history of of origin is within bone. Unlike multicystic solid
ameloblastoma, usually with multiple recurrences. ameloblastoma, peripheral ameloblastomas are not
Benign ameloblastomas have been found in extrao- aggressive, are easily treated with conservative local
ral sites. Many believe these are metastatic lesions excision, and pose little risk for recurrence. Peripheral
from the jaws; however, this activity is unusual for ameloblastomas must be differentiated from intraosseous
benign tumors. ameloblastomas because treatment and prognosis
differs significantly.
Peripheral Ameloblastoma
Peripheral ameloblastomas originate within the gingival Calcifying Epithelial Odontogenic Tumor
soft tissues of adults. Patients present with a painless, Calcifying epithelial odontogenic tumor (CEOT) is a
firm nodular swelling of the gingiva, most often in benign tumor composed of cells that resemble stratum
the mandibular premolar area, that has a smooth or intermedium of the enamel organ. Cells resembling
papillary surface (Fig. 8-3A). There is no bone involve- ameloblasts are not seen. CEOT is comparable to
ment and radiographs appear normal (Fig. 8-3B). ameloblastoma in age, location, and treatment. CEOT
Occasionally, multicystic solid ameloblastomas perfo- presents as a painless, slow-growing mass of the jaw
rate the cortex and cause gingival swelling. These are (Fig. 8-4A). Radiographic appearance is that of a uniloc-
not considered peripheral tumors because the point ular or multilocular radiolucency with a well-defined
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B
Figure 83 Peripheral ameloblastoma. A. Firm nodular
mass on lingual surface of mandible; underlying bone was
intact. B. Periapical radiograph shows no evidence of
intrabony lesion.
B
but noncorticated border (Fig. 8-4B), containing scat-
tered radiopacities. Significant expansion and a hon-
eycombed (Fig. 8-4C) appearance may be present.
The scattered radiopacities help differentiate it from
ameloblastoma. Treatment involves wide surgical ex-
cision and close clinical follow up for recurrence.
radiographic appearance. Myxomas frequently develop are considered a neoplasm, then they are the most
along the lateral aspect of teeth where early lesions can common odontogenic neoplasm, exceeding the
mimic periodontal bone loss (Fig. 8-5C). The tumor number of all other odontogenic neoplasms com-
is not encapsulated and has a gelatinous consistency. bined. They present as well-defined masses com-
This allows easy infiltration of the marrow spaces, posed of a combination of all tooth-forming and
making complete removal very difficult and recurrence hard dental tissues. The two basic types of odon-
common. tomas are described next.
Compound Odontoma
Clinical Implications
Compound odontoma is a well-defined encapsulated
Squamous odontogenic tumor and odontogenic mass of tiny toothlets, miniature teeth, often 4 to 8
myxoma should be suspected when there is a mm in length, with identifiable crowns and roots.
non-inamed isolated periodontal defect in an Compound odontomas may be seen anywhere in the
otherwise healthy adult mouth. jaws but favor areas where deciduous teeth are or have
been located. They may impede the eruption of per-
Odontogenic tumors seen predominantly in the pe- manent teeth, which is how they are often first recog-
diatric population are discussed next. nized. Radiographs show a radiolucent follicular
sac-like structure (Fig. 8-6A) filled with identifiable
toothlets (Fig. 8-6B). Treatment is surgical removal
Odontoma
and they do not recur.
Controversy exists as to whether odontomas represent
neoplasms or developmental hamartomas. If they Complex Odontoma
Complex odontoma is a well-defined, encapsulated,
disorganized, jumbled mass of mature tooth tissues
with no identifiable toothlets (Fig. 8-7A). Radi-
ographically, it appears as a radiodense structure with
a radiolucent rim that may be misinterpreted as a
more ominous tumor (Fig. 8-7B). The diagnosis is
often not apparent until it is removed and examined
microscopically. Once removed, they do not recur.
Occasionally, a compound odontoma may occur in
conjunction with a complex odontoma. When this
A happens, the term compound/complex odontoma is used
(Fig. 8-8).
Clinical Implications
Because odontomas are essentially impacted
teeth, they are vulnerable to the same problems
as other impacted or unerupted teeth. For example,
dentigerous cysts may develop within the follicular
sacs surrounding odontomas. Surgical removal not
only allows eruption of blocked teeth but prevents
further associated pathologic changes.
B C
Figure 85 Odontogenic myxoma. A. Swelling between
maxillary central incisors. B. Myxoma lateral slice radiograph. Cystic Ameloblastoma
Same case as 4A. Radiolucent lesion pushing cortex toward
facial surface. C. Myxoma periapical. Destruction of bone Cystic ameloblastoma (formerly termed unicystic
by odontogenic myxoma mimics periodontal bone loss. ameloblastoma) occurs in individuals under age 20, much
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A
A
B
Figure 87 Complex odontoma. A. Fragments of
calcified tooth material with no identifiable toothlets.
B B. Complex odontoma radiograph. Radiopaque mass with
no identifiable toothlets.
Figure 86 Compound odontoma. A. Compound
odontoma impeding eruption of maxillary incisors; toothlets
are clearly visible. B. Toothlets from compound odontoma;
crowns and roots recognizable.
Clinical Implications
The typical case scenario of AOT is an adolescent
girl with an impacted maxillary canine tooth. A
nonpainful, well-dened, mixed radiolucent/
radiopaque lesion is present surrounding the
crown and extending down along the root
surface below the CEJ. Figure 811 Ameloblastic fibroma. Eruption of molars
is impeded by radiolucent ameloblastic fibroma in a child.
2577_Ch08_269-286 09/07/14 3:37 PM Page 277
Clinical Implications
There are major clinical dierences between
ameloblastoma and ameloblastic broma.
Ameloblastoma is an aggressive recurrent lesion
that often requires major surgery and close A
clinical follow up throughout the patients life
span. AFs and AFOs in general require more
conservative surgical treatment and recurrence
is not common.
Cementoblastoma
Cementoblastoma is a benign neoplasm of cementum. It
presents in patients younger than age 30 as a solid,
radiopaque mass found most frequently around the apex
of a tooth. The neoplasm is most frequently associated
with the mandibular first molars (Fig. 8-13A). Deciduous
B
Figure 813 Cementoblastoma. A. Well-defined mixed
radiolucent/radiopaque mass attached to the roots of mandibular
first molar. B. Cementoblastoma CT scan. CT scan showing
expansion of mandible with cementoblastoma.
NEOPLASMS OF BONE
In addition to odontogenic tumors of the jaws, neo-
plasms of the bone itself can develop. Neoplasms of
bone often cause expansion. This is detectable by the
patient who presents to the dental office with a chief
complaint of swelling. Expansion of bone may also
occur when tumors from other parts of the body
metastasize to the jaws.
C
Osteoma Figure 814 Keratinizing cystic odontogenic tumor
Osteomas are benign tumors composed of mature bone (KCOT)/odontogenic keratocyst. A. Movement of teeth
and swelling of jaw with KCOT. B. Large unilocular
tissue. They are rarely seen outside the bones of the radiolucency and resorption of tooth roots. C. Multilocular
head and neck. They may resemble torus palatinus and radiolucent lesion originally thought to be an ameloblastoma.
torus mandibularis; however, tori are development Biopsy showed KCOT.
2577_Ch08_269-286 09/07/14 3:37 PM Page 279
A B
C D
Figure 818 Osteosarcoma. A. Swelling of maxilla with ulceration of mucosa. B. Early osteosarcoma destroying
lamina dura of lateral incisor. C. Same patient as A. Tumor in the right maxilla (arrow). D. CT of same patient that
shows extent of the tumor.
Metastasis of Other Tumors to Bone is occasionally identified and diagnosed before the dis-
Carcinomas from distant sites may favor metastasis to covery of the primary tumor.
bone, including the maxilla and mandible. The most Signs and symptoms vary widely depending on the
common tumors to metastasize to the jaws are adeno- location of the metastasis. Patients may complain of
carcinomas from the breast, prostate, thyroid, gas- swelling, loose teeth, and pain or numbness if the
trointestinal (GI) tract, and lung, as well as carcinomas tumor impacts the mandibular nerve. Radiographs typ-
from the kidney and lung (Table 8-2). Sarcomas other ically show ill-defined radiolucent alterations of the
than osteosarcoma rarely metastasize to bone. In most bone, sometimes described as moth eaten (Fig. 8-19).
cases, patients present with a previous history of car- Occasionally dystrophic calcifications may be seen
cinoma. Occasionally, metastasis of cancer to the jaws in breast and prostate cancer metastases. When the
2577_Ch08_269-286 09/07/14 3:37 PM Page 282
Table 8.2 Origin of Metastases to the Bone nasal septum, and occasionally the TMJ. They require
of the Jaws surgical removal and rarely recur. Multiple chondro-
mas are characteristic of several syndromes for which
Men Women the patient should be assessed.
Chondrosarcoma is cancer of cartilage composed of
Lung: 25% Breast: 36.6%
anaplastic chondroblasts producing immature cartilage
Kidney: 10.8% Genital organs (uterus, that fails to calcify properly. Chondrosarcomas may be
Liver: 8.6% ovaries, cervix, fallopian found in the anterior maxilla, nasal cartilage, sinuses,
tubes): 9.5% TMJ, and laryngeal cartilage of adults. Jaw tumors
Prostate :7.5%
Kidney: 8.5% cause bone expansion with loosening and resorption
Bone: 7.5%
Colorectum: 7.1% of teeth, but patients rarely complain of pain. Radi-
Adrenal gland: 5.3% ographic images show a poorly defined radiolucency
Bone: 6.7%
Colorectum: 4.7% with scattered radiopacities that may enlarge as the
Adrenal gland: 5.8% tumor progresses. Prognosis depends on surgical re-
Testis: 4.4%
Thyroid: 5.4% moval because chemotherapy and radiotherapy are not
Esophagus: 3.6%
Rare tumors: 20.4% always effective. Five-year survival rates are greater
Stomach: 2.5% than 60 percent but the rates decrease in later years
Bladder: 2.5% due to propensity for recurrence.
Rare tumors: 17.0%
Source: http://emedicine.medscape.com/article/1079102-overview#a0199,
July 2012. Critical Thinking Questions
Case 8-1: Your 14-year-old female patient pres-
ents to your oce with failure of eruption of tooth #6.
Her jaw is slightly swollen in the area. A radiograph
shows that the tooth is impacted. The well-defined,
well-corticated radiolucent lesion surrounding it
shows some fine radiopaque flecks.
Name two possible odontogenic neoplasms this may received radiation and chemotherapy. She has been
represent. disease-free for 13 months. During scaling, you no-
Because the swelling is not being caused by a third molar, tice that tooth #29 and tooth #30 have +1 mobility.
how do you explain the patients symptoms? Radiographs show a diffuse loss of bone density at
Your patient states he does not want to do anything the apices of the teeth. They respond positively to
about it now because he is about to leave for training. percussion tests. There is no tenderness or swelling
He proudly tells you he is planning on trying out for in the area.
the boxing team on the base.
Review Questions
1. Which one of the following is true of odonto- 3. Of the neoplasms listed below, which one is the
genic neoplasms? most common benign odontogenic tumor?
A. They include osteomas and osteosarcoma A. Ameloblastoma
of the jaws. B. Ameloblastic carcinoma
B. They arise from remnants of the developing C. Calcifying epithelial odontogenic tumor
tooth germ. D. Odontogenic myxoma
C. They include those that develop in a distant
site and metastasize to the jaws. 4. All of the following are true about multicystic
D. They are always malignant. solid ameloblastoma EXCEPT
A. the posterior mandible is the most common
2. Most odontogenic tumors are central. This location.
means they B. it is the most worrisome type of
A. originate in the soft tissues covering the ameloblastoma.
jaws. C. recurrence is rare.
B. closely approximate the center of the D. it is the conventional or most common
ramus. form of ameloblastoma.
C. are made up of mandibular or maxillary
bone.
D. develop within the bone of the jaws.
2577_Ch08_269-286 09/07/14 3:37 PM Page 284
5. Which one of the following is true of peripheral 10. Odontogenic keratocyst has recently been
ameloblastomas? reclassified as an odontogenic neoplasm. The
A. They originate within the soft tissues of the new name is
gingiva in adults. A. calcifying cystic odontogenic tumor.
B. They are aggressive and require wide B. keratinizing cystic odontogenic tumor.
surgical excision. C. ameloblastic cystic fibro-odontoma.
C. They are known to frequently recur after D. cystic odontogenic myxoma.
treatment.
D. They have a tendency to metastasize to the 11. What benign tumor is composed of mature
lung. bone and may resemble a torus?
A. Osteoblastoma
6. Under which one of the following circum- B. Osteosarcoma
stances can squamous odontogenic tumor and C. Osteoma
odontogenic myxoma be suspected? D. Chondroma
A. If the patient is under 12 years of age.
B. When there is a non-inflamed isolated 12. Multiple osteomas of the jaws are seen in
periodontal defect in an otherwise healthy what syndrome?
mouth. A. Gardners syndrome
C. When an encapsulated mass of tiny tooth- B. Nevoid basal cell carcinoma syndrome
lets is present. C. Chondroblastic osteosarcoma syndrome
D. When there is a well-defined radiolucency D. Ameloblastic cell syndrome
with snowflake calcifications.
13. All of the following are true about osteosar-
7. Compound odontomas coma EXCEPT
A. are well-defined encapsulated masses of tiny A. occurrence of jaw tumors tends to peak
toothlets. between 30 and 35 years of age.
B. may occur as both benign and malignant B. there is a direct link with tobacco and
neoplasms. alcohol use.
C. are well-defined, encapsulated, disorgan- C. it is a cancer of bone with osteoblasts
ized, jumbled masses of mature tooth undergoing malignant transformation.
tissues. D. swelling and pain are the most common
D. are not considered odontogenic. symptoms.
8. Which one of the following odontogenic tu- 14. A sunburst appearance is seen radiograph-
mors often occurs around the crown of an ically in approximately 25 percent of cases of
unerupted maxillary canine in an adolescent fe- which one of the following neoplasms?
male?
A. Osteosarcoma
A. Cystic ameloblastoma B. Osteoma
B. Adenomatoid odontogenic tumor C. Ameloblastoma
C. Squamous odontogenic tumor D. Compound odontoma
D. Odontogenic myxoma
15. Which one of the following tumors may
9. Which one of the following is a benign ra- metastasize to the mandible?
diopaque lesion that may be found around the
A. Breast cancer
apex of a tooth in a patient younger than 30?
B. Prostate cancer
A. Ameloblastic fibro-odontoma C. Lung cancer
B. Ameloblastoma D. All of the above
C. Odontogenic myxoma
D. Cementoblastoma
2577_Ch08_269-286 09/07/14 3:37 PM Page 285
C h a p t e r
9
Systemic Pathology and Oral
Manifestations of Systemic Diseases
Learning Outcomes
At the end of this chapter, the student will be able to:
9.1. Illustrate by examples how a Review endocrinopathies when onset occurs
of Systems can impact the dental in adulthood versus childhood.
hygiene appointment. 9.4. Discuss oral and systemic manifesta-
9.2. Describe systemic and oral manifes- tions of diabetes mellitus, and relate
tations of endocrine gland dysfunc- how this disorder impacts patients
tion, including endocrinopathies of and dental treatment.
the pituitary, adrenal, pancreas, 9.5. Compare the hemoglobinopathies in-
thyroid, and parathyroid glands. cluding their etiologies, clinical features,
9.3. Contrast the difference in oral laboratory findings, and dental appoint-
and systemic manifestations of ment management considerations.
Continued
287
2577_Ch09_287-316 09/07/14 3:38 PM Page 288
9.6. Describe the types and causes of 9.9. Explain how jaundice develops and
anemia, and laboratory tests used in its significance.
evaluation of red blood cells. 9.10. Discuss how connective tissue disor-
9.7. Discuss the different types of bleed- ders affect dental treatment.
ing disorders and their impact on 9.11. Compare systemic and oral features
dental care. of hyper-vitamin states and vitamin
9.8. Describe systemic and oral manifes- deficiencies.
tations of gastrointestinal tract 9.12. Explain how vitamin C and vitamin D
dysfunction, including the main deficiencies impact the oral hard and
difference between inflammatory soft tissues.
bowel disease and Crohns disease.
Hypothalamus
Releasing hormones
for anterior pituitary
Pineal gland
Pituitary (Hypophysis) Melatonin
gland
Anterior: Thyroid gland
Growth hormone Thyroxine and T3
Thyroid stimulating hormone Calcitonin
Adrenocorticotropic hormone
Follicle stimulating hormone Parathyroid glands
Luteinizing hormone Parathyroid hormone
Prolactin
Posterior:
Antidiuretic hormone
Oxytocin Thymus gland
Immune hormones
Adrenal (Suprarenal)
glands
Cortex: Pancreas
Aldosterone Insulin
Cortisol Glucagon
Sex hormones
Medulla:
Epinephrine
Norepinephrine
Ovaries
Testes
Estrogen
Testosterone
Progesterone
Inhibin
Inhibin
Figure 91 The endocrine system and its glands. (From Tamparo, CD: Diseases of
the Human Body, ed. 5. F.A. Davis, Philadelphia, 2011, p 284.)
Anterior Pituitary Growth hormone (GH) Mainly liver, bones, Overall growth and Excessive GH
muscles protein, fat and - Gigantism
glucose homeostasis - Acromegaly
Deciency of GH
- Dwarsm
Adrenocorticotrophic Adrenal gland Stimulates the Excess ACTH
hormone (ACTH) (cortex) production of - Cushings syndrome
glucocorticoids Deciency of ACTH
- Addisons disease
Melanocyte stimulating Melanin-producing Stimulates the produc- Seen in Addisons
hormone (MSH) cells, usually pres- tion of melanin, caus- disease
ent in the skin ing darkening of the
skin
Continued
2577_Ch09_287-316 09/07/14 3:38 PM Page 290
practice. Following is a brief discussion of selected morphologically normal but abnormally large. Enlarge-
disorders that may be encountered. ment of the jaws and generalized macrodontia are oral
Hyperpituitarism features commonly observed in gigantism.
Hyperpituitarism most often refers to an excess produc-
Acromegaly
tion of hormones originating from the anterior lobe
of the pituitary gland. The most common cause is Elevation of growth hormone after the closure of bone
pituitary adenoma, a benign neoplasm that overpro- (epiphyseal) growth plates results in acromegaly. Adults
duces hormones. Other causes include hyperplasia of with this condition exhibit generalized enlargement of
the gland or other pituitary and nonpituitary tumors. bones of the skeleton and soft tissues. Gradually
Disease manifestations depend on the type of pituitary enlarging hands, feet, and head are common features.
cells affected. For example, overproduction of growth Bone and soft tissue growth in the face creates coarse
hormone from the anterior pituitary during childhood features with an increasing lower facial height, enlarge-
results in gigantism, or acromegaly after the individual ment and protrusion of the mandible, enlarged tongue,
has achieved their growth potential. and widening of interproximal spaces between normal
sized teeth. Excess growth hormone production is a se-
Gigantism rious condition that contributes to many other systemic
Elevation of growth hormone levels in children before problems including hypertension, diabetes mellitus, os-
they reach their normal growth potential results in teoporosis, arthritis, and congestive heart failure.
gigantism. Children with gigantism are extremely tall Blood tests revealing elevated growth hormone levels
for their age because the disorder occurs before the bone are required to confirm the diagnosis of gigantism or
(epiphyseal) growth plates are closed. They have abnormal acromegaly. Appropriate treatment of the underlying
height, long arms and legs, large hands, and dispropor- cause is required. Early detection is vital to prevent serious
tionate body size. In the oral cavity, oral structures appear complications, which can ultimately result in death.
2577_Ch09_287-316 09/07/14 3:38 PM Page 291
Exophthalmos
(protruding eyes)
Goiter (enlarged
thyroid gland)
Both forms of hypothyroidism are diagnosed The classic signs and symptoms of primary hyper-
by measuring free thyroid hormone levels in the blood. parathyroidism are referred to as stones, bones, and
Management involves supplementation with synthetic groans. Stones refers to kidney stones. Individuals have
hormones. Early detection is critical to implementing increased risk of developing renal (kidney) stones due
treatment, mitigating permanent disability, and facili- to elevated serum calcium levels (hypercalcemia) caused
tating recovery where possible. by excess parathyroid hormone. This can result in kid-
ney failure. Bones refers to bone-related pathology.
Parathyroid Glands Bone and/or root resorption causes a ground-glass
The parathyroid glands are found attached (para, radiographic appearance similar to that seen in fibrous
meaning alongside) to each lobe of the thyroid gland, dysplasia. Additionally, loss of the lamina dura can be
hence the name parathyroid. However, despite the seen in dental radiographs (Fig. 9-4). In chronic cases,
name, their function is unrelated to thyroid function. especially with secondary hyperparathyroidism,
These glands help synthesize parathyroid hormone unilocular or multilocular radiolucencies may be seen
(PTH), which plays an important role in the regula- (Fig 9-5A), sometimes accompanied by jaw swelling
tion of serum calcium and bone health. When serum (Fig. 9-5B). These lesions are known as brown
calcium levels decrease, release of PTH from the
parathyroid glands occurs. PTH acts to elevate the
serum calcium by:
Mobilizing calcium from bone
Enhancing adsorption of calcium from the small
intestine
Decreasing calcium loss in the urine Figure 94 Hyperparathyroidism. Loss of lamina dura
in a patient with hyperparathyroidism.
Diseases of the parathyroid glands are clinically
evident when PTH levels are abnormally increased or
decreased. This may be due to insufficient parathyroid
hormone production related to disease states, parathy-
roid tumors, or loss of the parathyroid glands after
thyroid surgery.
Hyperparathyroidism
Hyperparathyroidism is caused by abnormal increased
synthesis and secretion of PTH. It may be primary or
secondary. Primary hyperparathyroidism involves overpro-
duction of PTH due to a benign or malignant tumor or
non-neoplastic hyperplasia of the parathyroid gland. Pri-
mary hyperparathyroidism most often occurs in adults,
predominantly women. Secondary hyperparathyroidism is
A
often a consequence of prolonged decreased calcium lev-
els (hypocalcemia) that leads to the continuous stimulation
of the parathyroid glands to compensate for the low cal-
cium levels. The most common cause of secondary hy-
perparathyroidism is chronic renal failure. Because the
kidneys play an important role in calcium metabolism,
renal failure can lead to impaired active vitamin D pro-
duction. Vitamin D is essential for calcium absorption
in the gut. Reduced absorption of calcium from the gut
and decreased reabsorption of calcium within the renal B
tubules leads to a lower serum calcium level. Lower
Figure 95 Brown tumor of hyperparathyroidism.
serum calcium levels send a feedback message to A. Swelling of maxilla in patient with brown tumor of
the parathyroid glands to remove calcium from bones, hyperparathyroidism. B. Radiograph showing brown
making up for calcium lost from the kidneys. tumor in right mandible.
2577_Ch09_287-316 09/07/14 3:38 PM Page 294
Cushings Addisons
Emotional Postural
disturbance hypotension
Moon face
Changes in
Buffalo hump distribution
Osteoporosis of body hair
Hypertension Bronze
pigmentation
of skin
Thin, wrinkled skin
GI disturbances
Obesity
Weight loss
Other signs:
Adrenal crisis:
Adrenal tumor
or hyperplasia Profound fatique
Amenorrhea Dehydration
Muscle weakness Vascular collapse ( BP)
Renal shut down
Serum NA
Serum K
Muscle
Purpura weakness
Skin ulcers
(poor wound
healing)
Figure 96 Comparison of Cushings syndrome and Addisons disease. Comparison of adrenocortical hyperfunction
(Cushings syndrome, left) and hypofunction (Addisons disease, right).
Clinical Implications
Onset of Addisons disease is subtle and may not B
be noticed unless the patient is observed over Figure 97 Addisons disease pigmentation. A. Gingiva;
time. Changes in oral mucosal pigmentation, diffuse hyperpigmentation of the gingiva. B. Lips of
over multiple dental visits, arouses suspicion of patient with Addisons disease.
Addisons disease, especially in individuals with
little tendency for racial pigmentation. Acute ad-
renal crisis, a life-threatening event due to insuf- metabolism associated with abnormally high levels of
cient cortisol, may result from stress during glucose (sugar) in the blood. Hyperglycemia may re-
medical or dental treatment. This disorder re- sult from destruction or dysfunction of beta cells in
quires prompt recognition and diagnosis. the islets of Langerhans in the pancreas, which pro-
duce insulin. The function of insulin is to facilitate
The Pancreas and Diabetes Mellitus transport of glucose from the blood into the cells. In-
The pancreas is the endocrine organ responsible for sufficient insulin production causes glucose to accu-
controlling release of insulin. Insulin is a hormone mulate in the blood (hyperglycemia). Cells then have
produced by beta cells of the islet of Langerhans in re- no glucose for energy production and must resort to
sponse to elevated blood glucose. Insulin is central to metabolizing fat for energy. Fat metabolism produces
regulating carbohydrate and fat metabolism in the ketones, which accumulate in the body fluids. Increase
body. The pancreas also is an exocrine gland produc- in ketones in the blood is a hallmark of uncontrolled
ing pancreatic juices that aid with digestion. diabetes. Ketones cause decreased pH, dehydration,
and electrolyte imbalance, a condition known as dia-
Diabetes Mellitus betic ketoacidosis (Table 9-2). Individuals with ke-
Diabetes mellitus (DM) is the most common disease toacidosis may have a distinctive aroma to their
of pancreatic dysfunction. DM is of great interest to breath. The odor has been described as sweet like fruit
oral health-care providers because it can lead to nu- or resembling acetone in nail polish remover.
merous systemic complications such as cardiovascular Diabetes mellitus can be classified as type 1 or 2.
abnormalities, neuropathies, renal failure, and im- Type 1 diabetes mellitus, also known as insulin-dependent
munosuppression (Fig. 9-8). Diabetes is a disorder of diabetes mellitus, is an autoimmune disorder of the
2577_Ch09_287-316 09/07/14 3:38 PM Page 297
Cardiovascular
abnormalities
Renal failure
From Tabers Cyclopedic Medical Dictionary, ed. 21. F.A. Davis, Philadelphia, 2009, p 631.
pancreas with an unknown cause. Autoantibodies Table 9.3 Signs and Symptoms of Diabetes
(see Chapter 4) are directed against the beta cells in the Mellitus
islets of Langerhans. This results in insufficient or
absent insulin production. Type 1 diabetes mellitus Signs Symptoms
most commonly occurs before age 25 and there is no
Acanthosis nigricans Polydipsia (feeling of thirst)
gender predilection. Individuals with a family history
of type 1 diabetes mellitus or other autoimmune disor- Ketone breath Polyuria (frequent urination)
ders have an increased risk of developing diabetes. Gingivitis Polyphagia (feeling of hunger)
Type 2 diabetes mellitus, also known as noninsulin- Periodontitis Tender gingiva and
dependent diabetes mellitus, is the most common form bleeding on brushing
Oral candidiasis
of diabetes. It is characterized by insufficient insulin
Delayed wound healing Weight loss despite
production in relation to what is known as insulin re- increased appetite
sistance. Insulin resistance is a condition in which cells
of the body ignore or become resistant to the effects Malaise (lack of energy)
of insulin. The cells do not respond adequately to nor- Xerostomia
mal levels of insulin. A number of etiologic factors play Loss of strength
a role in the development of insulin resistance includ- Recurrent infections
ing genetics, obesity, sedentary lifestyle, poor eating
habits, and age. Recently increasing rates of obesity in Blurred vision
younger individuals have been associated with rising Peripheral neuropathy
rates of type 2 diabetes. (numbness and tingling of
Patients with diabetes mellitus may present with the hands and feet) includ-
generalized fatigue and lethargy, increased appetite ing burning oral pain
(polyphagia) with weight loss, frequent urination
(polyuria), and increased thirst (polydipsia). They
may also experience xerostomia, changes in vision, tin-
gling and numbness of fingers and toes, and burning
sensation of the tongue and lips (Table 9-3).
The oral manifestations of diabetes mellitus depend
on the extent of blood glucose control. Common oral
manifestations of poor glucose control include increased
risk of bacterial and fungal infections such as oral candidi-
asis. Patients may experience burning mouth, impaired
wound healing, xerostomia, and parotid gland enlarge-
ment. Xerostomia increases the risk for dental caries,
candidiasis (Fig. 9-9), traumatic or aphthous ulcers, and
cheilitis, also called perlche (Fig. 9-10). Diabetes mellitus
is considered a major risk factor for periodontal disease Figure 99 Candidiasis. Oral candidiasis and ulceration
because of decreased resistance to bacterial infection. in a diabetic patient.
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Clinical Implications
Antibiotic prophylaxis is common because infec-
tions predispose these individuals to sickle cell
crisis. Dental appointments should be short be-
cause prolonged stress may also trigger a crisis.
Use of local anesthetic without epinephrine may
be preferred to reduce the risk of tissue hypoxia.
Normal hemoglobin
2
2
Oxygen
molecule
2
D
eo
xy
ge
na Sickle
tio
n hemoglobin (Hb S)
Figure 913 Sickle cells. Structure of hemoglobin A and S and their effect on erythrocytes.
(From Tabers Cyclopedic Medical Dictionary, ed. 21. F.A. Davis, Philadelphia, 2009, p 119.)
of child-bearing age. The etiology of iron deficiency related to chronic blood loss due to gastric problems
anemia includes: such as ulcers or other unknown conditions including
malignancy.
Lack of iron intake in diet
Oral manifestations of iron deficiency anemia
Impaired absorption of iron
include:
Increased demand of iron by the body
Excessive acute or chronic blood loss Atrophic glossitis seen as a smooth, glossy tongue
(Fig. 9-14)
Deficiency of iron in the diet is common in the eld-
Oral candidiasis and angular cheilitis (cracking at
erly where it may be associated with poor dental health
commissures, see Fig. 9-10)
or decreased consumption of iron-rich foods such as
Burning sensation on tongue
meats and vegetables. Patients with gastrointestinal
Pallor of oral mucosa
(GI) malabsorption may develop chronic diarrhea
leading to poor iron absorption. Increased body de- Treatment of iron deficiency anemia involves
mand for iron is seen during pregnancy. Deficiency is dietary iron supplements. In individuals with GI
common in young children during growth spurts and malabsorption, iron may be administered via nasal
around puberty. In premenopausal women, heavy spray or by injections. Signs and symptoms of iron
menstrual bleeding may lead to iron deficiency due to deficiency anemia are usually reversed after iron
excessive blood loss. In males, iron deficiency may be supplementation.
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Pernicious Anemia
Pernicious anemia is considered an autoimmune dis-
order (see Chapter 4 for discussion of autoimmunity)
usually associated with atrophic gastritis. In this dis-
order, the immune system produces antibodies
against parietal cells in the stomach. Parietal cells
produce a protein called intrinsic factor, which is nec-
essary for the absorption of vitamin B12 in the ileum.
Vitamin B12 is needed for the production of hemo-
globin. In pernicious anemia, parietal cells are de-
stroyed and vitamin B12 is not absorbed. Patients
who have undergone gastrointestinal bypass surgery
or who are strict vegetarians are at risk for vitamin
B12 deficiency and developing pernicious anemia. Figure 915 Pernicious anemia. Painful, tender tongue
Early detection of vitamin B12 deficiency is very in patient with pernicious anemia.
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vessels. This means patients with PCV are at higher risk Hemophilia A
of a heart attack (myocardial infarction) or stroke. Hemophilia A is an inherited bleeding disorder asso-
Patients with PCV complain of fatigue, dyspnea, ciated with a lack of clotting factor VIII. It is the most
dizziness, and frequent headaches. Shortness of breath common type of hemophilia. Hemophilia A involves
with bluish discoloration of the skin (cyanosis) and hy- a gene that occurs on the X chromosome and is
pertension are common. PCV is treated with phlebotomy passed to a son as a sex-linked recessive trait from a
to maintain normal red blood cell volume in the blood. mother who carries the gene. Therefore, hemophilia
occurs almost exclusively in males. The disease may
be mild, moderate, or severe depending on the
Clinical Implications amount of clotting factor in the blood. The preva-
PCV patients may be on blood thinners (antico- lence of hemophilia A in the United States is approx-
agulants) and require special consideration if imately 1 in 5,000 males and accounts for 80% to 85%
dental procedures that induce bleeding are of hemophilia.
planned. In addition, episodes of thrombosis may
alternate with episodes of hemorrhage in this Hemophilia B
disorder. Gingival bleeding, sometimes sponta- Hemophilia B is an inherited bleeding disorder asso-
neous, can be an oral manifestation of PCV ciated with a lack of clotting factor IX. The disorder
treated with blood-thinning medications. Med- is also known as Christmas disease, after the first pa-
ical consultation to ensure that the red blood cell tient described with this disorder. It is the second
counts and clotting are normal is advisable be- most common type of hemophilia. Like hemophilia A,
fore starting dental treatment. hemophilia B involves a gene that occurs on the X
chromosome and is passed to a son as a sex-linked
recessive trait from a mother who carries the gene.
Hemophilia Hemophilia B almost exclusively occurs in males. De-
Hemophilia, perhaps the best known bleeding disorder, ficiency of factor IX significantly impairs clot forma-
is characterized by deficiency of clotting factors re- tion and control of bleeding. Hemophilia B is less
quired to stop hemorrhage. However, there are many common than hemophilia A, with an estimated
other types of bleeding disorders associated with spe- prevalence of 1 in 25,000 males. Historically, hemo-
cific missing or defective clotting factors. Three bleed- philia was called the royal disease due to increased
ing disorders will be discussed in this section: Von incidence resulting from intermarriage in European
Willebrand disease, hemophilia A, and hemophilia B. royal families. Recently, genetic testing indicates that
hemophilia in European royal family members was
Von Willebrand Disease hemophilia B.
Von Willebrand disease is the most common inherited Clinical features of both hemophilia A and B de-
bleeding disorder in the United States and can affect pend on severity of the condition and levels of clot-
both men and women. It may also be acquired as a result ting factors. Spontaneous bleeding may occur at any
of medical conditions. Von Willebrand disease is char- time, along with hematomas, ecchymoses in soft tis-
acterized by deficient or defective von Willebrand sues and muscles, and bleeding into weight-bearing
clotting factor. This is a protein that causes platelets to joints causing hemarthroses. Hemarthroses may be-
adhere to walls of damaged blood vessels. There are sev- come a debilitating condition, eventually leading to
eral types of this bleeding disorder with manifestations joint deformity requiring replacement. Excessive
ranging from mild to severe. Patients with milder forms bleeding may occur after routine dental and surgical
may have symptoms such as nosebleeds, easy bruising procedures.
tendencies, and prolonged bleeding after injury. In mild In the oral cavity, gingival bleeding may be sponta-
cases, the disease may go undetected until the patient neous or induced by dental procedures or minor
suffers serious injury or has surgery. Bleeding gums and trauma from tooth brushing or flossing. This may
prolonged bleeding after dental procedures such as cause patients to be afraid of practicing good oral
tooth extraction or deep scaling may occur. Aspirin and home care or undergoing any type of dental treatment.
other nonsteroidal anti-inflammatory drugs are con- Hemorrhage into the oral soft tissues and/or temporo-
traindicated in Von Willebrand disease because they mandibular joint (TMJ) may form tumor-like masses
make the condition worse. known as pseudotumor of hemophilia.
2577_Ch09_287-316 09/07/14 3:38 PM Page 306
Location in gastrointestinal tract Mainly in the colon and terminal Part of the small intestines and the colon
ileum, sometimes the rectum
Distribution of lesion Involves the entire portion of the Tends to have multiple localize areas
colon involved, forming skip lesions
Involvement of intestinal mucosa Usually conned to the supercial Tends to involve the entire mucosal layer
mucosa
Ulcerations Supercial and broad-based Deep and knife-like; linear pattern
Perforation of intestinal mucosa No Yes, as ulcers tend to be transmural
Constrictions of intestinal mucosa Rare Yes
Presence of intestinal pseudopolyps Numerous pseudopolyps Seldom
Risk of development of colon cancer Markedly increased Moderate
Clinical symptoms Bloody diarrhea with lower Mild diarrhea with some abdominal pain
abdominal pain
The precise cause of Crohns disease is unknown. It (see Chapter 4) and cobblestone buccal mucosa
has been associated with a combination of environmental (Fig. 9-21) are two of the most common oral mani-
factors, immune system problems, and genetic factors. festations. Their detection in the dental office may
Family members of affected individuals are slightly more be the first clue that a patient has Crohns disease.
at risk of developing Crohns disease. Smokers are more
likely to develop Crohns disease than nonsmokers. Pyostomatitis Vegetans
Changes in oral mucosa can be found in both Crohns Pyostomatitis vegetans is another inflammatory condition
disease and ulcerative colitis; however, they are more fre- seen in patients with Crohns disease and ulcerative co-
quently seen in Crohns disease. litis. In the mouth, this condition is characterized by
The oral manifestations of Crohns disease occur yellowish pustules on the oral mucosa and superficial
in up to 30 percent of patients and may lead to the ulcerations. These yellow pustules have a snail-track
initial diagnosis. Major aphthous ulcers (Fig. 9-20) appearance that may appear on almost any oral mucosal
surface (Fig. 9-22). Management of pyostomatitis
vegetans depends mainly on the treatment of the un-
derlying GI disorder. Rarely, it occurs as the sole man-
ifestation of the disease.
Clinical Implications
Identifying patients with jaundice is important
because it may indicate underlying liver disease.
Liver diseases often increase bleeding tendencies
due to decreased levels of clotting factors pro-
duced by the liver. Medical consultation may be
indicated when deep scaling or invasive dental
procedures are contemplated in patients with
known liver disease and/or jaundice.
AMYLOIDOSIS
Amyloid refers to proteins produced in the bone mar-
row that become folded into insoluble fibrous
forms when they interact with normal body proteins.
Abnormal accumulation of amyloid in organs is
Figure 926 Sclerodactyly. Contracture of hands in known as amyloidosis. Amyloidosis can be seen in at
patient with scleroderma. least 20 diseases affecting various organs including
the skin (Fig. 9-29), heart, kidneys, liver, spleen, cen-
tral nervous system, and gastrointestinal tract. There
are three types of amyloidosis: primary, secondary,
and hereditary.
Primary systemic amyloidosis is the most
common form. It tends to occur in adults older than
age 50 and has an unknown etiology. Primary amy-
loidosis occurs by itself and is not associated with
other diseases. Secondary amyloidosis has been as-
sociated with other disorders such as multiple
myeloma (see Chapter 7), tuberculosis (see Chapter 3),
rheumatoid arthritis, and osteomyelitis. Renal dial-
ysis increases the risk of amyloidosis because dialysis
cannot remove large proteins from the blood. Treat-
ment of underlying disease may stop or help control
Figure 927 Trismus from scleroderma. Patient with
limited ability to open her mouth due to contracture of this form of amyloidosis. The hereditary (familial)
facial skin. type of amyloidosis primarily affects the liver, nerves,
heart, and kidneys.
The most common oral manifestation of amyloi-
dosis is macroglossia. Enlargement of the tongue de-
Ehlers-Danlos Syndrome velops and scalloping occurs along the lateral borders
Ehlers-Danlos syndrome (EDS) is an inherited due to imprinting from adjacent teeth along the bite
connective tissue disorder involving production of de- line. Clinically, the tongue may show yellow nodules
fective collagen. Various forms of Ehlers-Danlos syn- on the lateral surfaces. Salivary hypofunction may
drome are based on specific gene mutations but most also occur as a result of amyloid deposition within the
patients present with a set of classic features including salivary glands.
increased elasticity of the skin, extremely flexible
joints, easy bruising due to increased fragility of blood
vessel walls, abnormal wound healing, and connective
tissue scarring (Fig. 9-28). Clinical Implications
Oral manifestations of EDS include high palatal
Suspected amyloid deposition in the tongue or
vault and a narrow maxillary arch. Dental crowding
other intraoral site requires medical evaluation
may develop, making the teeth difficult to clean. Indi-
for underlying systemic disease. Occasionally,
viduals are more susceptible to periodontal disease at
isolated lesions of amyloidosis are found in areas
an early age, due to defective collagen weakening pe-
exposed to chronic inammation. These are not
riodontal ligament fibers. Increased joint flexibility in-
associated with a serious underlying disease.
creases the tendency for dislocation of the jaw. This is
2577_Ch09_287-316 09/07/14 3:38 PM Page 311
Fat-Soluble Vitamins
Figure 929 Amyloidosis. Amyloid nodule on left Fat-soluble vitamins include vitamins A, D, E, and K.
eyelid. (From Barankin, B: Derm Notes. F.A. Davis, Animal sources include fish and fish oil, fortified milk
Philadelphia, 2006, p 191.) and juices, beef liver, fortified cereals, and eggs; other
sources include green leafy vegetables and vegetable
oils. Excess consumption of fat-soluble vitamins pro-
NUTRITIONAL DISORDERS AND VITAMIN motes their storage. This can result in a potentially
DEFICIENCIES dangerous situation called hypervitaminosis. Deficiency
Nutritional disorders most often involve inadequate of fat-soluble vitamins also may result from compro-
intake or absorption of one or more nutrients essential mised absorption. This may occur as a result of drugs
for health. The disorders may also result from intake that interfere with absorption from the intestine. Cys-
of food that has poor nutritional value. tic fibrosis is associated with a deficiency of enzymes
Thirteen fat- and water-soluble vitamins and organic from the pancreas that interferes with fat absorption
substances are essential in minute amounts for normal in the intestines. Oral manifestations of vitamins A, E,
body growth and activity. These are obtained naturally and K deficiency are uncommon.
2577_Ch09_287-316 09/07/14 3:38 PM Page 312
Vitamin D plays an important role in bone metab- number of osteoclasts and osteoblasts. Clinical signs
olism, maintaining calcium and phosphate levels in the of osteomalacia include skeletal deformities, bone
body. Vitamin D is found in fortified milk, cheese, whole pain, fractures, and decreased muscle tone with weak-
eggs, liver, and salmon. Skin synthesizes vitamin D ness of arms and legs. Alveolar ridge resorption and
when exposed to sunlight. Deficiency of vitamin D in periodontal bone loss may occur.
children produces the condition known as rickets.
Adult onset of deficiency of vitamin D is known as Water-Soluble Vitamins
osteomalacia. Water-soluble vitamins include vitamin C, all of the
Rickets is most common in the first year of life. It B vitamins, and folic acid. These vitamins dissolve in
presents with spine deformities, bowing of legs, promi- water when they are ingested and excess amounts are
nence of costochondral junctions (called rachitic excreted in the urine. They cannot be stored and con-
rosary), and protrusion of the sternum (called pigeon tinuous supply is needed daily to prevent deficiency
breast deformity) (Fig. 9-30). Teeth may show enamel states. The B-complex group is found in a variety of
pits and hypoplasia, large pulp chambers, and a thin foods: cereal grains, meat, poultry, eggs, fish, milk,
layer of dentin. legumes, and fresh vegetables. These vitamins are eas-
Osteomalacia literally means soft bones. It is a dis- ily destroyed during food storage and preparation,
ease of defective bone mineralization. Causes include promoting dietary deficiencies. Water-soluble vitamin
dietary deficiency of calcium and phosphate due to vi- deficiencies have both systemic and oral manifestations
tamin D deficiency, gastrointestinal absorption prob- of interest to dentistry.
lems, lack of sunlight, gastrectomy, celiac disease, Vitamin B1 is called thiamine. Thiamine deficiency
kidney or liver disorder, renal dialysis, and medications leads to beriberi, a condition that occurs in chronic al-
that block cellular calcium or phosphorous uptake. cohol abusers or elderly patients who have poor or
The result is accumulation of nonmineralized bone limited diets. Beriberi is characterized by poor ap-
matrix (osteoid) and a decrease in the activity and petite, fatigue, and nausea. It may also present with
cardiovascular problems such as heart failure and pe-
ripheral edema. Neurological problems including tin-
gling and numbing sensation of the extremities and tip
of tongue may occur. Severe cases may result in neu-
rological symptoms such as mental deterioration,
coma, and death.
Vitamin B2 is called riboflavin. Deficiency of ri-
boflavin may result in glossitis, angular cheilitis, and
generalized erythema of the oral mucosa. Other clin-
ical features include twitching of eyelids and photo-
phobia (sensitivity to light).
Vitamin B3 is called niacin. Niacin deficiency in the
diet leads to a condition known as pellagra, classically
associated with the four Ds: diarrhea, dementia, der-
matitis, and death. Oral manifestations include beefy
red glossitis and aphthous ulcers. Prolonged niacin de-
ficiency eventually leads to death.
Vitamin B6 is called pyroxidine. Deficiency is rarely
due to poor diet but most often related to poor absorp-
tion caused by medications such as corticosteroids and
certain antibiotics. Vitamin B6 deficiency can be seen
in alcoholics and those with poor absorption due to
aging. This vitamin is important for neurological
Figure 930 Rickets. Enamel hypoplasia, prominent rib health. Signs of deficiency include weakness, dizziness,
joints and sternum, protruding abdomen are features of or episodic seizures. Oral manifestations include glos-
rickets. sitis and angular cheilitis.
2577_Ch09_287-316 09/07/14 3:38 PM Page 313
Vitamin B12 is called cobalamin. Cobalamin has a scorbutic gingivitis. Aphthous ulcers, tooth mobility, and
key role in normal brain and nervous system function loss of periodontal support also occur. Wound healing
as well as blood formation. Cobalamin deficiency is is affected and is a treatment consideration, particu-
called pernicious anemia (previously discussed in this larly in surgical patients.
chapter). It is typically associated with autoimmune
gastritis (see Chapter 4 for discussion of autoimmu-
nity). Intrinsic factor involved in the absorption of vi-
tamin B12 is not produced because the gastric parietal Critical Thinking Questions
cells are destroyed by autoantibodies. Resultant vita- Case 9-1: Your 12-year-old male patient pres-
min B12 deficiency leads to defective red blood cell
ents with a long history of painful oral ulcers. His
production and anemia. Gastrointestinal bypass pa-
father had similar ulcers as a child. He now shows
tients and strict vegetarians are at risk for vitamin B12
deficiency. Clinical features of pernicious anemia in- diffuse small yellow papules along the lips and buc-
clude dyspnea, fatigue, dizziness, weakness, frequent cal mucosa that are not painful. He has missed a
headaches, and tingling or numbness of fingers and lot of school lately because his stomach has been
toes. Oral manifestations include tingling and burning bothering him.
tongue, patchy areas of erythema, and atrophy of the
Case 9-3: A 14-year-old male patient has been Case 9-2: Your 63-year-old female patient
coming to see you for about 3 years. Since his last complains that her tongue feels like it is on fire.
visit (1 year ago) you notice he has suddenly lost a It has felt this way for several months.
lot of weight. He looks very thin and his mother is
Review Questions
1. The anterior pituitary gland secretes two main 4. Diagnosis of hyperparathyroidism is based on
types of hormones. Which one of the following blood serum assay. Which of the following
is NOT secreted by the anterior pituitary gland? results of a blood serum assay is positive in
A. Antidiuretic hormone (ADH) hyperparathyroidism?
B. Adrenocorticotropic hormone (ACTH) A. Decreased PTH, elevated serum calcium
C. Growth hormone (GH) levels
D. Follicle stimulating hormone (FSH) B. Decreased PTH, decreased serum calcium
levels
2. Elevation of growth hormone after the closure C. Elevated PTH, elevated serum calcium
of bone growth plates results in which one of levels
the following diseases? D. Elevated PTH, decreased serum calcium
A. Gigantism levels
B. Acromegaly
C. Dwarfism 5. Signs and symptoms of hyperparathyroidism
D. Cushings disease include all of the following EXCEPT
A. loss of lamina dura.
3. Thyrotoxicosis is defined as elevated serum B. renal stones.
levels of free T3 and T4 and presents with C. supernumerary teeth.
many signs. Which of the following is NOT a D. gastric ulcers.
sign of thyrotoxicosis?
A. Tremor and emotional instability
B. Heat intolerance
C. Decreased appetite with weight gain
D. Elevated blood pressure with palpitations
2577_Ch09_287-316 09/07/14 3:38 PM Page 315
6. Which of the following statements about the 11. Which of the following statements about dia-
adrenal gland and its disorders is false? betes mellitus is incorrect?
A. The adrenal glands are located on the su- A. Type 2 diabetes is more common than type
perior poles of the kidneys. 1 diabetes.
B. In Cushings syndrome, the adrenals pro- B. Patients with diabetes mellitus have a higher
duce too much cortisol. incidence of gingivitis and periodontitis.
C. In Addisons disease, the adrenals produce C. If a hypoglycemic episode occurs, it is usu-
too little cortisol. ally not an emergency and discontinuation
D. The adrenal glands produce too much of dental treatment is not necessary.
ACTH in CREST syndrome. D. Type 1 diabetes is an idiopathic autoim-
mune disorder of the pancreas, whereas
7. Which of the following is NOT a clinical fea- type 2 diabetes is due to insulin resistance
ture of Addisons disease? and insufficient secretion of insulin by the
A. Hyperpigmentation of the skin pancreas.
B. Hypocortisolism
C. Adrenal insufficiency 12. Which test is used to confirm the diagnosis of
D. Bruises and purplish striae Cushings syndrome?
A. Fasting blood cortisol level
8. You notice your patient has been sweating ex- B. 24-hour free cortisol level
cessively while you are treating her. She also ap- C. Random cortisol level
pears to have a wide staring gaze, exophthalmos, D. Random ACTH levels
and sagging eyelids. She mentions that her re-
cent physical examination showed a decrease in 13. Which of the following are common clinical
TSH levels and increased free T3 and T4 levels. features seen in a patient with thalassemia?
What condition does she most likely have? A. Enlarged maxilla and mandible
A. Addisons disease B. Altered trabecular pattern
B. Cushings disease C. Hair-on-end appearance on skull radiograph
C. Graves disease D. All of the above
D. Ehlers-Danlos syndrome
14. Which of the following is NOT a common
9. Uncontrolled diabetes mellitus may cause all etiologic factor of iron deficiency anemia?
of the following EXCEPT A. Lack of dietary iron intake
A. immune suppression. B. Excessive acute or chronic blood loss
B. ketoacidosis. C. Frequent blood transfusion
C. elevated insulin levels. D. Impaired absorption of iron by the body
D. elevated blood glucose levels.
15. Which of the following is NOT a common
10. Common oral manifestations of diabetes mel- oral manifestation of iron deficiency anemia?
litus include all of the following EXCEPT A. Bleeding gums
A. oral candidiasis. B. Atrophic glossitis
B. xerostomia. C. Angular cheilitis
C. erythema migrans. D. Burning sensation of the tongue
D. burning tongue.
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16. Pyostomatitis vegetans is an inflammatory 20. A 48-year-old female with anemia complains
condition characterized by yellow pustules in of a burning tongue and tingling and numb-
the oral mucosal surfaces. It is associated with ness of the fingers and toes. Laboratory stud-
which one of the following conditions? ies reveal decreased levels of intrinsic factor
A. Vitamin A deficiency and vitamin B12. Based on this information,
B. Crohns disease what specific type of anemia does this patient
C. Hepatitis A infection most likely have?
D. Addisons disease A. Iron deficiency anemia
B. Pernicious anemia
17. Jaundice is a clinical presentation that indi- C. Plummer-Vinson syndrome
cates an underlying liver disease. Which of D. Folic acid deficiency
the following is NOT a cause of jaundice?
A. Increased production of bilirubin REFERENCES
B. Decreased uptake of bilirubin into the liver Books
Tamparo, L: Diseases of the Human Body, ed. 5. F.A. Davis,
C. Increased excretion of bilirubin Philadelphia, 2011.
D. Decreased excretion of bilirubin Underwood, J, Simon, S: General and Systematic Pathology, ed. 5.
Churchill-Livingston, Elsevier, London, 2009.
18. Radiographic examination of a 42-year-old Wardlaw GM, Smith AM: Contemporary Nutrition, A Functional
woman reveals bilateral, generalized, diffuse Approach, ed. 3. McGraw-Hill Higher Education, New York, 2013.
Zelman M, Tompary, R, Holdaway, M: Human Diseases: A Systemic
decrease in bone density and apparent loss of
Approach, ed. 7. Prentiss Hall, Upper Saddle River, NJ, 2010.
lamina dura and cortical plates. Serum cal-
cium levels are elevated. Based on this infor- Journals
mation, which of the following is the most Chrysomali, E, Piperi, E, Sklavounou-Andrikopoulou, A: Oral acan-
likely diagnosis? thosis nigricans in chronic hepatitis B with a 21 year follow up.
A. Hyperparathyroidism Journal of Dermatology 38(12):11721176, December 2011.
Clayton, B: Stroke, dysphagia and oral care: What is best practice?
B. Cushings syndrome
Alta RN 68(1):2627, Spring 2012.
C. Graves disease Genco, RJ: Periodontal disease and association with diabetes
D. Addisons disease mellitus and diabetes: Clinical implications. Journal of Dental
Hygiene 83(4):186187, Fall 2009.
19. A 19-year-old male has multiple red papules Jen, M, Yan, AC: Syndromes associated with nutritional deficiency
and excess. Clinical Dermatology 28(6):669685, November-
on the tongue and vermilion zone of the lips. December 2010.
The papules are 1 to 2 mm in size and blanch Khocht, A, Schleifer, SJ, Janal, MN, Keller, S: Dental care and oral
upon pressure. The patient has a history of disease in alcohol-dependent persons. Journal of Substance Abuse
multiple episodes of epistaxis. Based on this Treatment 37(2):214218, September 2009.
information, which of the following is the Wilkins, T, Jarvis, K, Patel J: Diagnosis and management of
Crohns disease. American Family Physician 84(12):13651375,
most likely diagnosis? December 15, 2011.
A. Hereditary hemorrhagic telangiectasia
B. Pyostomatitis vegetans
C. Peutz-Jeghers syndrome
D. Vitamin D deficiency
2577_App_317-318 09/07/14 3:09 PM Page 317
Appendix
317
2577_App_317-318 09/07/14 3:09 PM Page 318
Glossary
319
2577_Glossary_319-328 09/07/14 3:40 PM Page 320
320 Glossary
from the body; molecules that inhibit or Bone marrow transplant: Replacement of diseased
counteract oxidation or damaged bone marrow with healthy bone
Apoptosis: The process of programmed or planned marrow; the replacement bone marrow may be
cell death; may be physiologic in embryonic the patients own (autologous) or it may come
development or pathologic in disease states from a donor (allogeneic)
Atopy: An allergic reaction that occurs within a few Bradycardia: A heart rate slower than normal heart
minutes when a sensitized individual comes into rate (usually less than 60 bpm)
contact with a particular antigen Bruxism: Wear of teeth due to clenching or grind-
Atrophy: Decrease in the size of an organ or tissue ing; a form of attrition
due to disease, injury, or lack of use; typically due
to degeneration of cells; example: atrophy of C
muscles occurs over time when an arm or leg is Capsule: A membranous structure made of brous
immobilized in a cast connective tissue that envelops an organ or tumor
Attrition: Loss of tooth tissue due to tooth-to-tooth Carcinoma: A malignancy arising from structures of
contact; example: chronic tooth grinding, bruxism epithelial origin; example: squamous cell carcinoma
Auscultation: Listening to body sounds to evaluate Carrier: A person who harbors an infectious microor-
if a structure is normal or abnormal. Example: ganism without clinical evidence of the disease;
using a stethoscope to hear sounds made by may transmit the infection to others. In genetics,
internal organs such as the heart and lungs an individual who inherits a trait or mutation, but
Autoantibodies: Antibodies inappropriately does not show signs of the disorder
produced by an individual in response to a Cell aging: Over time, the decrease in ability of the
component of ones own tissue cell to divide or proliferate
Autoimmune disease: A disease in which the Cellular response: Immune process characterized
immune system mistakenly attacks ones own body by direct attack on foreign antigens by sensitized
cells and tissues; example: rheumatoid arthritis T cells, and secretion of lymphokines that activate
Autoimmunity: The inappropriate production of the humoral response
antibodies against ones own tissues Central: Developing within bone
Autoinoculation: Spreading an infection from a Chancre: A painless ulcer, the primary lesion of
part of ones body to another by transferring the syphilis; occurs at a microorganisms site of entry
microorganism, usually with contaminated hands Chemotherapy: Treatment of disease by use of
Autosomal recessive disorder: A genetic disease chemical substances; typically using drugs to
that appears only in individuals who receive two destroy cancer cells, but can refer to other drug
copies of a particular disease allele, one from each therapies
parent Chromosome: A structure composed of nucleic
Autosome: A chromosome that is not a sex acids and proteins located within the nucleus of
chromosome living cells that carries genetic information in the
form of DNA; humans have 23 pairs of chromo-
B somes (22 pairs of autosomes and 1 pair of sex
Bacterium: (Pl. Bacteria) A unicellular microorganism, chromosomes)
some are capable of causing disease Chronic: Persisting for a long period of time; grad-
Benign: A tumor that does not threaten life by ual onset over time and of longer duration
invading and destroying tissue, or metastasizing; Cicatricial: Pertaining to a scar; cicatrix is synony-
example: lipoma mous with scar
Betel quid: A concoction composed of areca nut and Cleft: An opening or cleavage resulting from failure
other herbal drugs wrapped in the leaf of a betel of parts to fuse during embryonic development;
palm and chewed for its intoxicant properties; a example: cleft lip
habit seen in Asian countries Clinical trial: Research based upon the scientic
Bifid: Divided into two parts; example: bid tongue method in which a control group and a test group
Biopsy: The surgical removal of tissue or cells for are compared over a period of time in order to
microscopic examination; may be incisional or study a single, differing factor; a highly reliable
excisional form of research
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Glossary 321
322 Glossary
Encephalitis: Inammation of the brain, most often Exophytic: Protrudes from the surface
due to infection Exostosis: Exophytic growth of normal bone; tori
Endemic: A disease or condition regularly present in a are an example
specic region or within a specic group of people Expression: Process by which information from a
Endocrine: Related to secretion of hormones di- gene is used in the synthesis of a functioning gene
rectly into the bloodstream rather than through product
a duct system Extraglandular: Associated with a gland but occur-
Endogenous: Caused by factors within the body or ring outside the glandular tissues
arising from internal causes; example: endogenous Extraosseous: Occurring within soft tissues that
bilirubin stain of teeth in a child with liver disease surround bone, but not within the bone itself
Endophytic: Extends down and below the level of Extrapulmonary: Outside of or unrelated to the
the surrounding normal tissue lungs
Enucleation: Complete surgical removal of a cyst or Extrinsic stain: A substance that imparts color to a
tumor without cutting it into fragments tissue or cells or a discoloration caused by external
Epidemiology: Branch of medicine that studies dis- agents (exogenous); example: tobacco stain
ease within a population rather than in an individual
Eponymous: Named after a person; example is Gorlin F
syndrome, named after Dr. Robert Gorlin Factitial: Self-induced injury either intentionally or
Epistaxis: Nosebleed unintentionally; example: a pizza burn is a factitial
Epulis: Any raised mass of the gingiva injury
Erosion: Loss of tooth hard tissue due to exposure Fibromatosis: Formation of multiple brous,
to chemicals with a low pH or stomach acid; tumor-like nodules
example vomiting in bulimia Field cancerization: Generalized carcinogen-
Erythroleukoplakia: A clinical descriptive term induced changes within a tissue from which multi-
meaning red and white epithelial or mucosal color ple independent lesions may occur; example: skin
change to the mucosa or skin cancers arising in sun-damaged skin
Erythroplakia: A clinical descriptive term meaning Five-year survival rate: The percentage of patients
red epithelial or mucosal patch who are living 5 years after being diagnosed with a
Etiologic agent: A microorganism or substance, life-threatening condition
such as a toxin, that causes a disease; example: Foramen caecum: A shallow depression in the
mycobacterium tuberculosis is the etiologic agent posterior dorsal tongue that is a remnant of the
of tuberculosis embryonic thyroglossal duct
Etiologic factor: An agent or condition that con- Foreign body reaction: An immunologic reaction
tributes to the production of a disease; example: to substances not recognized by the immune
smoking as an etiologic factor for lung disease system as self; capable of inducing an inamma-
Etiology: The cause of a disease tory response
Exacerbation: Worsening; increase in severity of the Forensic medicine: The branch of medicine that
disease uses anatomic structures and physical characteris-
Excisional biopsy: The removal of an entire lesion tics for legal purposes
including a signicant margin of normal tissue for Forensic odontology: Branch of forensic medicine
microscopic examination and diagnosis that uses dental characteristics for legal purposes
Exfoliative cytology: Removal of surface epithelial Formalin: A colorless solution of formaldehyde in
or mucosal cells for microscopic examination of water; a 10 percent solution is used to x and pre-
individual cells to determine a diagnosis; example: serve tissues for study under the microscope
cytologic smear to diagnose candidiasis Free radicals: A highly unstable and reactive group
Exogenous stain: An external agent/substance that of atoms that have at least one unpaired electron
imparts color or discoloration to a tissue or cells in their outer shell, responsible for aging, tissue
(extrinsic); example: tobacco stain damage, and some diseases
Exophthalmos: Abnormal protrusion of the Friable: Easily crumbled
eyes from the orbit caused by disease; seen in Frictional keratosis: Benign reactive phenomenon
hyperthyroidism in which excess keratin builds up to protect the
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Glossary 323
underlying tissues from persistent irritation; a tissues, most often due to local trauma and rup-
form of hyperkeratosis ture of blood vessels
Fulminant: Occurring suddenly and with great Hematuria: Blood in the urine
severity Hemoglobin: Oxygen-carrying pigment in red
Fungi: A group of single-celled or complex multicel- blood cells
lular organisms, includes yeasts and molds Hemolysis: Breakdown or destruction of red blood
Fusion: The process of merging and making one cells with the resulting release of hemoglobin
unit; example: fusion of two tooth germs to form Hemoptysis: Coughing up blood due to bleeding in
one large tooth any portion of the respiratory tract
Hemostasis: The stoppage of blood loss from a
G damaged blood vessel
Gastritis: Inammation of the mucosal lining of the Histamine: A neurotransmitter released by cells (often
stomach mast cells) in response to injury and during an aller-
General pathology: The branch of pathology that gic reaction, triggers the inammatory response
studies disease processes occurring in any organ Hormone: A chemical released by a cell with the
system such as inammation, infection, genetic ability to affect or regulate cells in other parts of
disorders, and neoplasia the body
Genetics: The study of inheritance, a branch of Humoral response: The portion of the immune sys-
biology that deals with heritable factors that tem that is mediated by transformation of B cells
inuence development and maintenance of an into plasma cells producing antibodies; humoral
organism refers to the non-cellular components of blood
Genotype: The genetic makeup of an individual; Hutchinsons Triad: Three common clinical fea-
type of genes expressed; genotype predicts pheno- tures of congenital syphilis: 1) Hutchinsons teeth
type or appearance, the way a person looks with notched incisors and mulberry molars, 2) oc-
Globulomaxillary: An anatomic location that ular keratitis, and 3) eighth cranial nerve deafness
describes any lesion located between the maxillary Hypercalcemia: Abnormally high levels of calcium
canine and lateral incisor in the blood
Glossitis: Inammation of the tongue, often but not Hyperchromatism: Intense deep blue coloration to
always producing pain cell nucleus
Glucometer: A medical device for determining the Hyperglycemia: Abnormally high levels of glucose
approximate concentration of glucose in the in the blood
blood; used as a monitor in diabetes Hyperkeratosis: Thickening of the stratum
Granulation tissue: Nonspecic vascular brous corneum of the skin or supercial keratin layer of
connective tissue that replaces a brin clot in the mucous membranes, most commonly caused
wound healing, as the body repairs injury by chronic irritation
Granuloma: A specic form of chronic inamma- Hypermelanosis: Abnormal darkening of the skin
tion that attempts to wall off substances perceived or mucous membrane resulting from increased
as foreign but impossible or difcult to eliminate melanin production by normal melanocytes with-
Gummas: Soft, rubbery tissue masses associated out an increase in the number of melanocytes
with tertiary syphilis Hyperplasia: Increase in the size of an organ or
tissue due to an increase in the number of cells
H Hypersensitivity reaction: Excessive or inappropri-
Hamartoma: A benign tumor-like malformation ate reaction to an allergen; an allergic reaction
composed of an overgrowth of tissue elements nor- Hypertelorism: Abnormal increase in distance
mally found at the anatomic site where it develops between two organs or parts; example: ocular
Hemarthosis: Bleeding into a joint space hypertelorism indicates wide spacing of the eyes
Hematocrit: A blood test that measures the percent- Hypertrophy: Increase in the size of an organ or
age of whole blood made up of red blood cells tissue due to increase in the size of individual
Hematoma: A localized collection of blood that cells
accumulates outside blood vessels causing a bluish Hypodontia: Partial congenital absence of teeth
discoloration and enlargement of the surrounding Hypohidrotic: Decreased ability to sweat
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324 Glossary
Hypoxia: Decreased concentration or lack of oxygen Infarction: Obstruction of the blood supply to
within body tissues an organ or tissue typically by a thrombus or
embolus resulting in local death of the tissue;
I example: myocardial infarction or heart attack
Iatrogenic: Clinician-induced injury, occurs as a Infectious agent: An agent (microorganism) capable
result of treatment; example: accidental trauma of producing infection
or laceration from a dental instrument Infectious disease: A disease caused by microorgan-
Icteric/Icterus: Jaundiced, yellowing of the skin and isms; may or may not be contagious
sclera of the eyes, etc. Infiltration: Permeation into adjacent normal
Idiopathic: Term used when the cause of a disease is structures or tissues
unknown Injury: Damage, harm, or wounding
Immune system: A complex of organs involving the Inspection: The process of critically appraising
thymus, bone marrow, and lymphoid tissues that a lesion or condition: involves examination,
protects the body from foreign substances and measurement, and comparing the findings with
pathogenic microorganisms normal.
Immunodeficiency: Inability to produce a normal Insulin: A hormone produced in the pancreas by the
immune or inammatory response islets of Langerhans that regulates glucose levels
Immunoglobulins: Large glycoproteins secreted by in the blood
plasma cells; function as antibodies Intermaxillary segment: Wedge of tissue formed by
Immunomodulary: Refers to a class of drugs capa- growth of the medial nasal processes, participates
ble of modifying or regulating the immune in formation of the primary palate
response Intraosseous: Located within bone
Immunosuppressive: A substance that has the abil- Intrinsic: Caused by factors within the body or
ity to lower the bodys normal immune response; arising from internal causes; example: intrinsic
example: corticosteroid medications that suppress (endogenous) bilirubin stain of teeth in a child
the inammatory response with liver disease
In situ: In its original place or position; example: Inversion: Occurs when a chromosome breaks and
squamous cell carcinoma-in-situ that remains the part is reinserted in the wrong location
within the surface epithelium and has not inl- Ischemia: Temporary deciency of blood in a body
trated or metastasized part due to obstruction or constriction of local
Incidence: In epidemiology, the number of blood vessels; example: angina pectoris is caused
new cases of the disease during a designated by ischemia of the heart muscle
period of time; example: the incidence of
hepatitis B in the United States was 8,064 cases K
in 2002 Keloid: Exuberant proliferation of scar tissue
Incisional biopsy: Surgical removal of part of a resulting from excess collagen production
lesion to be used in establishing the diagnosis, during healing
most often used when the lesion is large and Keratinaceous: Composed of, containing, or
will require a more extensive procedure to totally characterized by keratin
remove it Keratin: An insoluble protein that makes up the
Indigenous microflora: Microorganisms regularly uppermost layer of surface skin and some mucous
found at any anatomical site; also known as the membranes
normal ora Keratosis: Refers to rough raised areas of epithelium
Indolent: Slow-growing; causing few symptoms; with extra keratin build-up
example: prostate cancer tends to be an indolent Ketoacidosis: A change in the pH of blood due
disease in many cases and not discovered until to increased accumulation of ketone bodies in
autopsy the blood from excess fat breakdown; seen in
Indurated: Soft tissue that is abnormally firm due diabetes
to influx of fibrous or cellular tissue elements Kopliks spots: Highly characteristic sign of early
or exudate; example: oral cancer tends to be measles, small red spots with white centers on
indurated mucous membranes of cheeks and lips
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Glossary 325
326 Glossary
Nodule: A solid mass that is greater than 5 mm in Pathogenic: Capable of causing or producing
diameter disease
Nonodontogenic: Arising from cells or tissue unre- Pathogenicity: The ability of microorganisms to
lated to tooth development produce pathological changes or disease
Nosocomial infection: A disease originating in a Pathognomonic: Relating to a sign or symptom
health-care setting; an infection acquired in a hos- unique to a particular disease, which distinguishes
pital, nursing home, or other health-care facility it from other diseases; example: blistering skin le-
sions following a dermatome are pathognomonic
O of herpes zoster (shingles)
Obturator: A prosthetic device used to close an Pathologic atrophy: Atrophy due to a physical
opening, often due to a developmental defect or process, such as disuse of muscles, reversible
disease process Pathologic calcification: Abnormal deposition of
Odontogenic: Arising from cells or tissues related to calcium in tissues other than bones and teeth
tooth development Pathologic hyperplasia: Increase in the size of an
Odontogenic cysts: Cysts arising from remnants of organ or tissue due to an increase in the number
epithelium associated with tooth development of cells due to underlying disease
Oncogene: Altered gene capable of causing a neoplasm Pathologic hypertrophy: Increase in the size of
Oncogenic: Agent with potential to cause develop- an organ or tissue due to an increase in the size
ment of a cancer or neoplasm of individual cells, due to underlying disease
Operculum: Flap or hood of brotic tissue around Pathophysiology: The study of physiologic processes
or over an erupting tooth, most often third molars that are altered in the presence of disease or injury
Opportunistic infection: An infection by a mi- Pedunculated: A raised mass with a stalk
croorganism that normally does not cause disease; Penetrance: The proportion of individuals who
however, due to an impaired immune system carry a particular variant of a gene for a trait; com-
infection occurs plete penetrance refers to the trait being present in
Oral pathologist: One who studies the characteris- all individuals who carry the allele; incomplete
tics, causes, and effects of diseases of the mouth, penetrance refers to the trait being expressed in
oral cavity, and associated structures only a portion of the individuals carrying the allele
Oropharyngeal: Relating to the mouth and pharynx Percussion: The act of tapping on a surface to de-
Osteoradionecrosis: Death of bone following irra- termine the condition of the underlying structure;
diation; radiation exposure may be therapeutic or example: percussion is used on teeth to help iden-
accidental; bone death results from destruction of tify which tooth is most sensitive and has associ-
the blood supply within bone ated inammation of the periodontium
Peripheral: Developing outside the bone within the
P overlying soft tissues
Palliative: Treatment provided to relieve the symp- Petechiae: Small red or purple pinpoint spots, due
toms of the disease rather than cure it to hemorrhage of capillaries
Palpation: A procedure in which the sense of touch Pharyngotonsillitis: Concurrent inammation of
is used to gather data for diagnosis the pharynx and tonsils
Palsy: An involuntary uncontrollable tremor or Phenotype: Observable physical characteristics of an
paralysis individual that are determined by the individuals
Papillary: Having a nger-like protuberances or genetic makeup (genotype) and effects of environ-
projections mental factors
Papule: A circumscribed, solid, elevated lesion; less Physiologic atrophy: Irreversible atrophy, often due
than 5 mm to aging of an organ or body part
Parulis: Mass of granulation tissue on the alveolar Physiologic hyperplasia: An increase in the size of an
ridge or gingiva, contains the opening through organ or tissue due to an increase in the number of
which an abscess drains cells in response to normal metabolic function
Passive immunity: Short-term immunity resulting Physiologic hypertrophy: An increase in the size of an
from administration of antibodies produced by organ or tissue due to an increase in the size of indi-
another person or animal vidual cells in response to normal metabolic function
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Glossary 327
Plasma cells: Antibody-producing cells found in Rests of Serres: Epithelial remnants of the dental
lymphoid tissue derived from B lymphocytes lamina
Pleomorphism: Wide range in cell size and shape Reticular: Resembling a network; a net-like form;
(cells are dissimilar to the size and shape of nor- intricate
mal cells; pleo = many; morphism = shape) Risk factor: A characteristic statistically associated
Polydipsia: Excessive thirst with, but not causally related to, increase of disease;
Polyphagia: Excessive hunger example: smoking is a risk factor for heart disease
Polyuria: Excessive urination
Prevalence: The total number of cases of a disease S
within a population; example: the prevalence of Saliva substitutes: Sprays and rinses that temporar-
hepatitis B in the United States is approximately ily replace lost saliva and provide relief from the
730,000 people physical discomfort of dry mouth
Primary tumor: The original neoplasm from which Sarcoma: A cancer arising from nonepithelial or
metastatic disease originated connective tissues, such as muscle or bone
Protozoa: Single-celled microscopic microorgan- Sclera: The white external covering of the eyeball;
isms, many of which are motile; example: amoebas known as the white of the eye
Pruritic: Severe itching Sclerotic: Becoming rigid; losing the ability to
Pseudocyst: A cavity that is not lined by epithelium; adapt; hardening; example: atherosclerosis
example: traumatic bone cavity, formerly called Self-limiting: A disease that runs a limited or de-
traumatic bone cyst ned course; self-limiting diseases tend to end
Purpura: Discoloration of the skin comprised of without treatment; example: the common cold
purple spots caused by hemorrhage into surface Septicemia: Presence of microorganisms or toxins
skin or mucosa; generally greater than 3 mm in within the blood; blood poisoning
diameter Serologic: Pertaining to the study of blood serum
Pus: A thick creamy yellow-or green-colored uid Sessile: Exophytic lesion in which the base is broader
produced by infected tissue, composed of dead than the mass; example: the common wart
white blood cells (primarily neutrophils), bacteria, Sex chromosomes: Chromosomes that carry the
and tissue debris genes that transmit sex-linked traits and condi-
tions; can be X or Y
R Sialadenosis: Enlargement of the salivary glands
Radiation therapy: Treatment of disease using ion- often seen in alcoholism and malnutrition
izing x-rays or other forms of radiation to destroy Sialogogue: A drug that increases the ow of saliva
or shrink a lesion Sign: Any objective evidence of a disease; example:
Radiosensitive: Vulnerable to the effects of ionizing ra- radiolucency is a sign of apical tooth pathology in
diation; example: white blood cells are radiosensitive the jaws
RBCs: Abbreviation for red blood cells, also known Sinus tract: Communication between a pathologic
as erythrocytes space and an anatomic body cavity or the skin
Recessive: A gene that produces its characteristics Sinusitis: Inammation of the nasal and/or paranasal
when the two inherited alleles are identical, in the sinuses
homozygous state Sporadic: A disease that occurs as single and scattered
Remission: A period of time during which symp- cases, which are usually self-limiting and acute
toms of disease disappear or are greatly reduced Sputum: A mixture of mucous and saliva expelled by
Resectable: Capable of being excised coughing or clearing the throat
Resection: Surgical excision Staging: Describes the extent to which cancer has
Resorption: Loss or reduction of volume of a tissue spread in a patients body
by physiologic or pathologic means; example: Stasis: Stoppage of the ow of a body uid
root resorption occurs prior to exfoliation of the Stem cell transplant: A cancer treatment that
primary teeth involves removal of stem cells from a donor for
Rests of Malassez: Epithelial remnants of Hertwigs placement into a new patient; the stem cells mi-
root sheath that remain within the periodontal grate to the bone marrow of the recipient where
ligament space they replace the diseased marrow; stem cells are
2577_Glossary_319-328 09/07/14 3:40 PM Page 328
328 Glossary
Index
Note: Page number followed by f indicate figures; page numbers followed by t indicated tables
329
2577_Index_329-342 10/07/14 1:20 PM Page 330
330 Index
Index 331
332 Index
of the dentition, 172179 Dysplasia, 2930, 234235, 235f, 236f thymus gland, 289
concrescence, 174 cleidocranial, 205206, 205f, 206f thyroid gland, 289, 291293
congenitally missing teeth, 173 dentin, 218t, 219, 220f Endogenous intracellular pigments, 30
dens evaginatus, 176, 176f ectodermal, 202203, 203b, 203f Endophytic lesions, 12f
dens invaginatus, 176f fibrous, 19, 212213, 212f, 213f Endophytic mass, 237, 237f
dilaceration, 177f florid cemento-osseous, 6566, 65t, 66f Endothelial cells, 304
enamel hypoplasia, 178179 focal cemento-osseous, 62, 65, 65f, 65t End-stage COD, 6465
enamel pearl, 176, 176f hypohidrotic ectodermal, 202203, 202f Enteroviruses, 106
fusion, 174, 174f osseous, 18f Enucleation, 182
gemination, 174, 174f periapical cemental, 65, 65t Eosinophilic granuloma, 265
hyperdontia, 173, 174f Dystrophic calcification, 32, 59t, 281282 Eosinophils, 36f, 37, 124, 261
macrodontia, 175 Ephelis, 43
microdontia, 175 Epidemic parotitis, 107108
peg lateral, 175, 175f
E Epidemiology, 232
supernumerary roots, 177f EBV. See Epstein-Barr virus Epidermal inclusion cyst, 180t, 185
talon cusp, 175, 175f Ecchymosis, 304 Epidermoid cyst, 180t, 185
taurodontism, 177, 177f Ectodermal dysplasia, 202203, 202f, 203b Epinephrine, 45, 294
tooth color abnormalities, 177178 Ectomesenchyme, 164 Epistaxis, 306
tooth shape abnormalities, 174175 Ectopic thyroid, 170, 170f Epithelial neoplasms, 247252
facial and oral cavity abnormalities, Edema, 35, 35t Eponymous syndromes, 199
167171 Edwards syndrome, 204t Epstein-Barr virus, 79, 100t, 105, 106, 232
aglossia, 168169 Ehlers-Danlos syndrome (EDS), 310, 311f Epulis, 51
ankyloglossia, 168169, 169 Electrical burns, 45, 46t Epulis fissuratum, 57, 57f
bifid tongue, 168 Electromyography, 20 Epulis granulomatosum, 52, 52f, 262
bifid uvula, 168, 168f ELISA (enzyme-linked immunosorbent Erosion, 12f, 16, 70, 71f, 71t, 72, 72f
cleft lip/palate, 167168, 167f, 169 assay), 20 Erosive lesion, 15f
double lip, 168, 169 EM. See Erythema multiforme (EM) Erosive lichen planus, 142, 142f, 151t
fissured tongue, 169170, 170f Emboli, 306 Eruption cyst, 180t, 181182, 182f
hemifacial microsomia, 171, 171f Embryological development, 164167, Erysipelas, 8485, 85f
lingual thyroid, 170, 170f 164f, 165f Erythema migrans, 4748, 48f
lingual varicosities, 170171, 171f Embryology, 164 Erythema multiforme (EM), 138139,
lip pits, 168 Embryonic germ layers, 165f 139, 140f
macroglossia, 169, 170f Enamel epithelium, 171172 Erythema multiforme major, 139
microglossia, 169 Enamel hypoplasia, 178179 Erythematous candidiasis, 9293, 109
DI. See Dentinogenesis imperfecta (DI) Enamel organ, 171 Erythroblastosis fetalis, 178
Diabetes mellitus (DM), 296299, 297f, Enamel pearl, 176, 176f Erythrocytes, 36
298t Encapsulation, 231232, 232f Erythroleukoplakia, 235
Diabetic ketoacidosis, 296, 297t298t Encephalitis, 103 Erythroplakia, 235
Diagnosis Encephalitis, herpetic Ethnic tattoo, 41, 44f
definition of, 3 Endemic Burkitts lymphoma, 264 Etiologic agents, 78, 164
definitive, 20 Endocrine system, 288299 Etiologic factors/agents, 3
differential, 2021, 21t adrenal glands, 289, 294296 Etiology, 3
of infection, 8182 disorders Euploid, 195
Diagnostic aides, 16, 1920 acanthosis nigricans, 299, 299f Exacerbations, 129
Differential diagnosis, 2021, 21t acromegaly, 290 Excisional biopsy, 19
Differentiation, 231 Addisons disease, 295296, 295f, Exfoliative cytology, 20
Diffuse systemic sclerosis/scleroderma, 296f Exogenous stain, 177
154, 154f causes of, 288 Exophthalmos, 207, 291, 292f
DiGeorge syndrome, 129 Cushings syndrome, 294295, 295f Exophytic lesions, 11f
Dilaceration, 176177, 177f diabetes mellitus, 296299, 297f, Exophytic mass, 236237, 237f
Diphtheria, 86 298t , 298f Exostoses, 22
Diploid, 195 gigantism, 290 Exotoxins, 81
Discoid lupus erythematosus, 150, 150f, hyperparathyroidism, 293294, 293f Expression, of genes, 196
151f, 151t hyperpituitarism, 290 Expressivity, 198
Disease, 1 hyperthyroidism (Graves disease), External resorption, 72, 72f
Disseminated histoplasmosis, 97, 97f 128t, 129, 291292, 292f Extraglandular thyroid tissue, 291
DM. See Diabetes mellitus (DM) hypoparathyroidism, 294 Extraoral head and neck examination, 3, 4t6t
DNA. See Deoxyribonucleic acid (DNA) hypopituitarism, 291 Extraosseous cysts, 179, 180t
DNA-PCR (polymerase chain reaction), hypothyroidism, 292293 Extrapulmonary lesions of TB, 88
20 hormones of, 288, 289, 289t290t Extrinsic risk factors, 232
Dominant genes, 196197 hypothalamus, 289t Extrinsic stain, 177
Double lip, 168, 169 ovaries, 289t
Down syndrome, 199 overview of, 288
Drug-associated osteonecrosis of the jaw, pancreas, 289t, 296299
F
6667, 66t parathyroid glands, 289, 293294 Facial and oral cavity abnormalities, 167171
Drug-induced gingival overgrowth pineal gland, 289t aglossia, 168169
(hyperplasia), 5253 pituitary gland, 288, 289, 290, 290291 ankyloglossia, 168169, 169f
DSPP gene, 219 testes, 289 bifid tongue, 168
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Index 333
bifid uvula, 168, 168f Free radicals, 32, 33f necrotizing ulcerative, 8384, 83f
cleft lip/palate, 167168, 167f, 169 Friable masses, 240 plasma cell, 132
double lip, 168, 169f Frictional keratosis, 40, 41f, 42t, 234 scorbutic, 313
fissured tongue, 169170, 170f Fungal infections, 9199 Globulomaxillary cyst, 180t, 186187,
genetic and inherited disorders, 202214 angular cheilitis, 9394 186f
hemifacial microsomia, 171, 171f aspergillosis, 98 Glomerulonephritis, 128t
lingual thyroid, 170, 170f blastomycosis, 99 Glucocorticoid, 38, 294
lingual varicosities, 170171, 171f candidiasis, 9194, 92f, 93f, 9497, 96f, Goiter, 291, 291f
lip pits, 168 97f Goldenhar syndrome, 171
macroglossia, 169, 170f coccidioidomycosis, 99 Goltz-Gorlin syndrome, 201t
microglossia, 169 cryptococcosis, 99 Gorlin cysts, 183
Facial development, 164, 165166, 166f histoplasmosis, 9697 Gorlin syndrome, 199, 208209, 210f
Facial skin, epithelial neoplasms of the, mucormycosis, 9798, 97f, 98f Graft-versus-host disease (GVHD), 148,
247252 Fungi, 79, 80f, 82f 149, 150
Factitial lesion, 3 Fusion, 174, 174f Gram-negative bacteria, 79
Familial polyposis syndrome, 213214, 213f Gram-positive bacteria, 79
Fat-soluble vitamins, 311312 Granular cell tumor, 256, 256f
FCOD. See Focal cementoosseous dysplasia
G Granular leukocytes, 36
Fever, 38 Gamma globulins, 126 Granulation tissue, 39, 39f
Fibrin, 36 Gardners syndrome, 213214, 213f Granulocytes, 123124
Fibrinogen, 36 Gastritis, 313 Granulocytic cells, 261
Fibrinolytic system, 36 Gastro-esophageal reflux disease (GERD), Granuloma gravidarum, 49
Fibroblast growth factor receptor (FGFR) 72f Granulomas, 34
gene, 206 Gastrointestinal disorders, 306308 Granulomatous diseases, 151
Fibroblasts, 39, 39f Crohns disease, 306307, 307f, 307t Granulomatous inflammation, 34, 35t
Fibroepithelial polyp, 57, 58f hyperbilirubinemia, 308, 308f Graphite tattoo, 41
Fibroids, 259 inflammatory bowel disease, 306308 Graves disease, 128t, 129, 291292, 292f
Fibromas jaundice, 308 Ground glass (frosted glass), 19t
ameloblastic, 271t, 276, 276f pyostomatitis vegetans, 307, 308f Growth hormone (GH), 288, 289t, 290,
cemento-ossifying, 279 ulcerative colitis, 306307, 307t 291
ossifying, 279, 280f Gemination, 174, 174f Gummas, 90, 90f
peripheral ossifying, 51 Gene therapy, 199 Gutka, 248
traumatic, 4950, 50f Gene transmission, 195196 GVHD. See Graft-versus-host disease
Fibrosarcoma, 230t, 255 General pathology, 2 (GVHD)
Fibrous dysplasia, 19, 212213, 212f, 213f Genes, 195 Gynecomastia, 214
FICOD. See Florid cemento-osseous dominant vs. recessive, 196197
dysplasia (FICOD) oncogenes, 229
Field cancerization, 233234 Genetic and inherited disorders, 193225
H
Fight-or-flight response, 294 autosomal disorders, 196 Hair on end appearance, 301, 301f
Filiform papillae, 48 basic principles of, 194199 Hairy tongue, 22, 22f
Fissure, 12f, 16 of the dentition, 216221 Hamartomas, 215, 257
Fissured tongue, 7f, 169170, 170f dominant vs. recessive, 196197 Hand-Schller-Christian disease, 265
Fistula, 5960 of head and neck, 199221 Hand-foot-and-mouth disease, 106, 107f
5-year survival rate, 242243 jaws and facial structures, 202214 Haploid, 195
Flat lesions, 12f13f oral soft tissue disorders, 214216 Haptens, 127
Floor of mouth, oral cancer of, 238239, periodontium/gingiva, 199201 Hard palate, oral cancer of, 239240, 242f
329f penetrance and expressivity, 198 Hard tissue injuries, 6472
Florid cemento-osseous dysplasia syndromes, 199 bone injury, 6470
(FICOD), 6566, 65t, 66f X-linked disorders, 197, 198f external injuries to teeth, 7072, 71f, 71t
Focal cemento-osseous dysplasia (FCOD), Genetic mutations, 199, 260 Hardikar syndrome, 203t
62, 65, 65f, 65t Genetics Harelip. See Cleft lip
Focal epithelial hyperplasia, 111, 114f basics of, 194195 Hashimotos thyroiditis, 128t, 129, 292
Focal fibrous hyperplasia, 4950 study of, 194 Head and neck
Folate. See Folic acid Genotype, 198199 developmental cysts of, 179188
Foliate papillae, 22, 23f Geographic tongue (erythema migrans), examination of, 3, 4t6t
Folic acid, 299, 312, 313 4748, 48f genetic and inherited disorders of,
Follicle-stimulating hormone (FSH), 288, German measles, 107, 107f 199221
290t Germline mutations, 199 jaws and facial structures, 202214
Follicular cysts, 181182, 181f, 182f Gigantism, 290 oral soft tissue disorders, 214216
Folliculitis, 185 Gingiva periodontium/gingiva, 199201
Foramen Caecum, 167, 170 injuries, 5153 Healing, 3940
Fordyce granules, 21, 22f oral cancer of, 239, 240f Hecks disease, 111, 112t, 114, 114f
Foreign body gingivitis, 48 Gingival cyst, 180t, 183, 183f Heerfordts syndrome, 152
Foreign body reaction, 48 Gingival histoplasmosis, 98f Helper T cells, 123
Forensic odontology, 2 Gingival overgrowth, 52, 53f Hemangiomas, 257258, 258f
Forensic pathologists, 2 Gingivitis Hemarthroses, 305
Formalin, 19, 19f desquamative, 143146, 147f, 150, 151t Hematocrit, 300, 300t
Fracture, tooth, 72 foreign body, 48 Hematoma, 48
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334 Index
Hemifacial microsomia, 171, 171f, 204t Hyperpigmentation, 296, 296f Immunoglobulin E (IgE), 122
Hemochromatosis, 30 Hyperpituitarism, 290 Immunoglobulin G (IgG), 122, 126
Hemoglobin, 300 Hyperplasia, 28, 29t Immunoglobulin M (IgM), 122
Hemoglobin pigment, 178 Hyperplastic candidiasis, 94, 96, 96f, 97f Immunoglobulins (Igs), 121122, 123f
Hemoglobinopathies, 300303 Hyperplastic dental follicle, 187188, Immunologic disease, 119161
Hemolysis, 178 187t, 188f allergic disorders, 130133
Hemophilia, 305306 Hypersensitivity reactions, 126127, autoimmune diseases, 127129
Hemophilia A, 305 130133 clinical features of, 130156
Hemophilia B, 305306 to dental amalgam, 132133, 133f desquamative gingivitis, 143146, 147f
Hemosiderin, 30, 31f to dental restorative materials, 132133, hypersensitivity reactions, 126127
Hemosiderosis, 30 133f immunodeficiency diseases, 129130
Hemostasis, 303304 Hypertelorism, 205 immunopathology, 126130
Herbal supplements, 2 Hypertension, 2 lichen planus, 139142, 140f
Hereditary benign intraepithelial Hyperthermia, 202 lichenoid mucositis, 143, 143f
dyskeratosis (HBID), 214, 215f Hyperthyroidism, 128t, 129, 291292, systemic immunodysregulation, 147156
Hereditary gingival enlargement, 200201, 292f ulcerative conditions, 133139
201f, 201t Hypertrophy, 28, 29t Immunomodulary drugs, 68
Hereditary hemorrhagic telangiectasia Hypervitaminosis, 311 Immunopathology, 126130
(HHT), 306, 306f Hypocalcemia, 293 Immunosuppressive medicines, 129
Herpangina, 106 Hypocalcified amelogenesis imperfecta, Impetigo, 84, 84f
Herpes labialis, 102f 217, 217f, 217t Incidence, 232
Herpes simplex virus (HSV-1, HSV-2), Hypodontia, 173, 173f Incisional biopsy, 19
82f, 100102, 100t, 101f, 103f, Hypogammaglobulinemia, 129 Incisive canal cyst, 180t, 184, 184f
135t, 138 Hypoglycemia, 297t298t Incubator carrier, 78
Herpes stomatitis, 102, 102f Hypogonadism, 214 Indigenous microflora, 78
Herpes virus, 100106, 100t Hypohidrosis, 202 Indurated lesions, 235236
Herpes zoster, 5f, 103105, 104f, 105f Hypohidrotic ectodermal dysplasia, Infarction, 31
Herpetic conjunctivitis, 103 202203, 202f Infectious agents, 78
Herpetic encephalitis, 105 Hypomaturation amelogenesis imperfecta, Infectious diseases, 77117
Herpetic whitlow, 102103, 103f, 104f 216217, 217f, 217t bacterial infections, 8391
Herpetiform aphthous ulcers, 135136, 136f Hypomaturation/hypoplasia/taurodontism, actinomycosis, 8687, 86f
Hertwigs epithelial root sheath, 172, 173f 217 cat-scratch disease, 87, 87f
Highly active anti-retroviral therapy Hypoparathyroidism, 294 diphtheria, 86
(HAART), 110111, 138 Hypophosphatasia, 220221, 221f erysipelas, 84, 85f
Histamine, 35122 Hypopituitarism, 291 impetigo, 84, 84f
Histiocytes, 37 Hypoplastic amelogenesis imperfecta, leprosy, 91, 91f
Histoplasmin skin test, 97 216, 217f, 217t necrotizing ulcerative gingivitis,
Histoplasmosis, 9697, 98f Hypothalamus, 289 8384, 83f
HIV. See Human immunodeficiency virus Hypothyroidism, 292293 noma (Cancrum oris), 84
(HIV) Hypoxia, 31 scarlet fever, 85, 86f
HIV sialadenitis, 110f strep throat, 85, 85f
Hives, 147148, 147f syphilis, 88, 9091
HIV-salivary gland disease (HIV-SGD),
I tuberculosis, 8788, 89f
110 Iatrogenic burns, 47 basic principles of, 7883
Hodgkins lymphoma, 262, 262f Iatrogenic etiology, 3 carrier state, 7879
Hormones, 289. See also specific hormones Ibandronate, 66t constitutional symptoms, 78
adrenal, 294 IBD. See Inflammatory bowel disease (IBD) control of, 8283
definition of, 288 Icterus, 308 diagnostic procedures, 8182
parathyroid, 293 Immune cells, 121124, 122f fungal infections, 9199
pituitary gland, 288, 289t290t Immune complex-mediated reactions, angular cheilitis, 9394
thyroid, 288, 291 127 aspergillosis, 98
Human embryo, 4 weeks, 164f Immune response, 124126, 125f blastomycosis, 99
Human immunodeficiency virus (HIV), Immune system, 120126, 121f candidiasis, 9194, 92f, 93f, 9497,
108111, 130, 138, 259 function of, 120 96f, 97f
Human leukocyte antigens (HLAs), 120 immune cells and molecules, 121124, coccidioidomycosis, 99
Human papillomavirus (HPV), 111, 112t, 122f cryptoccosis, 99
113f, 114, 232, 239, 248 normal immune response, 124126 histoplasmosis, 9697, 97f, 98f
Humoral immunity, 125 structure of, 120121 mucormycosis, 9798, 98f
Hutchinsons incisors, 90, 91f Immunity infection and transmission of, 7981
Hutchinsons triad, 90 active, 126 opportunistic, 78, 130
Hydroxyproline, 20 cellular, 125126 oral manifestations of, 83114
Hyperbilirubinemia, 308, 308f humoral, 125 pathogenic microorganisms and, 7879
Hypercalcemia, 32, 220, 293 nonspecific, 124125 pathways of entry, 7980, 80f
Hyperchromatism, 231 passive, 126 protozoal infections, 99
Hyperdontia, 173, 174f specific, 125126 signs and symptoms of, 8182
Hyperglycemia, 294, 296 Immunization, 82 variables affecting outcomes of, 81f
Hyperkeratosis, 40, 42t Immunodeficiency diseases, 129130 viral infections, 100114
Hypermelanosis, 4145, 45t Immunoglobulin A (IgA), 122 enteroviruses, 106
Hyperparathyroidism, 293294, 293f Immunoglobulin D (IgD), 122123 herpes virus family, 100106, 100t
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336 Index
Index 337
Oral hairy leukoplakia (OHL), 105, 106f, Osteitis, condensing, 18f Pemphigoid
109 Osteoblastic stage, of COD, 64, 64f bullous, 144
Oral lymphoepithelial cyst, 180t, 186 Osteoblastoma, 280 cicatricial, 144
Oral melanotic macule, 4243, 44f, 45t Osteogenesis imperfecta (OI), 210211, mucous membrane, 143144, 144f,
Oral pathologic lesions 211f 145f, 151t
assessment of, 2 Osteolytic stage, of COD, 64, 64f Pemphigus vulgaris, 145146, 145f, 146f
due to injury, 28 Osteomalacia, 312 Penetrance, 198
Oral pathologists, 2 Osteomas, 5f, 278279, 279f Percussion, 3
Oral ranulas, 5354 Osteomyelitis, 17f, 6263, 63f Periapical cemental dysplasia (PCOD), 65,
Oral soft tissue Osteopetrosis, 211212, 211f 65t
disorders, 214216 Osteoradionecrosis, 245246, 246f Pericoronitis, 52, 53f
injuries. see Soft tissue injuries Osteosarcoma, 280, 281f Peri-implantitis, 58
metastases to, 260, 261f Ovaries, 289f Periodic acid Schiff stain, 82f
Oral squamous cell carcinoma, 232247, Over-the-counter (OTC) toothache Periodontal disease, 1, 109
238f, 249 remedies, 47 Periodontitis, 39
5-year survival rate, 243t Oxytocin, 290t apical, 16f
clinical features of, 234235 Periodontium/gingiva, disorders of,
dysplasia, 234235, 235f, 236f 199201
epidemiology and risk factors,
P Peripheral ameloblastoma, 272, 273f
232234 p arm, 194 Peripheral giant cell granuloma (PGCG),
high-risk locations for, 237240, 241f, Paget disease, 19, 6768, 67f 51, 52f
242f Palatal torus, 22f Peripheral ossifying fibroma (POF), 51
progression to, 235237 Palate. See also Hard palate; Soft palate Perlche, 298, 299f
Oral submucous fibrosis, 248249 cleft, 167168, 168f Pernicious anemia, 128t, 303, 303f, 313
Oral-Mandibular-Auricular syndrome, development of, 165166, 166f Persistent generalized lymphadenopathy
171 Palliative surgery, 244 (PGL), 109
Orofacial complex, developmental Pallor, 35 Petechiae, 304, 304f
disorders of, 163191 Palmoplantar keratosis, 200, 215 Peutz-Jeghers syndrome, 214215, 215f
Orofacial embryology, 164167 Palpation, 3 Peyers patches, 121
Orofacial granulomatosis (OFG), 153 Pamidronate, 66t PGCG. See Peripheral giant cell granuloma
Orofacial injury, 2776 Pan masala, 248 (PGCG)
cell and tissue response to, 2832 Pancreas, 289f, 296299 Phagocyte system, 37
hard tissue, clinical and radiographic Papillary lesion, 14f Phagocytes, 121, 123124
features, 6472 Papilloma, 113f Phagocytosis, 37
bone injury, 6470 Papillomatosis, 299 Pharyngeal arches, 165, 165f
external injuries to teeth, 7072, 71f, Papillon-Lefvre syndrome, 199200, Pharyngotonsillitis, 101
71t 200f Phenotype, 199
inflammatory response to, 3234 Papule, 11f, 15 Phenytoin (Dilantin), 28
postinflammatory tissue healing, Paracortex, 121 Physiologic atrophy, 28, 29
3840 Paramedian lip pits, 168 Physiologic hyperplasia, 28, 29
proliferative repair responses, 4951 Paramolar, 173 Physiologic hypertrophy, 28, 29
soft tissue, clinical features of, 4063 Parathyroid glands, 289f, 293294 Physiologic pigmentation, 21, 22f
amalgam tattoo, 41 disorders Pierre Robin sequence, 204t
body piercing, 49 hyperparathyroidism, 293294, 293f Pineal gland, 289f
denture and prosthesis-related injury, hypoparathyroidism, 294 Pink tooth of Mummery, 61
5658 Parathyroid hormone (PTH), 293 Pituitary dwarfism, 291
foreign body reaction, 48 Parulis, 60 Pituitary gland, 288, 289f, 290
frictional keratosis, 40, 41f Passive immunity, 126 disorders
geographic tongue, 4748, 48f Patau syndrome, 203t acromegaly, 290
hematoma, 48 Pathogenic microorganisms, infectious gigantism, 290
hyperkeratosis, 42t disease and, 7879 hyperpituitarism, 290
hypermelanosis, 4145 Pathogenicity, 8081 hypopituitarism, 291
injuries to gingiva, 5153 Pathognomonic, 20 hormones, 288, 289t290t
keloids, 4849 Pathologic atrophy, 28, 29 Plaque, 11f, 16
nicotine stomatitis, 47, 48f Pathologic calcification, 32 Plasma cell gingivitis, 132
pulpal injury, 5860 Pathologic hyperplasia, 28, 29 Plasma cells, 37, 37f, 121, 264
radiolucent periapical pathology, Pathologic hypertrophy, 28, 29 Plasma proteases, 36
6061 Pathology. See also Oral and maxillofacial Plasmacytoma, 264, 264f
radiopaque periapical pathology, pathology Platelets, 38, 38f
6163 definition of, 1 Pleomorphic adenoma, 252253, 252f,
traumatic salivary gland disorders, general, 2 253f
5356 mucosal, 9t15t Pleomorphism, 231
traumatic ulcer, 4547, 46f, 46t systemic, 2 Plummer-Vinson syndrome, 303
types of, 28 Patient assessment, 23 Plunging ranulas, 5354
Oropharyngeal carcinoma, 112t Patient history, 23 PMNs. See Polymorphonuclear leukocytes
Osler-Weber-Rendu disease, 306 Pedunculated lesion, 11f (PMNs)
Osseous dysplasia, 18f Peg lateral, 175, 175f Pneumonia, 109
Ossifying fibroma, 279, 280f Pellagra, 312 Polycythemia vera (PCV), 304305
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338 Index
Polydipsia, 298 R S
Polymorphonuclear leukocytes (PMNs),
34, 3637, 36f RA. See Rheumatoid arthritis (RA) Saliva substitutes, 245
Polymorphous low-grade adenocarcinoma Radiation caries, 245f Salivary function tests, 20
(PLGA), 254, 254f Radiation therapy, 244246 Salivary gland duct cyst, 5455
Polymyositis, 309t Radiation-induced mucositis, 245, Salivary gland neoplasia, 252255, 252f,
Polyphagia, 298 245f 252t
Polyuria, 298 Radiation-induced xerostomia, Salivary glands
Popliteal pterygium, 203t 244245 benign tumor of, 5f
Porcelain-fused-to-metal (PFM) crowns, Radiographic lesions, 16t19t metaplasia in, 29
133 Radiolucency, 16t17t radiosensitivity of, 244245
Port wine stain, 258 Radiolucent periapical pathology, 6061 traumatic salivary gland disorders,
Postherpetic neuralgia, 105 Radiopacity, 18t 5356
Postinflammatory hypermelanosis, 4142 Radiopaque periapical pathology, 6163 Sarcoidosis, 151152, 152f
Postinflammatory hyperpigmentation, Radiosensitivity, 244 Sarcomas, 230
4142, 44f, 45t Ramo syndrome, 201t Scaphocephaly, 206f
Postinflammatory tissue healing, 3840 Ranulas, 5354, 54f Scar, 16
Post-surgical hyperostosis, 69, 69f RAU. See Recurrent aphthous ulcer Scarlet fever, 85, 86f
Pregnancy tumors, 49 (RAU) Schwann cells, 256
Premature tooth loss, 221b Raynauds phenomenon, 154, 308309, Schwannoma, 256
Prevalence, 232 309f Sclerodactyly, 309, 310f
Primary hyperparathyroidism, 293 RBCs. See Red blood cells (RBCs) Sclerosis, 258
Primary immunodeficiency, 129130 Reactive process, 228 Sclerotic bone, 280
Primary intention, 39 Recessive genes, 196197 Scorbutic gingivitis, 313
Primary jaw tumors, 270 Recurrent aphthous ulcer (RAU), 134135, Sebaceous cysts, 185
Primary response, 126 134f, 135t Seborrheic keratosis (SK), 247248
Primary Sjgren syndrome, 154 Recurrent intraoral herpes, 102, 102f Secondary hyperparathyroidism, 293
Primary syphilis, 88 Red blood cells (RBCs), 36, 299300, Secondary immunodeficiency, 130
Primary tumors, 281 300t, 301, 304, 308 Secondary intention, 3940
Primordial cysts, 179t, 182 Red plaque, 236f Secondary Sjgren syndrome, 154155
Proliferative repair responses, 4951 Red strawberry tongue, 85, 86f Secondary syphilis, 88, 90f
pyogenic granuloma, 49, 49f Reduced enamel epithelium, 171172 Secondary TB, 88
traumatic (irritation) fibroma, 4950 Regeneration, 39 Selective IgA deficiency, 129
traumatic neuroma, 5051 Regional lymphadenopathy, 38, 87 Selective toxicity, 82
verruciform xanthoma, 51, 51f Relapsing polychondritis, 309t Septicemia, 246
Proliferative verrucous leukoplakia (PVL), Remission, 129 Serological tests, 20, 81
248 Renal osteodystrophy, 69 Serotonin, 3536
Proopiomelanocortin (POMC), 296 Resectable tumors, 244 Sessile lesion, 11f
Proteinuria, 150 Rests of Malassez, 172 Severe combined immunodeficiency
Protozoa, 79 Rests of Serres, 171, 183 (SCID), 129
Protozoal infections, 99 Reticular lesion, 13f Sex chromosomes, 196
Pruritic skin rash, 103 Reticular/papular lichen planus, 140142, Sexually transmitted diseases (STDs), 80,
Pseudocysts of the jaw, 187188, 187f, 141f 111
187t, 188f Retinoids, 200 SH3BP2 gene, 204
Pseudomembranous candidiasis, 7f, 92, Retromolar pad, oral cancer of, 239 Sialadenosis, 56
92f, 109 Reversible cell injury, 2830 Sialocyst, 5455
Pseudotumor of hemophilia, 305 atrophy, 2829 Sialagogues, 245
Pulp calcification, 34f dysplasia, 2930 Sialolithiasis, 5556, 56f
Pulp canal obliteration, 62 endogenous intracellular pigments, 30 Sickle cell crisis, 301
Pulp necrosis, 61 hyperplasia, 28 Sickle cell disorder, 301, 302f
Pulp polyp, 59, 59f hypertrophy, 28 Sickle cell trait, 301
Pulp stones, 62, 62f metaplasia, 29 Signs, 3
Pulpal injury, 5860 Review of Systems, 288 of infection, 8182
Pulpitis, 5859, 59t Rh disease, 178 of inflammation, 3435
Purified protein derivative (PPD) skin Rhabdomyoma, 259 Simple bone cavity, 187, 187t, 188f
test, 88 Rhabdomyosarcoma, 259 Sinus tract, 12f, 16, 59
Purpura, 304, 304f Rheumatoid arthritis (RA), 39, 128t, Sjgrens syndrome, 20, 128t, 129, 154156,
Pustule, 11f, 15, 104 309t 154f, 155f, 155t, 156f, 309t
PVL. See Proliferative repair responses Rhinocerebral mucormycosis, 98 SK. See Seborrheic keratosis (SK)
Pyogenic granuloma, 39, 49, 49f, 50f, Riboflavin, 312 Slurry, 41
263f Rickets, 312, 312f Smokeless tobacco, 241
Pyostomatitis vegetans, 6f , 307, 308f Risedronate, 66t Smokers melanosis, 44, 45f
Pyrogens, 38 Risk factors, 232 Smooth lesions, 14f
Pyroxidine, 312 Roseolovirus, 100t Snuff dippers keratosis, 40, 42t, 43f
Rough lesions, 14f15f Social history, 3
Rubella (German measles), 107, 107f Soft palate
Q Rubeola (measles), 106107 oral cancer of, 239, 239f
q arm, 194 Rutherford syndrome, 201t tumors of, 239
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Index 339
Soft tissue injuries, 4063 Systemic diseases, oral manifestations of, Th cells, 123
denture and prosthesis-related injury, 287316 Thalassemia major and minor, 300301
5658 amyloidosis, 310, 311f Thermal burns, 45, 46t
foreign body reaction, 48 anemia, 299303 Thiamine, 312
to gingiva, 5153 bleeding disorders, 303306 Three Ps, 51
to mucous membranes, 4049 connective tissue disorders, 308310 Thrombocytopenia, 261
amalgam tattoo, 41 endocrine disorders, 288299 Thrombus, 304305, 304f
body piercing, 49 gastrointestinal disorders, 306308 Thrush, 92
frictional keratosis, 40, 41f nutritional disorders, 311313 Thymus gland, 120, 289
geographic tongue, 48f vitamin deficiencies, 311313 Thyroxin (T4), 291
hematoma, 48 Systemic effects, of inflammation, 38 Thyroglossal duct cyst, 180t, 186, 186f
hyperkeratosis, 42t Systemic immunodysregulation, 147156 Thyroid gland, 291293
hypermelanosis, 4145 angioedema, 147f, 148 development of, 166167
keloids, 4849 bone marrow transplant rejection, 148, disorders
nicotine stomatitis, 47, 48f 149f, 150 hyperthyroidism (Graves disease),
traumatic ulcer, 4547, 46f, 46t Crohns disease, 152, 153f 128t, 129, 291292, 292f
proliferative repair responses, 4951 graft-versus-host disease, 148, 149f, 150 hypothyroidism, 292293
pulpal injury, 5860 granulomatous diseases, 151 hormones, 288, 290t, 291
radiolucent periapical pathology, 6061 hives, 147148, 147f lingual, 170, 170f
radiopaque periapical pathology, 6163 orofacial granulomatosis, 153 Thyroid-releasing hormone (TRH),
traumatic salivary gland disorders, sarcoidosis, 151152, 152f 288, 291
5356 Sjgrens syndrome, 154156, 154f, Thyroid-stimulating hormone (TSH),
Soft tissue neoplasia, 255260 155f, 156f 288, 290t, 291
Somatic mutations, 199 systemic lupus erythematosus, 150, Thyrotoxicosis, 291
Space infections, 60 150f Tissue
Specific immunity, 125126 systemic sclerosis, 154, 154f culture, 81
Spleen, 121 Systemic infections, 80 granulation, 39, 39f
Sporadic Burkitts lymphoma, 264 Systemic lupus erythematosus (SLE), healing, 3940
Sputum culture, 88 128t, 150, 150f, 309t regeneration, 39
Squamous cell carcinoma (SCC), 114, Systemic pathology, 2 repair, 39
230, 232, 238f Systemic sclerosis (scleroderma), 154, Tissue response, to injury, 2832
oral. see Oral squamous cell carcinoma 154f, 309t TMJ. See Temporomandibular joint
of the skin, 250251, 251f (TMJ)
Squamous odontogenic tumor (SOT), TNM Classification of Malignant Tumors
271t, 273
T (TNM), 242243, 243t
Squamous papilloma, 111, 112t T cells, 37, 120121, 125126, 130 Tobacco consumption, 3
Stafne defect, 187, 187f T lymphocytes, 120, 123 Tongue
Staging, of oral cancer, 242243, 243t Tachycardia, 292 aglossia, 168169
Static bone cavity, 187, 187f, 187t Talon cusp, 175, 175f ankyloglossia, 168169
STDs. See Sexually transmitted diseases Tattoo, amalgam, 8f bifid, 168
(STDs) Taurodontism, 177, 177f development of, 166167, 166f
Stem cell transplant, 261 TB. See Tuberculosis (TB) fissured, 7f, 169170, 170f
Stevens-Johnson syndrome, 139, 140f T-cell receptors, 123 hairy, 22, 22f
Sticklers syndrome, 203t Teeth macroglossia, 169, 170f
STK11 gene, 215 examination of, 9t microglossia, 169
Stomatitis, chronic ulcerative, 146, 147f, genetic and inherited disorders of, oral cancer of, 237238, 238f
151t 216221 piercings, 49
Strep throat, 85f causing premature tooth loss, 221b Tonsillar carcinoma, 112t
Streptococcal pharyngitis, 85, 85f cementum disorders, 220221 Tonsils, 121f
Stroke, 31, 60 dentin formation disorders, 218220 lingual, 23, 23f
Sturge-Weber syndrome, 258 enamel disorders, 216218 Tooth color abnormalities, 177178
Submental swelling, 6f injuries, external, 7072, 71f, 71t Tooth development, 171172, 172f
Subpontine hyperostosis, 58, 58f Telangiectasias, 306, 309 abnormalities, 172179
Sun damage, 237 Temporal arteritis, 309t concrescence, 174
Superficial mucocele, 54, 54f Temporomandibular joint (TMJ), 3, 279, congenitally missing teeth, 173
Supernumerary roots, 177, 177f 282 dens evaginatus, 176, 176f
Supernumerary teeth, 173, 174f diagnosis of, 20 dens invaginatus, 176f
Supplements, herbal, 2 osteoma of, 5f dilaceration, 176177, 177f
Surgery, for oral cancer, 244 Teratogens, 164, 167 enamel hypoplasia, 178179
Swollen glands, 38, 38f Teratology, 167 enamel pearl, 176, 176f
Symblepharon, 144, 145f Terminology, 3, 1516 fusion, 174, 174f
Symptomatic, 3 mucosal pathology, 9t15t gemination, 174, 174f
Symptoms, 3 radiographic lesions, 16t19t hyperdontia, 173, 174f
of infection, 8182 Tertiary intention, 40 macrodontia, 175
of inflammation, 3435 Tertiary syphilis, 90, 90f microdontia, 175
Syndactyly, 207 Testes, 289 peg lateral, 175, 175f
Syndromes, 199 Testosterone, 294 supernumerary roots, 177f
Syphilis, 88, 9091 Tetracycline stain, 177178, 178f talon cusp, 175, 175f
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340 Index