Chapter 3

Immunity

Elsevier items and derived items 2009 by Saunders, an imprint of Elsevier Inc. 1
 Outline
The Acquired Immune Response
Antigens
Cellular Involvement in the Immune Response
Major Divisions of the Immune Response
Memory and Immunity
Types of Immunity
Immunopathology
Oral Diseases with Immunologic Pathogenesis
Autoimmune Diseases that Affect the Oral
Cavity

Elsevier items and derived items 2009 by Saunders, an imprint of Elsevier Inc. 2
The Acquired Immune Response
(pg. 82)
Defends the body against injury
Has the capacity to remember and respond
more quickly the second time a foreign
material enters the body
Works with the inflammatory response and a
working repair process
Involves white blood cells, especially
lymphocytes and their products

Elsevier items and derived items 2009 by Saunders, an imprint of Elsevier Inc. 3
 Antigens
(pg. 82)
Foreign substances
Mainly proteins, often microorganisms and
their toxins
Human cells that have been transformed
May be tumor cells, or cells infected with
viruses

Elsevier items and derived items 2009 by Saunders, an imprint of Elsevier Inc. 4
 Antigens (cont.)
Human tissue
Organ transplants, tissue grafts, incompatible
blood types during a transfusion
Autoimmune diseases
Tissue from the persons own body becomes
an antigen

Elsevier items and derived items 2009 by Saunders, an imprint of Elsevier Inc. 5
 Cellular Involvement in the
Immune Response
B lymphocytes
T lymphocytes
Macrophages
Cytokines

Elsevier items and derived items 2009 by Saunders, an imprint of Elsevier Inc. 6
 Cellular Involvement in the
Immune Response (cont.)
(pg. 83)
Cells that are able to recognize and
respond to antigen
Derived from precursor cells in bone marrow
(stem cells)
20% to 25% of the WBC (white blood cell)
population
Two main types
B lymphocytes (B cells)
T lymphocytes (T cells)
Also there are natural killer cells (NK cells)
Can destroy cells recognized as foreign without
recognizing specific antigens
Elsevier items and derived items 2009 by Saunders, an imprint of Elsevier Inc. 7
Cellular Involvement in the
Immune Response (cont.)

Elsevier items and derived items 2009 by Saunders, an imprint of Elsevier Inc. 8
 B Lymphocytes
(pgs. 83-84)
Mature and reside in lymphoid tissue
Lymph nodes, tonsils, and other body tissue
B lymphocytes travel to the site of injury
when stimulated by antigen
Two main types
Plasma cells
Produce specific antibodies
B memory cell
Retains the memory of previously encountered antigen
and will clone itself in the presence of antigen

Elsevier items and derived items 2009 by Saunders, an imprint of Elsevier Inc. 9
B Lymphocytes (cont.)

Elsevier items and derived items 2009 by Saunders, an imprint of Elsevier Inc. 10
 Plasma Cells
Produce antibodies that are categorized
into 5 classes of immunoglobulins, which
are carried in blood serum
All have the same basic Y structure, but have
an area with variable (V) structure at the tips of
the Y
The stem of the Y is constant (C) for all 5
types, and links the antibody to other
components of the immune response
Immune complex
Antigen combined with antibody

Elsevier items and derived items 2009 by Saunders, an imprint of Elsevier Inc. 11
 T Lymphocytes
(pgs. 83, 85)
Travel to the thymus and mature
The thymus is large in an infant, shrinks as the
child matures
Several different types of T lymphocytes
Memory cells
T-helper cells
Increase functioning of B lymphocytes
T-suppressor cells
Turn off functioning of B lymphocytes
T-cytotoxic cells
Attack virally infected cells or tumor cells
Elsevier items and derived items 2009 by Saunders, an imprint of Elsevier Inc. 12
T Lymphocytes (cont.)

Elsevier items and derived items 2009 by Saunders, an imprint of Elsevier Inc. 13
 Macrophages
(pg. 84)
Active in phagocytosis of foreign material
Produce cytokines called monokines
Help both B and T lymphocytes
After phagocytosis, they process and present
antigen to lymphocytes
This stimulates lymphocytes to travel from
lymphoid tissue to the injury site
Amplify the immune response but do not
remember the antigen like lymphocytes

Elsevier items and derived items 2009 by Saunders, an imprint of Elsevier Inc. 14
 Cytokines (Cont.)

Elsevier items and derived items 2009 by Saunders, an imprint of Elsevier Inc. 15
 Cytokines (Cont.)
(pgs. 84-85) (Table 3-2)
Proteins made by cells that are able to
affect the behavior of other cells
Different cytokines have different functions.

Elsevier items and derived items 2009 by Saunders, an imprint of Elsevier Inc. 16
 Major Divisions of the Immune
Response
(pgs. 85-86)
Humoral response
B lymphocytes are the primary cells.
Involves production of antibodies
Cell-mediated response
T lymphocytes are the primary cells.
Lymphocytes may work alone or be assisted by
macrophages.
The cell-mediated portion regulates both major
responses.

Elsevier items and derived items 2009 by Saunders, an imprint of Elsevier Inc. 17
Major Divisions of the Immune
Response (cont.)

Elsevier items and derived items 2009 by Saunders, an imprint of Elsevier Inc. 18
 Memory and Immunity
(pg. 86)
The immune system has memory; the
inflammatory system does not.
Some lymphocytes retain memory of an
antigen after an initial encounter.
This means the immune response will be faster
and stronger the next time an antigen enters
the body.
The retained memory is called immunity.

Elsevier items and derived items 2009 by Saunders, an imprint of Elsevier Inc. 19
 Types of Immunity
Passive Immunity
Active Immunity

Elsevier items and derived items 2009 by Saunders, an imprint of Elsevier Inc. 20
 Passive Immunity
(pg. 86)
Using antibodies created by another
person to prevent infectious disease
Natural
When antibodies from the mother pass through the
placenta to the developing fetus
Acquired
When antibodies are acquired through an injection
Short lived but fast acting

Elsevier items and derived items 2009 by Saunders, an imprint of Elsevier Inc. 21
 Active Immunity
(pg. 86)
Antibodies created by the person
themselves
Natural
Protection conferred following survival from an
infectious disease
Acquired
Injection or ingestion of either altered pathogenic
microorganisms or products of those
microorganisms immunization with a vaccine

Elsevier items and derived items 2009 by Saunders, an imprint of Elsevier Inc. 22
 Immunopathology
(pg. 86)
The study of immune reactions involved in
disease
The immune system can malfunction and
cause tissue damage.
Hypersensitivity
Autoimmune diseases

Elsevier items and derived items 2009 by Saunders, an imprint of Elsevier Inc. 23
 Hypersensitivity (Cont.)
(pgs. 87-88) (Table 3-3)
An allergic reaction
An exaggerated response
Tissue destruction occurs as a result of the
immune response.
Four main types

Elsevier items and derived items 2009 by Saunders, an imprint of Elsevier Inc. 24
Hypersensitivity (Cont.)

Elsevier items and derived items 2009 by Saunders, an imprint of Elsevier Inc. 25
 Type I Hypersensitivity
(pg. 87)
Immediate (anaphylactic type)
The reaction occurs within minutes after
exposure to an antigen.
Plasma cells produce IgE.
IgE causes mast cells to release histamine, causing
increased dilation and permeability of blood vessels
and constricting smooth muscle in bronchioles of the
lungs.
The reaction may range from hay fever to
asthma and life-threatening anaphylaxis.

Elsevier items and derived items 2009 by Saunders, an imprint of Elsevier Inc. 26
 Type II Hypersensitivity
(pg. 87)
Cytotoxic type
Antibody combines with an antigen bound to
the surface of tissue cells, usually a circulating
RBC (red blood cell).
Activated complement components, IgG
and IgM antibodies in blood, participate in
this type of hypersensitivity reaction.
This destroys the tissue that has the antigens
on the surface of its cells (e.g., Rh
incompatibility).

Elsevier items and derived items 2009 by Saunders, an imprint of Elsevier Inc. 27
 Type III Hypersensitivity
(pg. 87)
Immune complex type (serum sickness)
Immune complexes are formed between
microorganisms and antibody in circulating
blood.
These complexes leave the blood and are deposited
in body tissues, where they cause an acute
inflammatory response.
Tissue destruction occurs following
phagocytosis by neutrophils.

Elsevier items and derived items 2009 by Saunders, an imprint of Elsevier Inc. 28
 Type IV Hypersensitivity
(pg. 87)
Cell-mediated type (delayed)
T lymphocytes that previously have been
introduced to an antigen cause damage to
tissue cells or recruit other cells.
Responsible for the rejection of tissue grafts
and transplanted organs

Elsevier items and derived items 2009 by Saunders, an imprint of Elsevier Inc. 29
 Hypersensitivity to Drugs
(pgs. 87-88)
Drugs can act as antigens.
Topical administration may cause a greater
number of reactions than oral or parenteral
routes.
But the parenteral route may cause a more
widespread and severe reaction.

Elsevier items and derived items 2009 by Saunders, an imprint of Elsevier Inc. 30
 Autoimmune Diseases
(pg. 88)
Immunologic tolerance
The body learns to determine self from nonself.
Autoimmune disorder
The recognition mechanism breaks down;
some body cells are not tolerated and are
treated as foreign antigens.

Elsevier items and derived items 2009 by Saunders, an imprint of Elsevier Inc. 31
 Immunodeficiency
(pg. 88)
An immunopathologic condition
A deficiency in number, function, or
interrelationships of the involved WBCs and
their products
May be congenital or acquired
Infections and tumors may occur as a result of
the deficiency.

Elsevier items and derived items 2009 by Saunders, an imprint of Elsevier Inc. 32
 Oral Diseases with Immunologic
Pathogenesis
Aphthous ulcers
Urticaria and angioedema
Contact mucositis and dermatitis
Fixed drug eruptions
Erythema multiforme
Lichen planus
Reactive arthritis (Reiter syndrome)
Langerhans cell disease

Elsevier items and derived items 2009 by Saunders, an imprint of Elsevier Inc. 33
 Aphthous Ulcers
(pgs. 88-91)
Painful oral ulcers with an unclear cause
Occur in about 20% of the population
Trauma is the most common precipitating
factor.
May be caused by emotional stress or certain food
May be associated with certain systemic
diseases
Thought to have an immunologic pathogenesis
Occur in three forms: minor, major, and
herpetiform

Elsevier items and derived items 2009 by Saunders, an imprint of Elsevier Inc. 34
 Minor Aphthous Ulcers
(pg. 89)
Discrete, round or oval ulcers
Occur on movable mucosa
Up to 1 cm in diameter with a erythematous halo
surrounding a yellowish-white fibrin surface
May have single or multiple lesions
May have a prodrome of 1 to 2 days

Elsevier items and derived items 2009 by Saunders, an imprint of Elsevier Inc. 35
Minor Aphthous Ulcers (cont.)

Elsevier items and derived items 2009 by Saunders, an imprint of Elsevier Inc. 36
 Major Aphthous Ulcers
(pgs. 89-90)
Larger (>1 cm), deeper, and longer lasting
than minor aphthous ulcers
Very painful
Occur in the posterior of the mouth more often
than minor aphthous ulcers
May require several weeks to heal
May require a biopsy

Elsevier items and derived items 2009 by Saunders, an imprint of Elsevier Inc. 37
Major Aphthous Ulcers (cont.)

Elsevier items and derived items 2009 by Saunders, an imprint of Elsevier Inc. 38
 Herpetiform Aphthous Ulcers
(pgs. 89-90)
Tiny (1 to 2 mm)
Resemble herpes simplex ulcers
Painful, generally occur in groups

Elsevier items and derived items 2009 by Saunders, an imprint of Elsevier Inc. 39
Diagnosis of Minor Aphthous
Ulcers (Cont.)

Elsevier items and derived items 2009 by Saunders, an imprint of Elsevier Inc. 40
 Diagnosis of Minor Aphthous
Ulcers (Cont.)
(pgs. 89-91) (Table 3-4)
Clinical appearance
Location
Herpetic lesions appear on mucosa fixed to
bone, aphthous lesions appear on movable
tissue
Clinical history
Aphthous ulcers do not produce systemic signs
or symptoms as do herpetic lesions

Elsevier items and derived items 2009 by Saunders, an imprint of Elsevier Inc. 41
Treatment of Minor Aphthous Ulcers
(pg. 91)
Treatment
There are several OTC medications such as
Orabase and Zilactin.
Topical or systemic steroids may help.
Topical anesthetic may help.

Elsevier items and derived items 2009 by Saunders, an imprint of Elsevier Inc. 42
 Urticaria (Hives)
(pg. 91)
Appears as multiple
areas of well-
demarcated swelling of
skin
May have itching (pruritis)
Lesions caused by
localized areas of
vascular permeability in
superficial connective
tissue

Elsevier items and derived items 2009 by Saunders, an imprint of Elsevier Inc. 43
 Angioedema
(pg. 91)
Lesions caused by
diffuse swelling due to
increased permeability
of deeper blood vessels
The skin covering the
swelling appears normal
Usually do not have
itching

Elsevier items and derived items 2009 by Saunders, an imprint of Elsevier Inc. 44
 Urticaria and Angioedema
(pgs. 91-92)
Often idiopathic cause
May be due to infection, trauma, emotional
stress, and certain systemic diseases
May be due to ingested allergens

Elsevier items and derived items 2009 by Saunders, an imprint of Elsevier Inc. 45
Contact Mucositis and Dermatitis
(pg. 92)
Lesions resulting from contact of an
allergen with skin or mucosa
Involves CMI (cell-mediated immunity)
The mucosa initially becomes erythematous
and edematous.
Often there is burning and pruritus
Later, the area becomes white and scaly.
Treatment
Topical and/or systemic corticosteroids

Elsevier items and derived items 2009 by Saunders, an imprint of Elsevier Inc. 46
Contact Mucositis and Dermatitis
(cont.)

Elsevier items and derived items 2009 by Saunders, an imprint of Elsevier Inc. 47
 Fixed Drug Eruptions
(pgs. 92-93)
Lesions that appear in the same site each
time a drug is introduced
Generally appear suddenly after a latent period
and subside when the drug is discontinued
May be single or multiple slightly raised,
reddish patches or clusters of macules on
the skin, or sometimes the mucous
membranes
May have pain or pruritis

Elsevier items and derived items 2009 by Saunders, an imprint of Elsevier Inc. 48
 Fixed Drug Eruptions (cont.)
A type of allergic reaction (Type III)
Immune complexes are deposited along the
endothelial walls of blood vessels.
Inflammation causes vasculitis with damage to
the vessel wall.
This creates erythema and edema in
superficial layers of skin or mucosa.
Treatment
The drug causing the reaction should be
identified and discontinued.

Elsevier items and derived items 2009 by Saunders, an imprint of Elsevier Inc. 49
 Erythema Multiforme
(pgs. 93-94)
Cause is not clear; may be a
hypersensitivity reaction
Most commonly occurs in young adults, affects
men more commonly than women
Target lesion
Characteristic skin lesion with concentric
erythematous rings alternating with normal skin
color

Elsevier items and derived items 2009 by Saunders, an imprint of Elsevier Inc. 50
Erythema Multiforme (cont.)

Elsevier items and derived items 2009 by Saunders, an imprint of Elsevier Inc. 51
 Erythema Multiforme (cont.)
(pgs. 93-94)
Skin lesions can range from macules to
papules to bullae.
Oral lesions are usually ulcers
Frequently form on lateral borders of the
tongue
Crusted and bleeding lips are frequently seen.
Gingival involvement is rare.
May be chronic or may have recurrent
acute episodes

Elsevier items and derived items 2009 by Saunders, an imprint of Elsevier Inc. 52
Erythema Multiforme (cont.)

Elsevier items and derived items 2009 by Saunders, an imprint of Elsevier Inc. 53
 Erythema Multiforme (cont.)
(pgs. 93-94)
Stevens-Johnson
syndrome
The most severe form
More extensive and
painful oral lesions
Genital mucosa and
mucosa of eyes may be
involved.
Lips generally are
encrusted and bloody.

Elsevier items and derived items 2009 by Saunders, an imprint of Elsevier Inc. 54
 Erythema Multiforme (cont.)
(pg. 93)
Diagnosis
Based on clinical features and by exclusion of
other diseases
Treatment and prognosis
Topical or systemic corticosteroids
Eye lesions may lead to blindness.

Elsevier items and derived items 2009 by Saunders, an imprint of Elsevier Inc. 55
 Lichen Planus
(pgs. 93-96)
A benign, chronic disease affecting skin
and oral mucosa
Unknown cause
Lesions have characteristic Wickham striae
Most commonly on buccal mucosa
Lesions may be on the tongue, lips, floor of
mouth, and gingiva.
Present in about 1% of the U.S. population
Most common in middle age
Slightly more common in women

Elsevier items and derived items 2009 by Saunders, an imprint of Elsevier Inc. 56
Lichen Planus (cont.)

Elsevier items and derived items 2009 by Saunders, an imprint of Elsevier Inc. 57
 Types of Lichen Planus
(pgs. 95-96)
Reticular lichen planus
Most common form
Erosive and bullous lichen planus
Epithelium separates from connective tissue
Desquamative gingivitis can be caused by
lichen planus.
Skin lesions
2 to 4 mm papules most commonly in lumber
region, flexor surfaces of the wrist, anterior
ankle
Elsevier items and derived items 2009 by Saunders, an imprint of Elsevier Inc. 58
Types of Lichen Planus (cont.)

Elsevier items and derived items 2009 by Saunders, an imprint of Elsevier Inc. 59
 Diagnosis of Lichen Planus
(pgs. 95-96)
Based on clinical
appearance and
possibly biopsy
Epithelial atypia
and dysplasia may
occur in lesions
that clinically
appear to be lichen
planus.
These lesions may
be premalignant.
Elsevier items and derived items 2009 by Saunders, an imprint of Elsevier Inc. 60
Treatment and Prognosis of Lichen
Planus
(pg. 96)
A chronic disease
Treated when symptomatic
Regular oral examination and biopsy of
suspicious lesions are necessary as these
patients may be at increased risk of
development of squamous cell carcinoma.

Elsevier items and derived items 2009 by Saunders, an imprint of Elsevier Inc. 61
 Reactive Arthritis
(Reiter Syndrome)
(pgs. 96-97)
Classic syndrome includes arthritis, urethritis, and
conjunctivitis, but all components may not be
present.
An antigenic marker called HLA-B27 is present in most
patients, meaning there may be a genetic influence.
Probably an abnormal immune response to a microbial
antigen
Skin and mucous membrane lesions may be
observed.
May see aphthous ulcers, erythematous lesions,
and geographic tonguelike lesions

Elsevier items and derived items 2009 by Saunders, an imprint of Elsevier Inc. 62
 Reactive Arthritis
(Reiter Syndrome) (cont.)

Elsevier items and derived items 2009 by Saunders, an imprint of Elsevier Inc. 63
 Reactive Arthritis
Diagnosis
Clinical signs and symptoms
HLA-B27 antigenic marker
Treatment and prognosis
Disease lasts for weeks to months.
Recurrent attacks are common.

Elsevier items and derived items 2009 by Saunders, an imprint of Elsevier Inc. 64
 Langerhans Cell Disease
(Histiocytosis X)
(pgs. 97-98)
Includes three entities
Letterer-Siwe disease
Hand-Schuller-Christian disease
Solitary eosinophilic granuloma
All have Langerhans cells and eosinophils.

Elsevier items and derived items 2009 by Saunders, an imprint of Elsevier Inc. 65
Langerhans Cell Disease
(Histiocytosis X) (cont.)

Elsevier items and derived items 2009 by Saunders, an imprint of Elsevier Inc. 66
 Langerhans Cell Disease
A type of macrophage
An immunocompetent cell of the mononuclear
phagocyte series and participates in CMI
Treatment
Eosinophilic granuloma is treated by
conservative surgical excision.
Low-dose radiation may be used

Elsevier items and derived items 2009 by Saunders, an imprint of Elsevier Inc. 67
Autoimmune Diseases that Affect the
Oral Cavity
Sjgren Syndrome
Systemic Lupus Erythematosus
Pemphigus Vulgaris
Mucous Membrane Pemphigoid
Bullous Pemphigoid
Behet Syndrome

Elsevier items and derived items 2009 by Saunders, an imprint of Elsevier Inc. 68
 Autoimmune Diseases that Affect
the Oral Cavity (cont.)
(pgs. 98-105) (Table 3-5)
Several autoimmune diseases affect the oral
cavity.
The immune system treats the persons own
cells and tissues as antigens.

Elsevier items and derived items 2009 by Saunders, an imprint of Elsevier Inc. 69
Autoimmune Diseases that Affect
the Oral Cavity (cont.)

Elsevier items and derived items 2009 by Saunders, an imprint of Elsevier Inc. 70
 Sjgren Syndrome
(pgs. 99-100)
Affects the salivary and lacrimal glands
Results in a decrease in saliva and tears
causing a dry mouth (xerostomia) and dry eyes
(xerophthalmia)
The combination may be called sicca
syndrome.

Elsevier items and derived items 2009 by Saunders, an imprint of Elsevier Inc. 71
 Sjgren Syndrome (cont.)
(pg. 99)
May be associated with other
autoimmune disorders
Primary Sjgren syndrome
when it occurs alone
Secondary Sjgren syndrome -
when it occurs with other
autoimmune disorders
Patient may complain of oral
discomfort due to dry mouth.
May see loss of filiform and
fungiform papillae on the
dorsum of the tongue
Elsevier items and derived items 2009 by Saunders, an imprint of Elsevier Inc. 72
 Sjgren Syndrome (cont.)
(pg. 99)
Affects both major and minor salivary
glands
Parotid gland enlargement occurs in about
50% of patients.
Biopsy reveals a characteristic
appearance.
Major salivary glands
Replacement with lymphocytes and the presence of
islands of epithelium called epimyoepithelial islands
Minor salivary glands
Aggregates of lymphocytes surrounding the salivary
gland ducts

Elsevier items and derived items 2009 by Saunders, an imprint of Elsevier Inc. 73
Sjgren Syndrome (cont.)

Elsevier items and derived items 2009 by Saunders, an imprint of Elsevier Inc. 74
 Sjgren Syndrome (cont.)
Patient may complain of burning and
itching of eyes and photophobia.
Severe eye involvement may lead to ulceration
and opacification of the eyes.
Raynaud phenomenon
20% of these patients will have this disorder
affecting fingers and toes
Initial pallor and subsequent cyanosis of skin due to
cold or stress
Hyperemia when blood vessels are warmed

Elsevier items and derived items 2009 by Saunders, an imprint of Elsevier Inc. 75
 Sjgren Syndrome (cont.)
90% of these patients have a positive
response to rheumatoid factor, an antibody
to IgG present in serum
It is an antibody to an antibody.
Other autoantibodies, anti-Sjgren
syndrome A, and anti-Sjgren syndrome B
are also present.

Elsevier items and derived items 2009 by Saunders, an imprint of Elsevier Inc. 76
 Diagnosis and Management of
Sjgren Syndrome
(pg. 100)
Diagnosis is made when two of three
components are present.
Xerostomia
Measurement of salivary flow and biopsy can help
Keratoconjunctivitis sicca
Confirmed with eye examination
Rheumatoid arthritis
For most patients, the course of the
disease is chronic and benign but these
patients are at risk for the development of
other more serious diseases.
Elsevier items and derived items 2009 by Saunders, an imprint of Elsevier Inc. 77
Diagnosis and Management of
Sjgren Syndrome (cont.)

Elsevier items and derived items 2009 by Saunders, an imprint of Elsevier Inc. 78
Treatment of Sjgren Syndrome
(pg. 100)
Symptomatic
Nonsteroidal antiinflammatory agents for
arthritis
May need corticosteroids and immunosuppressive
drugs for severe cases
Saliva substitutes for xerostomia
Humidifier, sugarless gum, or lozenges
Pilocarpine
Glasses and/or artificial tears to protect eyes

Elsevier items and derived items 2009 by Saunders, an imprint of Elsevier Inc. 79
 Systemic Lupus Erythematosus
(SLE)
(pgs. 100-102)
An acute and chronic inflammatory
autoimmune disease
No known cause
Affects women 8 times more frequently
than men, predominantly during
childbearing years
Three times more frequent in black women
than in white women

Elsevier items and derived items 2009 by Saunders, an imprint of Elsevier Inc. 80
 Systemic Lupus Erythematosus
(SLE) (cont.)
A syndrome with a wide range of disease
activity
Usually chronic and progressive
Periods of remission and exacerbation
Autoantibodies to DNA are present in
serum
May have a genetic component

Elsevier items and derived items 2009 by Saunders, an imprint of Elsevier Inc. 81
 Clinical Features of Systemic
Lupus Erythematosus (SLE)
(pgs. 101-102)
Skin lesions occur in 85% of individuals
Butterfly rash on bridge of nose
May be erythematous lesions on fingertips
Arthritis and arthralgia are common.
Oral lesions accompany skin lesions in
about 25% of patients with discoid LE.
Erythematous plaques or erosions
May have white striae; resemble lichen planus but
are less symmetric

Elsevier items and derived items 2009 by Saunders, an imprint of Elsevier Inc. 82
Clinical Features of Systemic Lupus
Erythematosus (SLE) (cont.)

Elsevier items and derived items 2009 by Saunders, an imprint of Elsevier Inc. 83
 Diagnosis of Systemic Lupus
Erythematosus (SLE)
(pgs. 101-102)
Usually based on multiorgan involvement
and presence of antinuclear antibodies in
serum
Inflammatory infiltrate is around blood vessels
in connective tissue.

Elsevier items and derived items 2009 by Saunders, an imprint of Elsevier Inc. 84
Treatment and Prognosis of Systemic
Lupus Erythematosus (SLE)
Aspirin and antiinflammatory drugs for mild
signs and symptoms
Hydroxychloroquine and corticosteroids along
with immunosuppressive agents may be used.
The text recommends consultation with the
patients physician before initiating dental
treatment.

Elsevier items and derived items 2009 by Saunders, an imprint of Elsevier Inc. 85
 Pemphigus Vulgaris
(pgs. 102-104)
A severe, progressive autoimmune
disease affecting skin and mucous
membranes
Characterized by intraepithelial blister
formation resulting from acantholysis, a
breakdown of cellular adhesion between
epithelial cells
Genetic and ethnic factors have been
reported.
Often seen in Ashkenazic Jews
Elsevier items and derived items 2009 by Saunders, an imprint of Elsevier Inc. 86
 Pemphigus Vulgaris (cont.)
(pg. 103)
Oral lesions
The first signs of disease occur in the oral
cavity in more than 50% of cases.
May be shallow ulcers, to fragile vesicles, to bullae
Nikolsky sign
Rubbing with a finger can produce a bullae.

Elsevier items and derived items 2009 by Saunders, an imprint of Elsevier Inc. 87
Pemphigus Vulgaris (cont.)

Elsevier items and derived items 2009 by Saunders, an imprint of Elsevier Inc. 88
 Pemphigus Vulgaris (cont.)
(pgs. 103-104)
Skin lesions
Erythema, bullae,
erosions, ulcers
Microscopic
appearance
Acantholytic cells
The loss of
attachment
between epithelial
cells leads to cells
that appear
rounded.
Tzanck cells
Elsevier items and derived items 2009 by Saunders, an imprint of Elsevier Inc. 89
 Diagnosis of Pemphigus Vulgaris
Made by biopsy and microscopic
examination
Direct immunofluorescence
Identifies autoantibodies present in tissue
Indirect immunofluorecence
The patients serum is used to detect circulating
autoantibodies.

Elsevier items and derived items 2009 by Saunders, an imprint of Elsevier Inc. 90
 Treatment and Prognosis of
Pemphigus Vulgaris
High doses of corticosteroids
May include immunosuppressive drugs
Mortality rate of 8% to 10% in 5 years is
related to complications of corticosteroid
treatment.

Elsevier items and derived items 2009 by Saunders, an imprint of Elsevier Inc. 91
 Mucous Membrane Pemphigoid
(Cicatricial Pemphigoid) (BMMP)
(pgs. 104-105)
A chronic autoimmune disease
Affects oral mucosa, conjunctiva, genital
mucosa, and skin
Not as severe as pemphigus vulgaris
Gingival lesions have been called
desquamative gingivitis, but this may be seen
with lichen planus and pemphigus as well.
Will see positive Nikolsky sign

Elsevier items and derived items 2009 by Saunders, an imprint of Elsevier Inc. 92
 Mucous Membrane Pemphigoid
(Cicatricial Pemphigoid) (BMMP) (cont.)

Elsevier items and derived items 2009 by Saunders, an imprint of Elsevier Inc. 93
 Diagnosis of Mucous Membrane
Pemphigoid
(pg. 104)
Made by biopsy and
histologic
examination
No degeneration of
epithelium occurs
An inflammatory
infiltrate, usually with
predominant plasma
cells and eosinophils,
is seen in connective
tissue.

Elsevier items and derived items 2009 by Saunders, an imprint of Elsevier Inc. 94
 Treatment and Prognosis of
Mucous Membrane Pemphigoid
A chronic disease with a benign course
Topical corticosteroid for mild cases
Systemic corticosteroids may be required for
more severe cases.
Eye lesions can lead to eye damage.

Elsevier items and derived items 2009 by Saunders, an imprint of Elsevier Inc. 95
 Bullous Pemphigoid
(pg. 105)
Some investigators believe bullous and
mucous membrane pemphigoid are
variants of a single disease, but 80% of
these patients are older than 60.
Oral lesions are less common than in cicatricial
pemphigoid.
Treatment
Systemic corticosteroids and nonsteroidal
antiinflammatory drugs

Elsevier items and derived items 2009 by Saunders, an imprint of Elsevier Inc. 96
 Behet Syndrome
(pg. 105)
A chronic, recurrent autoimmune disease
Primarily oral ulcers, genital ulcers, ocular
inflammation
No sex predilection; mean onset is 30 years
Autoantibodies to human mucosa may be
found.
Oral ulcers are similar in appearance to
aphthous ulcers.

Elsevier items and derived items 2009 by Saunders, an imprint of Elsevier Inc. 97
Behet Syndrome (cont.)

Elsevier items and derived items 2009 by Saunders, an imprint of Elsevier Inc. 98
Diagnosis of Behet Syndrome
Requires that two of three types of lesions
(oral, genital, and ocular) be present.
A pustular lesion after needle puncture
suggests Behet syndrome.

Elsevier items and derived items 2009 by Saunders, an imprint of Elsevier Inc. 99
 Treatment and Prognosis of
Behet Syndrome
(pg. 105)
Systemic and topical corticosteroids
Chlorambucil is used for ocular lesions.

Elsevier items and derived items 2009 by Saunders, an imprint of Elsevier Inc. 100
 Discussion Questions
What is an autoimmune disorder?
What is the difference between an antigen and
an antibody?
What are the differences between active and
passive immunity?
What oral diseases have an immunologic
pathogenesis?
What is an autoimmune disorder?
What are the oral symptoms of Sjgren
syndrome?
What are differences between pemphigus and
pemphigoid?
Elsevier items and derived items 2009 by Saunders, an imprint of Elsevier Inc. 101