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BSc, MB BS (Lond), FRCP

Consultant Dermatologist
The Leicester Royal Infirmary, Leicestev, and Honorary Senior Lecturer in Dermatology,
University of Leicester School oJMedicine

MB BS (Lond), FRCP
Emeritus Consultant Dermatologist
The Leicester Royal Infirmary, Leicester

Eighth Edition

To all medical students, and t o our children:
James, Matthew, John Joseph and David.
C3 1965, 1969, 1973, 1977, 1983, 1990, 1996, 2002 by Blackwell Science Ltd
a Blackwell Publishing Company

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First published 1965 Fourth edition 1977 Seventh edition 1996 Reprinted 2003, 2004, 2005
Second edition 1969 Fifth edition 1983 Reprinted 2001
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Library o f Congress Cataloging-in-Publication Data

Graham-Brown, R. A. C. (Robin A. C.)
Lecture notes on dermatology.+th ed./Robin Graham-Brown. Tony Burns.
p. cm.
Includes index.
ISBN 0-632-06494-3 (pbk.)
I. Dermatology-Outlines, syllabi, etc. 2. Skin-Diseases-Outlines, syllabi, etc.
[DNLM: l. Skin Diseases. W R 140 G742L 20021 1. Title: Dermatology.
II. Burns, Tony, FRCP. Ill. Title.
RL74.3.G73 2002
6 1 6.5--dc2 I 2001008761

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Preface, iv
Acknowledgements, v

Structure and Function o f the Skin, Hair and Nails, I

Approach t o the Diagnosis of Dermatological Disease, 9
Bacterial and Viral Infections, 17
Fungal Infections, 28
Ectoparasite Infections, 37
Acne, Acneiform Eruptions and Rosacea, 48
Eczema, 58
Psoriasis, 67
Benign and Malignant SkinTumours, 77
Naevi, 94
Inherited Disorders, 102
Pigmentary Disorders, 1 09
Disorders o f the Hair and Nails, I 14
Bullous Disorders, 122
Miscellaneous Erythematous and Papulosquamous Disorders and
Light-induced Skin Diseases, 130
Vascular Disorders, 14 1
Connective Tissue Disorders, 148
Pruritus, 156
Systemic Disease and the Skin, 16 1
Skin and the Psyche, 169
Cutaneous Drug Reactions, 174
Treatment o f Skin Disease, 180

Index, 185
In this, the eighth edition of Lecture Notes on Dermatology, we have updated the text
with particular regard t o advances in treatment. Numerous tables of salient points
provide ready reference but, as in previous editions, we have attempted t o create a
'user-friendly' readability.
W e hope that the book will be of value not only t o medical students, but also t o
general practitioners, and nurses involved in the care of dermatology patients. W e
also hope that exposure t o Lecture Notes on Dermatology will prompt a deeper
interest in this important medical specialty.

Robin Graham-Brown
Tony Burns

We would like to thank Drs lmrich Sarkany and Charles Calnan, under whose guid-
ance we both learned dermatology, and we are grateful to them for some of the
illustrations.We are also grateful t o our students, who remind us constantly of the
importance of clarity in communication. Finally, we thank the staff at Blackwell
Publishingwho have helped us through the editingand production stages.
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Structure and Function of
the Skin, Hair and Nails

Skinstructurc,l Functions ofthe skin, 6

Skin,skin is o wonderful thing, (basal layer), move progressively towards the

Keeps the outside out and the inside in. skin surface, and as they ascend they undergo a
I t is essential t o have some baclqround lknowl- process lknown as 'terminal differentiation' t o
edgeofthe normal structure and function of any produce the surface layer of cells (stratum
organ before you can hope t o understand the corneum). Components o f the internal skele-
abnormal. Skin is the icing on the anatomical ton of all cells include so-called 'intermediate
cale, it is the decorative wrapping paper, and filaments' which, in epithelia1 cells, are com-
without it not only would we all look rather posed of a group of fibrous proteins lknown
unappealing, but a variety o f unpleasant physio- as lkeratins, each of which is the product of a
logical phenomena would bring about our separate gene. Mutations in these genes are
demise.You have probably never contemplated responsible for certain sldn diseases. During
your sldn a great deal, except in the throes of differentiation, keratin filaments in the corneo-
narcissistic admiration, o r when it has been cytes aggregate under the influenceof filoggriha
blemished by some disorder, but hopefully by process lknown as lkeratinization, and bundles of
the end of this first chapter you will have been filaments form a complex intracellular networlc
persuaded that it is quite a remarkable organ, embedded in an amorphous protein matrix
and that you are lucky t o be on such intimate derived from the keratohyalin granules of the
terms with it. granular layer.
A cell ralkes approximately 8-10 w e e l ~t o
pass from the basal layer t o the surface of the
Skin structure epidermis (epidermal transit time), and loss of
cells from the surface is matched by production
The skin is composed of t w o layers, the epider- in the basal layer so that epidermal thickness is
mis and the dermis.The epidermis, which is the constant.This balance is maintained by growth
outer layer, and its appendages (hair, nails, seba- stimulators and growth inhibitors such as epi-
ceous glands and sweat glands), are derived dermal growth factor (EGF) and transforming
from the embryonic ectoderm.The dermis is of growth factors alpha and beta.The cells on the
mesodermal origin. surface of the skin (squames o r corneocytes),
which constitute thestratum corneum, are ker-
The epidermis atinized dead cells that are gradually abraded by
The epidermis is a stratified squamous epithe- daily wear and tear. If you batheafter a period of
lium, with several well-defined layers. The several days' avoidance of water (a house with-
principal cell type i s lknown as a'leratinocyte'. out central heating in mid-winter, somewhere
Keratinocyter, which are produced by cell in the Northern Hemisphere, i s ideal for this
division in the deepest layer of the epidermis experiment), you will note that as you towel
2 Chapter I :Structure and Function of the Skin, Hair and Nails

Fig. 1.1The epidermis.

yourself you are rubbing off small balls of kera- rate, and persist through the full thickness of the
tin -which has built up because of your insani- epidermis. The main stimulus t o melanin pro-
tary habits.When a plaster cast is removed from duction is ultraviolet (UV) radiation. Melanin
a fractured limb after several weeks in situ there protects the cell nuclei in the epidermis from
is usually a thick layer of surface keratin, the the harmful effects of UV radiation. A sun tan is
removal of which provides hours of absorbing a natural protective mechanism, not a cosmetic
occupational therapy. boon! Skin neoplasia is extremely uncommon in
Look at the layers more closely (Fig. I.I).The dark-skinned races because their skin is pro-
basal layer is composed of columnar cells which tected from UV damage by the large amounts of
are anchored t o a basement membrane-a mul- melanin i t contains.This is not the case in the
tilayered structure from which anchoring fibrils pale, pimply lager-swilling advert for British
extend into the superficial dermis. Interspersed manhood who dashes onto the beach in Ibiza
among the basal cells are melanocytes-large and flash-fries himself t o lobster thermidor on
dendritic cells derived from the neural crest- day one of his annual holiday.
which are responsible for melanin pigment The prickle cell layer acquires its name from
production. Melanocytes contain cytoplasmic the spiky appearance produced by intercellular
organelles called melanosomes, in which bridges (desmosomes) that connect adjacent
melanin is synthesized from tyrosine. The cells. Scattered throughout the prickle cell layer
melanosomes migrate along the dendrites of are Langerhans' cells. These dendritic cells are
the melanocytes, and are transferred t o the probably modified macrophages, which origin-
keratinocytes in the prickle cell layer. In white ate in the bone marrow and migrate t o the epi-
people the melanosomes are grouped together dermis.They are the first line of immunological
in membrane-bound 'melanosome complexes', defence against environmental antigens, and are
and they gradually degenerate as the ker- responsible for the uptake of such antigens and
atinocytes move towards the surface of the their presentation t o immunocompetent lym-
skin. In black people, the skin contains the same phocytes so that an immune response can be
number of melanocytes as that of white people, mounted.
but the melanosomes are larger, remain sepa- Above the prickle cell layer is the granular
Skin structure 3

layer, which is composed of flattened cells con- Apocrine sweat glands .

taining numerous darkly staining particles Apocrine sweat glands are found principally in
known as keratohyalin granules. Also present in the axillae and anogenital region. Specialized
the cytoplasm of cells in the granular layer are apocrine glands include the wax glands of the
organelles known as lamellar granules (Odland ear and the milk glands of the breast. Apocrine
bodies). These contain lipids and enzymes, and glands are also composed of a secretory coil
they discharge their contents into the intercel- and a duct, but the duct opens into a hair follicle,
lular spaces between the cells of the granular not directly onto the surface of the skin. Apo-
layer and stratum corneum-providing the crine glands produce an oily secretion contain-
equivalent of 'mortar' between the cellular ing protein, carbohydrate, ammonia and lipid.
'bricks', and contributingt o the barrier function These glands become active at puberty, and
of the epidermis. secretion is controlled by adrenergic nerve
The cells of the stratum corneum are flat- fibres. Pungent axillary body odour (axillary
tened, keratinized cells which are devoid of bromhidrosis) is the result of the action of bac-
nuclei and cytoplasmic organelles. Adjacent teria on apocrine secretions. In some animals
cells overlap at their margins, and this locking apocrine secretions are important sexual at-
together of cells, together with intercellular tractants, but the average human armpit pro-
lipid, forms a very effective barrier.The stratum vides a different type of overwhelming olfactory
corneum varies in thickness according t o the experience.
region of the body. It is thickest on the palms of
the hands and soles of the feet. Hair
Hairs grow out of tubular invaginations of the
Epidermal appendages epidermis known as follicles, and a hair follicle
The eccrine and apocrine sweat glands, the hair and its associated sebaceous glands are referred
and sebaceous glands, and the nails, constitute t o as a 'pilosebaceous unit'. There are three
the epidermal appendages. types of hair: fine, soft lanugo hair is present in
utero and is shed by the eighth month of fetal life;
Eccrine sweat glands vellus hair is the fine downy hair which covers
Eccrine sweat glands are important in body most of the body except those areas occupied
temperature regulation. A human has between by terminal hair; thick and pigmented term-
two and three million eccrine sweat glands cov- inal hair occurs on the scalp, eyebrows and
ering almost all the body surface.They are par- eyelashes before puberty-after puberty, under
ticularly numerous on the palms of the hands the influence of androgens, secondary sexual
and soles of the feet. Each consists of a secret- terminal hair develops from vellus hair in the
ory coil deep in the dermis, and a duct that con- axillae and pubic region, and on the trunk and
veys the sweat t o the surface. Eccrine glands limbs in men. O n the scalp, the reverse occurs
secrete water, electrolytes, lactate, urea and in male-pattern balding-terminal hair becomes
ammonia.The secretory coil produces isotonic vellus hair under the influence of androgens. In
sweat, but sodium chloride is reabsorbed in the men, terminal hair on the body usually increases
duct so that sweat reachingthe surface is hypo- in amount as middle age arrives, and hairy ears
tonic. Patients sufferingfrom cystic fibrosis have are a puzzling accompaniment of advancing
defective resorption of sodium chloride, and years. One struggles t o think of any biological
rapidly become salt depleted in a hot environ- advantage that hairyears might confer.
ment. Eccrine sweat glands are innervated by Hair follicles extend into the dermis at an
the sympathetic nervous system, but the neuro- angle (Fig. 1.2). Asmall bundle of smooth muscle
transmitter is acetylcholine. fibres, the arrector pili muscle, is attached t o
the side of the follicle.
Arrector pili muscles are supplied by adren-
4 Chapter I: Structure and Function of the Skin, Hair and Nails

Fig. 1.2 The structure of the


ergic nerves, and are responsible for the erec-

tion of hairs in the cold o r during emotional
stress ('goose flesh', 'goose pimples', horripila-
tion). The duct of the sebaceous gland enters
the follicle just above the point of attachment of
the arrector pili muscle. A t the lower end of the
follicle is the hair bulb, part of which, the hair
matrix, is a zone of rapidly dividing cells which is
responsible for the formation of the hair shaft.
Hair pigment is produced by melanocytes in the
hair bulb. Cells produced in the hair bulb be-
come densely packed, elongated and arranged
parallel t o the long axis of the hair shaft. They
gradually become keratinized as they ascend in Fig. 1.3 The structure of hair.
the hair follicle.
The main part of each hair fibre is the cortex,
which is composed of keratinized spindle- tical in cross-section and Mongoloid hair is
shaped cells (Fig. 1.3).Terminal hairs have a cen- circular.
tral core known as the medulla, consisting of The growth of each hair is cyclical -periods
specialized cells which contain air spaces. Cov- of active growth alternate with resting phases.
ering the cortex is the cuticle, a thin layer of cells After each period of active growth (anagen)
which overlap like the tiles on a roof, with the the? is a short transitional phase (catagen), fol-
free margins ofthe cells pointing towards the tip lowed by a resting phase (telogen), after which
of the hair. The cross-sectional shape of hair the follicle reactivates, a new hair is produced,
varies with body site and race. Negroid hair is and the old hair is shed. The duration of these
distinctly oval in cross-section, and pubic, beard cyclical phases depends on the age of the indi-
and eyelash hairs have an oval cross-section in all vidual and the location of the follicle on the
racial types. Caucasoid hair is moderately ellip- body.The duration of anagen in a scalp follicle is
Skin structure 5

genetically determined, and ranges from 2 t o glands are part of the pilosebaceous unit, and
more than 5 years.This is why some women can their lipid-rich product (sebum) flows through a
grow hair down t o their ankles, whereas most duct into the hair follicle. They are holocrine
have a much shorter maximum length. Scalp hair glands -sebum is produced by disintegration of
catagen lasts about 2 weeks and telogen from 3 glandular cells rather than an active secretory
t o 4 months.The daily growth rate of scalp hair process. Modified sebaceous glands which open
is approximately 0.45 mm. The activity of each directly on the surface are found on the eyelids,
follicle is independent of that of its neighbours, lips, nipples, glans penis and prepuce, and the
which is fortunate, because if follicular activity buccal mucosa (Fordyce spots).
was synchronized, as it is in some animals, we Sebaceous glands are prominent at birth,
would be subject t o periodic moults, thus under the influence of maternal hormones, but
adding another dimension t o life's rich tapestry. atrophy soon after, and do not enlarge again
A t any one time approximately 85% of scalp until puberty. Enlargement of the glands and
hairs are in anagen, I % in catagen and 14% in sebum production at puberty are stimulated by
telogen. The average number of hairs shed androgens. Growth hormone and thyroid hor-
daily is 100. In areas other than the scalp anagen mones also affect sebum production.
is relatively short-this is also fortunate, be-
cause if it was not so, we would all be kept Nails
busy clipping eyebrows, eyelashes and nether A nail is a transparent plate of keratin derived
regions. from an invagination of epidermis on the dor-
It is a myth that shaving increases the rate of sum of the terminal phalanx of a digit (Fig. 1.4).
growth of hair and that it encourages the devel- The nail plate is the product of cell division in the
opment of'thicker' hair; nor does hair continue nail matrix, which lies deep t o the proximal nail
growing after death-shrinkage of soft tissues fold, but is partly visible as the pale 'half-moon'
around the hair produces this illusion. (lunula) at the base of the nail. The nail plate is
Human hair colour is produced by two types firmly adherent t o the underlying nail bed.The
of melanin-eumelanins in black and brown cuticle is an extension of the horny layer of the
hair, and phaeomelanins in auburn and blond
Greying of hair is the result of a decrease in
tyrosinase activity in the melanocytes of the hair
bulb. The age of onset of greying is genetically
determined, but other factors may be involved
such as auto-immunity-premature greying of
the hair is a recognized association of perni-
cious anaemia.The phenomenon of'going white
overnight', usually attributed t o a severe fright,
is physically impossible. It is, however, possible
t o 'go white' over a period of a few days as a re-
sult of selective loss of remaining pigmented
hairs in someone who has extensive grey hair-
this occurs in one type of alopecia areata.

Sebaceous glands
Sebaceous glands are found everywhere on the
skin apart from the hands and feet.They are par-
ticularly numerous and prominent on the head
and neck, the chest, and the back. Sebaceous Fig.1.4 The nail.
6 Chapter I: Structure and Function ofthe Skin, Hair and Nails

proximal nail fold onto the nail plate. I t forms a mediators such as histamine, prostaglandins.
seal between the nail plate and proximal nail leul<otrienes and eosinophil and neutrophii
fold, preventing penetration of extraneous chemotactic factors. Macrophages are phago-
material. cytic cells that originate in the bone marrow,
Nail growth is continuous throughout life, and they act as scavengers of cell debris and
but is more rapid in youth than in old age.The extracellular material.
average rate of growth of fingernails is approxi- The dermis is also richly supplied with
mately I mm per week, and the time taken for a blood vessels, iymphatics, nerves and sensory
fingernail t o grow from matrix t o free edge is receptors.
about 6 months. Nails on the dominant hand Beneath the dermis, a layer of subcutaneous
grow slightly more rapidly than those on the fat separates the skin from underlying fasciaand
non-dominant hand.Toenails grow at one-third muscle.
the rateoffingernails,and taleabout 18 months
t o grow from matrixto free edge. Dermatoglyphics
Many factors affect nail growth rate. It is ln- Fingerprints, the characteristic elevated ridge
creased in psoriasis, and may be speeded up in patterns on the fingertips of humans, are unique
the presence of inflammatory change around t o each individual.The fingers and toes, and the
the nail. A severe systemic upset can produce a palms and soles, are covered with a system of
sudden slowing of nail growth, causing a trans- ridges which form patterns.The term'dermato-
verse groove in each nail plate.These grooves, glyphics' is applied t o the configuration of the
Iknown as Beau's lines, subsequently become ridges. If you look closely at your hands you will
visible as the nails grow out. Nail growth may see these tiny ridges, which are separate from
also be considerably slowed in the digits of a the skin creases. O n the tips ofthe fingers there
limb immobilized in plaster. are three basic patterns: arches, loops and
whorls (Fig. I.S).The loops are subdivided into
The dermis ulnar o r radial, depending on whether the loop
The dermis is a layer of connective tissue lying is open t o the ulnar o r radial side ofthe hand.A
beneath the epidermis, and forms the bull< of triangular intersection of these ridges is known
the skin.The dermis and epidermis interdigitate as a triradius,and these triradii are notonly pre-
via downward epidermal projections (rete sent on fingertips, but also at the base of each
ridges), and upward dermal projections (dermal finger, and usually on the proximal part of the
papillae) (Fig. I .2).The main feature of the der- palm.
mis is a nenvorkofinrerlacingfibres, mostly col- N o t only are the ridge patterns of finger-
lagen, but with some elastin.These fibres give prints useful for the identification and convic-
the dermis great strength and elasticity. The tion of those who covet their neighbours'
collagen and elastin fibres, which are protein, goods, but characteristic dermatoglyphic ab-
are embedded in a ground substance of mu- normalities frequently accompany many chro-
copolysaccharides(glycosaminoglycans). mosomal aberrations.
The main cellular elements of the dermis are
fibrobiasts, mast cells and macrophages. Fibro-
blasts synthesize the connectivetissue matrix of
the dermis, and are usually found in close prox-
imitytocollagenandelarrin fibres. Mastcellsare Skin is like wax paper that holds everythingin
specialized secretory cells present throughout without dripping. ( A r t Linkletter, A Child's
the dermis, but they are more numerous Garden ofMisinformotion, 1965)
around blood vessels and appendaner.
, - They It is obvious from the complex structure of the
contain granules whose contents include skin that it is not there simply t o hold all the
Functions of the skin 7

Fig. 1.5 Dermatoglyphics: (a)

arch; (b)loop; (c)whorl.

other bits of the body together. Some of the organ and plays an important role in host
functions of skin are as follows: defence. N o t only Langerhans' cells but also
keratinocytes prepare external antigens for
presentation t o T lymphocytes, which then
mount an immune response.
The protective effect of melanin against UV
* Prevents loss ofessentialbody fluids damage has already been mentioned.
* Protects againstentryoftoxic The skin is a vital part of the body's tem-
environmental chemicals and perature regulation system. The body core
microorganisms temperature is regulated by a temperature-
* Immunolagicalfunctions sensitive area in the hypothalamus, and this is

* Protects against damage from Wradiation
Regulates bodytemperature
* Synthesis afvitaminD
influenced by the temperature of the blood
which perfuses it. The response of the skin t o
* Important in sexual attraction and social cold is vasoconstriction and a marked reduction

interaction in blood flow, decreasing transfer of heat t o

~ . . ~~ ~ ~ . . ... the body surface.The response t o heat is vaso-
dilatation, an increase in skin blood flow and
In the absence of a stratum corneum we loss of heat t o the environment. Perspiration
would lose significant amounts of water t o the helps t o cool the body by evaporation of sweat.
environment, and rapidly become dehydrated. These thermoregulatoryfunctions are impaired
The stratum corneum, with its overlapping cells in certain skin disease-patients suffering
and intercellular lipid, blocks diffusion of water from exfoliative dermatitis (erythroderma) ra-
into the environment. If it is removed by strip- diate heat t o their environment because their
ping with tape, water loss t o the environment skin blood flow is considerably increased
increases I0-fold o r more. and they are unable t o control this by vasocon-
The stratum corneum is also quite an effec- striction. In a cold environment their central
tive barrier t o the penetration of external core temperature drops, in spite of producing
agents. However, this barrier capacity is consid- metabolic heat by shivering, and they may die of
erably reduced if the stratum corneum is hy- hypothermia.
drated, o r its lipid content is reduced by the use Vitamin D (cholecalciferol) is produced in the
of lipid solvents. The structural integrity of the skin by the action of UV light on dehydrocholes-
stratum corneum also protects against invasion terol. In those whose diets are deficient in vita-
by microorganisms. min D this extra source of the vitamin can be
The skin is an immunologically competent important.
8 Chapter I : Structure and Function of the Skin, Hair and Nails

The skin is also a huge sensory receptor, per- In addition t o all these mechanistic functions,
ceivingheat, cold, pain, light touch and pressure, the skin plays an essential aesthetic role in social
and even tickle. As you are probably still grap- interaction and sexual attraction.
pling with the conundrum of the biological sig- Hence, you can see that your skin is doing
nificance of hairy ears in the elderly male, t r y a good job. Apart from looking pleasant, i t is
switching your thoughts t o the benefits of tickly saving you from becoming a cold, UV-damaged,
armpits! brittle-boned, desiccated'prune'.
Approach to the Diagnosis
ofDevmatological Disease

Introduction, 9 Dermatological diagnosis,9 Investigation,l 2

The value o i a diagnosis, 9 Examination,11 Conclusion, 16

Baglivi hassoid, "The patient is the doctor's

best text-book':That"'text-boak"however, hos
to be intmducedtothe student and those who
effect the introductions are not always wise.
(Dannie Abse. Doctors andpotients)
-Provides a workinglabel which will be
recognizedby others
* Implies some commonalitywith other
The dermatologist's art is giving a disease a long patientswith thesamediseasestateor
Greek name . . . and then o topicalsteroid. condition:in aetiology;pathology;clinical
features;responsiveness to treatment
Offers a prognosis and informationabout
contaaon orheredity
* Gives access to treatment modalities

Dermatology is essentially a specialty where

clinical information is at the forefront of the
Dermatological diagnosis
diagnostic process, and it is important for any
aspiringclinicianto realizethat, before prescrib- That which we calla rose.
ing treatment or offering prognostic informa- by any other bame wouldsmellassweet
(Shal~espeare, Romeo ondjuliet)
tion about a patient's problem, he o r she must
Aspiring dermatologists must begin by becom-
first make a diagnosis. This chapter is about
ingfamiliarwith the diagnostic labels used in the
reaching a diagnosis in a patient with a skin
disorder. description and classification of sldn disease.
This can seem daunting, but remember that
diagnostic labels in medicine are bound by
The value of a diagnosis convention and rooted in history: the nomen-
clature of disease, and its signs and symptoms.
The facts on which a clinician makes a diagnosis has emerged from hundreds of years of classif-
cation and categorization.Therc is nothing spe-
must always come first and foremost from the
cial about dermatology, except perhaps in the
patient and there is no substitute for talking t o
degree t o which subtle clinical variations areaf-
and examining patiencs.This is especially true of
fol-ded separate names.The fact that diagnostic
skin disease.
terms often bear no relationship to modern
A diagnosis is a short statement about a dis-
thinldng is not of itself imporrant. An apple is
ease state o r condition.
still an apple, even if we don't lknow who first
called it that o r why!
10 Chapter 2: Diagnosis of Dermatological Disease

Therefore, as in any other branch of medi-

cine, the diagnostic terminology in dermatology I nmw
* Not onlysystemicmedicationbut also topical;
has t o be learned.This is not as hard as it may
many patientsapplymultiplecreamsand
at first seem. In the same way as a visitor t o
ointments: topicals maybe medicinal 1
Mars would have t o become familiar with what
things on Mars were called, the dermatological
tyro who pays attention rapidly becomes ac- Topical medication mayalsobeself-
quainted with commoner skin diseases (e.g. administeredaspartofa'cosmetic'regimen
eczema, psoriasis o r warts). In time, he o r she
will also begin t o recognize rarer disorders and
less 'classical' variants of commoner ones. There are also specific features of dermato-
However, this remains a dynamic process which logical histories t o watch out for.
involves seeing, reading, asking and learning-
always with the eyes, ears and mind open! Symptoms
Patients with skin disease talk about symptoms,
The steps t o making a especially itching, which you may not have met
dermatological diagnosis before.You will have t o learn t o assess and quan-
In principle there is nothing difficult about tify these.You will soon get used t o this. For ex-
dermatological diagnosis. The process of iden- ample, a severe itch will keep patients awake
tifying skin diseases consists of taking a history, o r stop them from concentrating at work o r
examining the patient, and performing inves- school.
tigations where necessary. In practice many
dermatologists will ask questions afler a quick Patients9language
look t o assess the problem, and also during the Be careful about terms which patients use t o
formal examination. However, we should con- describe their skin problems. In Leicestershire,
sider the elements of the process separately. where the authors work, weals are often called
A dermatological history contains most of 'blisters' and it is easy t o be misled. Always ask
the questions you will be used t o asking: onset the patient t o describe precisely what he o r she
and duration, fluctuation, nature of symptoms, means by a specific term.
past history. There are some differences, how-
ever, which are largely in the emphasis placed on Quality of life
certain aspects, shown as follows: It may be helpful t o assess the impact of the
problem on the patient's normal daily activities
and self-image: work, school, sleep, self-
confidence, personal relationships.

Past history Patient preconceptions

Shauldinclude generalproblems, such as ! Patients often have their own ideas about the


* Diabetes andTB cause of skin problems and will readily offer

Pastskinproblems j them! For example, washing powder is almost
* Significant ailergies !
universally considered t o be a major cause of
Family history
rashes, and injuries t o be triggers of skin tu-
* Some disorders are infectious: others have mours. Never ignore what you are told but take
strong genetic backgrounds care t o sieve the information in the light of your

Occupation andhobbies
The skinis frequently affectedby materials
encountered atwork and in the home
Watch out, too, for the very high expecta-
tions of many patients. They know that visible
evidence is there for all t o see: dermatology
Examination II

often truly requires a'spot'diagnosis! Everyone fundamental elements of a good dermatological

from the patient and hislher relatives t o the examination are:
local greengrocer can see the problem and ex- I Site andlor distribution ofche problem.
press their opinion. 2 Characteristics of individual lesion(+
3 Examination of 'secondary' sites.
4 'Special' techniques.
Examination Unfortunately, names and terms can appear
t o get in the way of learning in dermatology.
The next step is t o examine the patient. Wise Indeed this seems t o be one reason why many
counsels maintain that you should always exam- cliniciansclaim that dermatology is amysterious
ineapatlentfrom head t o foot. In realitythis can and impenetrable mixture of mumbo-jumbo
be hard on both patient and doctor, especially if and strange potions.There is really no need for
the problem is a solitary wart on the thumb! this attitude: the terms In use have developed
However, as a genera rule, and especially with for good reasons. They provide a degree of
inflammatory dermatoses and conditions with precision and a framework for diagnosis and
several lesions, it is Importantto havean overall decision making. Try t o familiarize yourself
look at the sites involved.You may also find the with them, and t o apply them correctly. They
unexpected, such as melanomas and other skin will provide the building-blocks with which you
cancers. will go on t o make dermatological diagnoses.
Inspect and palpaie the lesion@) o r rash (it So, in theearly days,describeeverythingyousee
may help t o use a magnifying hand lens). The in these terms as fir as possible.

1 Sic+) and/ordis~bution.Thiscanbevey rough, andtodistinguishcrust(dried

~ helpful: for example, psoriasis has a serum)from scale (hyperkeratosis);some
redilection forknees,elbows,scalp and lower assessmentofscale canbe helpfu1,e.g.
rack; eczemafavaurs the Remresinchildren; 'silvey'in psoriasis
tcneoccurs predaminantlyon the face and * The texhlre-superficial?deep?Useyour
rppermnkbasal cell carcinomas are more fingerfipsonthesurface; assess thedepth
:ommon on the head and neck and position in orbeneath the skin;lift
! Characteristics of individual lesion(s): scaleorcrusttoseewhatisundemeath;
* Thetype.Somesimplepreliminay trytomake thelesionblanchwith
readingis essential.UseTable 2.1forthe pressure
most common and important terms and 3 Secondaly sites. Look for additional
their definitions features which may assist in diagnosis.Good
* The size,shape, outlineand border. Sire examples of this include:
isbestmeosured,rathherthanbeinga * The nails in psoriasis
comparison with peas, oranges or coins of
the realm. Lesionsmay be various shapes.
e.g. round, oval.annular, linearor'irregular';
-- The fingers and wrist sin scabies
The toe-webs in fungalinfections
Themouthinlichen planus
straightedgesand anglesmaysuggest 4 'Special' techniques. These will be covered
external factors.Theborder is well defined in the appropriate chapters, but there are
inpsoriasis, but blurredinmost patches of s0metlicks.e.g.:

Thecolaurltisalwaysuseful tonotethe
colour: red,purple.brawn. slate-black.etc.
* Scrapingapsoriatic plaque for capillay
* The Nikolskysigninblisteringdiseases
* Surface features (Table 2.1).It is helpful to * 'Diascopy'in suspected cutaneous
assesswhetherthesurfaceissmoothor tuberculosis
I2 Chapter 2: Diagnosis of Dermatological Disease

Table 2.1 'Types and characteristics oflesions.

* M a d e : a flat, circumscribed area of skin discoloration
Papule: acircumscribedelwatianoftheskinlessthan0.5mindiameter
* Nodule: acircumscribedvisiblearpalpablelump,largerthan0.5cm
Plaque: acircumscribed, disc-shaped, elevated area ofskin:
'small'Qcm in diameter

- 'large' > 2 min diameter

Vesicle: a smallvisiblecollection offluid ~~essthan0.5cmindiameter)

* Bulk a largevisiblecollection offluid(over0.scm)
Pustule: avisibleaccumulationofpus
Ulcer: a loss of epidermis (often with loss ofunderlyingdermis and subcutis as well)
* Weal: a circumscribed. elevated areaofcutaneous oedema

Surface characteestic'es
Scale: visible and palpable flakes due to aggregation andlor abnormalities ofshed epidermal cells
Crust: accumulateddriedexudate,e.g.semm

* Horn: an elevatedprojectionofkeratin
Excoriation: asecondary,superficiaI ulceration.due to scratching
* Maceration: an appearance ofsurface softeningdue to constantwetting
Lichenification:aflat-toppedthickeningofthe skin often secondaryto scratching.
~ ~ ~~ ~ ~~ ~~~ ~~ ~ ~

I t is fair t o say that in inflammatory der- These situations are covered later in the appro-
matosesacomplication is havingtodecide which priate chapters.The advances in modern genet-
lesion or lesions to select for this descriptive ics, too, mean that blood (or other tissues) can
process. Skin diseases are dynamic. Some le- be analysed for evidence of specific defects.
sions in any rash will be very early, some very Sometimes clinical tindings alone will not pro-
late, and some at various intermediate evolu- duce a satisfactory working diagnosis, o r fur-
tionary stages. ther information is required in order t o plan
Try t o examine as many patients as you can: optimal management.
frequent exposure t o skin diseases develops an A number of important techniques are avail-
ability t o recognize those lesions which provide able t o provide further information. Some of
the most useful diagnostic information, these, such as appropriate blood tests and
This diagnostic process willgradually become swabs for bacteriology and virology, should be
one that you will perform increasingly easily and familiar from other branches of medicine, and
confidently as experience develops. are fully covered in other introductory text-
books. Others, however, are more specific t o
dermatological investigation. Common, useful
investigations in sldn diseases include the
Inevitably, history and examination alone will Blood tests-for underlying systemic
not always provide all the information required. abnormalities and, increasingly, for genetic
Thereare some sldn disorders in which further analysis.
investigation is nearly always necessary: either Swabs and other samples-for infections.
t o confirm a diagnosis with important prognos- Wood's light-some disorderslfeatures are
tic o r therapeutic implications (e.g. blistering easier t o see.
disorders), o r t o seek an underlying, associated Skin scrapes o r nail clippings-microscopy
systemic disorder (e.g. in generalized pruritus). and mycological culture.
Investigation 13

Skin biopsy- histopathology; electron cytodiagnosis of suspected viral blisters and

microscopy; immunopathology; D N A pheno- pemphigus, using a 'Tzanck preparation', which
typing. enables material t o be examined directly in the
Patch tests -for evidence of contact allergy. clinic.

Wood's light Skin biopsy

This is a nickel oxide-filtered ultraviolet light Skin biopsy is a very important technique in the
source, used t o highlight three features of skin diagnosis of many skin disorders. In some, it is
disease: critical t o have confirmation of a clinical diagno-
I Certain organisms which cause scalp ring- sis before embarking on treatment. Good
worm produce green fluorescence (useful in examples of this are skin cancers, bullous
initial diagnosis and helpful in assessingtherapy). disorders, and infections such as tuberculosis
2 The organism responsible for erythrasma and leprosy. In others it is necessary t o take a
fluoresces coral-pink. biopsy because clinical information alone has
3 Some pigmentary disorders are more clearly not provided all the answers.
visible-particularly the pale patches of There are two methods commonly used
tuberous sclerosis, and cafe-au-lait marks of t o obtain a skin sample for laboratory
neurofi bromatosis. examination:
Wood's light can also be used t o induce I lncisionallexcisionaI biopsy.
fluorescence in the urine in some of the 2 Punch biopsy.
porphyrias. Specimens obtained by either technique
may be sent for conventional histopathology-
Scrapings/clippings normally fixed immediately in formal-saline-
Material from the skin, hair o r nails can be ex- andlor other specialized examinations, for
amined directly under the microscope andlor example for D N A phenotyping of specific cells
sent for culture.This is particularly useful in sus- o r for viral DNA. For immunopathology the
pected fungal infection, o r in a search for scabies skin is usually snap frozen but for electron
mites (see Chapters 4 & 5). Scraping lightly at microscopy skin is best fixed in glutaraldehyde.
the epidermis will lift scales from the surface of Always check the details with the
the suspicious area. laboratory before you start.
The scales are placed on a microscope slide,
covered with 1 0% potassium hydroxide (KOH) IncisionaVexcisionaIbiopsy
and a coverslip. After a few minutes t o dissolve This provides good-sized samples (which can be
some ofthe epidermal cell membranes, they can divided for different purposes if required) and
be examined. I t is helpful t o add some Parker can be used t o remove quite large lesions (see
Quink ink if an infection with Malassezia (the Figs 2.1 & 2.2):
cause of pityriasis versicolor) is suspected. I Administer local anaesthetic. Usually
Nail clippings can also be treated this way, but 1-2% lidocaine (lignocaine); addition of
need stronger solutions of KOH, o r longer t o I : I0000 adrenaline (epinephrine) helps reduce
dissolve. bleeding, but never use on fingers and toes.
Microscopy of hair may also provide informa- 2 For incisional (diagnostic) biopsy. Make
tion about fungal infections, may reveal struc- two cuts forming an ellipse; ensure that the
tural hair shaft abnormalities in certain genetic specimen is taken across the edge of the lesion,
disorders, and can be useful in distinguishing retaining a margin of normal perilesional skin
some causes of excessive hair loss (see Chapter (Fig. 2. l a). For complete excision. Widen
13). the ellipse around the whole lesion (Fig. 2.1 b);
Scrapelsmear preparations are also used as a ensure that the excision edge is cut vertically
diagnostic aid by some dermatologists for the and does not slant in towards the tumour,
14 Chapter 2: Diagnosis of Dermatological Disease

Fig. 2.1 The technique for

incisional (a)and (b) excisional

Fig. 2.2 Equipment needed for

an incisionaVexcisional
biopsy: sterile towel; gauze
squares; cotton-wool balls;
galley pot containing
antiseptic; needle; cartridge of
lidocaine (lignocaine) and
dental syringe; scalpel; skin
hook; scissors; small artery
forceps; needle holder and
suture; fine, toothed forceps;
needle and syringe
(alternative to dental syringe).

which can result in inadequate deeper excision samples and is only appropriate for diagnostic
(Fig. 2.3). biopsies o r removing tiny lesions (see Fig.
3 Repair the defect. Edges left by either inci- 2.4a-c):
sional o r excisional biopsy are brought neatly I Administer local anaesthesia (see above).
together with sutures; the choice of suture 2 Push the punch biopsy blade into the lesion
material is not critical, but for the best cosmetic using a circular motion.
result use the finest possible, preferably a 3 Lift out the small plug, and separate with
synthetic monofilament suture (e.g. prolene). scissors o r a scalpel blade.
Note: if there will be significant tension on the 4 Achieve haemostasis with silver nitrate o r a
suture line, consider asking a trained plastic o r small suture.
dermatological surgeon for advice.
Patch tests
Punch biopsy If a contact allergic dermatitis is suspected, a
This is much quicker, but produces small patch test is performed. In this process possible
Investigation 15

allergens are usually diluted in suitable vehicles.

The test materials are placed in small discs in
contact with the skin (usually on the back) for 48
h (Fig. 2.5a). A positive reaction (at 48 h, o r oc-
casionally later) confirms a delayed hypersensi-
tivity (type IV) reaction t o the offending
substance (Fig. 2.5b).
This technique can be extended t o include
testing for photoallergy.

Fig. 2.3 Excisionalbiopsy: the correct (J)and

incorrect (X) excision edge.

Fig. 2.4 (a-c) The technique for

a punch biopsy.

Fig. 2.5 Patch testing: (a)metal

cups containing allergens; (b)
positive patch test reactions.
I6 Chapter 2: Diagnosis of Dermatological Disease

is in each instance, and make sure that you

read a little about each entity when the clinic is
You are now ready t o smrc examining and
The remaining chapters of this book are de-
talldng r o patients with skin disease. Attend
signed t o help you t o make specific diagnoses
some dermatology clinics and put these princi-
and t o provide your patients with information
ples into practice.When seeing patients, t r y t o
about their problems, and t o assist you in
rerain a mental picture of their skin lesions.Ask
choosing appropriate treatment.
the dermatologist in charge what the diagnosis
Bacterial and
Viral Infections

Bacterialinfectians, 17 Viralinfections, 22

A mighty creature is the germ fer from leg oedema of cardiac, venous o r lym-
Though smaller than the pachyderm phaticorigin.
His customary dwelling place The affected area becomes erythematous,
Is deep within the human roce hot and swollen (Fig. 3.l), and blister formation
His childish pride he often pleases and areasofskin necroris mayoccur.The patient
By giving peoplestrange diseases is pyrexial and feels unwell. Rigors may occur
Do you,my poppet, feel infirm? and, in the elderly, a toxic confusional state.
Yau probably contain agerm In presumed streptococcal cellulitis penicillin
(Ogden Nash, The Germ) is the treatment of choice, initially given as ben-
r/lpenicillin intravenously. If the leg is affected,
bed rest is an important aspect of treatment.
Bacterial infections Where there is extensive tissue necroris, surgi-
cal debridement may be necessary.
Streptococcal infection A particularly severe, deep form of cellulitis,
Cellulitis involvingfasciaand muscles, is known as'necro-
Cellulids isa bacterial infection of subcutaneous tizingfasciitis'.Thir disorder achieved notoriety
tissues which, in immunologically normal a few years ago when it attracted the attention
individuals, is usually caused by Streptococcus of the UK popular press and was described as
pyogenes.'Erysipelas' is a term applied t o super- being caused by a'flerh-eatingvirus'. I t is assocl-
ficial streptococcal cellulitis which has a well- ated with extensive tissue necroris and severe
demarcated edge. Occasionally, other bacteria toxaemia, and is rapidly fatal unless urgenttreat-
areimplicated in cellulitis-Hoemophilusinpuen- ment, including excision of the affected area, is
zoe is an important cause of facial cellulitis in undertaken.
children, often in association with ipsilateral Some patients have recurrent episodes of
otitis media. In immunocompromised individu- cellulitis, each episode damaging lymphaticsand
als, a variety of bacteria may be responsible for leading t o further oedema.These cases should
cellulitis. be treated with prophylactic oral penicillinv o r
Cellulitis frequently occurs on the legs, but erythromycin, t o prevent further episodes.
other parts of the body may be affected-the
face is a common site for erysipelas.Thc organ- Staphylococcal infection
isms may gain entry into the skin via minorabra- Folliculitis
sions, o r fissures between the toes associated Infection of the superficial part of a hair follicle
with tinea pedis, and leg ulcers provide a portal with Staphyiococcus aureus produces a small pus-
of entry in many cases. A frequent predisposing tule on an erythematous base, centred on the
factor is oedema of the legs, and cellulitis is a follicle.
common condition in the elderly,who often suf- Folliculitis is a common problem in eczema
18 Chapter 3: Bacterial and Viral Infections

treated with a topical antibacterial such as

mupirocin, applied t o the nostrils.They may also
be helped by an antibacterial bath additive, for
example 2% triclosan, and a prolonged course
of flucloxacillin.

A carbuncle is a deep infection of a group of ad-
jacent hair follicles with S.aureus. A frequent site
for a carbuncle is the nape of the neck. Initially,
the lesion is a dome-shaped area of tender ery-
thema, but after a few days suppuration begins,
and pus is discharged from multiple follicular
orifices. Carbuncles are usually encountered in
middle aged and elderly men, and are associated
with diabetes and debility.They are uncommon
nowadays. Flucloxacillin should be given for

This is a contagious superficial infection which
occurs in two clinical forms, non-bullous and
bullous. Non-bullous impetigo is caused by
Fig. 3.1 Cellulitis. reus, streptococci, o r both organisms together.
The streptococcal form predominates in warm,
humid climates, for example the southern USA.
patients treated with ointment-base topical Bullous impetigo is caused by S. aureus. Lesions
steroid preparations. may occur anywhere on the body. In the non-
Mild folliculitis can be treated with a topical bullous form the initial lesion is a small pustule
antibacterial agent, but if it is extensive a sys- which ruptures t o leave an extending area of
temic antibiotic may be required. exudation and crusting (Fig. 3.2). The crusts
eventually separate t o leave areas of erythema,
Furunculosis ('boils') which fade without scarring. In the bullous
A boil (furuncle) is the result of deep infection of form, large, superficial blisters develop. When
a hair follicle by S. aureus. A painful abscess de- these rupture there is exudation and crusting,
velops at the site of infection, and over a period and the stratum corneum peels back at the
of a few days becomes fluctuant and'points' as a edges of the lesions.
central pustule. Once the necrotic central core Streptococcal impetigo may be associated
has been discharged, the lesion gradually re- with poststreptococcal acute glomeruloneph-
solves. In some patients boils are a recurrent ritis.
problem, but this is rarely associated with a sig- Impetigo may occur as a secondary phenom-
nificant underlying disorder. Such individuals enon in atopic eczema, scabies and head louse
may be nasal o r perinea1 carriers of staphylo- infection.
cocci, and organisms are transferred on the In localized infection, treatment with a topi-
digits t o various parts of the body. cal antibiotic such as mupirocin will suffice, but
Patients sufferingfrom recurrent boils should in more extensive infection treatment with a
have swabs taken from the nose for culture, and systemic antibiotic such as flucloxacillin o r ery-
if found t o be carrying staphylococci should be thromycin is indicated.
Bacterial infections 19

Fig. 3.2 Impetigo.

Fig. 3.3 Erythrasma.

Staphylococcal scalded skin syndrome

This uncommon condition occurs as a result Corynebacterium minutissimum produces a por-
of infection with certain staphylococcal phage phyrin which fluoresces a striking coral-pink
types which produce a toxin that splits the under Wood's light.
epidermis at the level of the granular layer. Erythrasma may be treated with topical imi-
The superficial epidermis peels off in sheets, dazoles.(e.g. clotrimazole, miconazole), topical
producing an appearance resembling scalded fusidic acid, o r a 2-week course of oral
skin. Infants and young children are usually af- erythromycin.
fected. It responds t o parenteral therapy with
flucloxacillin. Mycobacterialinfection
Cutaneous tuberculosis
Erythrasma Cutaneous tuberculosis is now uncommon in
Caused by a Gram-positive organism, Europe and the USA, but may be encountered
Corynebacterium minutissimum, erythrasma oc- in immigrants from other parts of the world
curs in intertriginous areas-axillae, groins and where tuberculosis remains problematical.
submammary regions. However, the common-
est site colonized by this organism is the toe Scrofuloderma
web-spaces, where i t produces a macerated Scrofuloderma results from involvement of
scaling appearance identical t o that caused by the skin overlying a tuberculous focus, usually a
fungal infection. In other sites it produces lymph node, most commonly in the neck. The
marginated brown areas with a fine, branny sur- clinical appearance is of multiple fistulae and
face scale (Fig. 3.3). It is usually asymptomatic. dense scar tissue.
20 Chapter 3: Bacterial and Viral Infections

Fig. 3.4 Lupus vulgaris on the


Lupus vulgaris infected sputum.The clinical appearance is of a

The majority of lesions of lupus vulgaris occur warty plaque. It respondst o standard antituber-
on the head and neck.The typical appearance is culous chemotherapy.
of a reddish-brown, nodular plaque (Fig. 3.4).
When pressed with a glass slide (diascopy), the Tuberculides
brown nodules, which are referred t o as 'apple This term is applied t o skin lesions which occur
jelly' nodules, are more easily seen.The natural in response t o tuberculosis elsewhere in the
course is gradual peripheral extension, and in body.They are probably the result of haemato-
many cases this is extremely slow, over a period genous dissemination of bacilli in individuals
of years. Lupus vulgaris is a destructive process, with a moderate o r high degree of immunity.
and the cartilage of the nose and ears may be se- Included in this group are papulonecrotic
verely damaged. tuberculide, lichen scrofulosorum and ery-
Histology shows tubercles composed of epi- thema induratum (Bazin's disease).
thelioid cells and Langhans' giant cells, usually
without central caseation. Tubercle bacilli are Atypical mycobacteria
present in very small numbers. The Mantoux The commonest ofthe skin lesions produced by
test is strongly positive. The patient should be atypical mycobacteria is'swimming pool' or'fish
investigatedfor an underlying focus of tubercu- tank' granuloma.This is usually a solitary granu-
losis in other organs, but this is only found in a lomatous nodule, caused by inoculation of
small proportion of cases. Mycobaaerium marinum into the skin via an
Treatment should be with standard antitu- abrasion sustained whilst swimming, o r in
berculous chemotherapy. tropical fish fanciers whilst cleaning out the
There is a risk of the development of squa- aquarium-often after the demise of the fish
mous cell carcinoma in the scar tissue of long- contained therein. Occasionally, in addition t o
standing lupus vulgaris. the initial lesion, there are several secondary le-
sions in a linear distribution along the lines of
Warty tuberculosis lymphatics (sporotrichoid spread) (Fig. 3.5).
This occurs as a result of direct inoculation of Most cases respond t o treatment with minocy-
tubercle bacilli into the skin of someone previ- dine o r cotrimoxazole.
ously infected, who has a high degree of immu-
nity. It may develop on the buttocks and thighs Leprosy (Hansen's disease)
as a result of sitting on ground contaminated by The Norwegian Armauer Hansen discovered
Bacterial infections 21

Fig. 3.5 Fish tank granuloma

showing sporotrichoid spread.

the leprosy bacillus, Mycobaaerium leprae, in ripheral nerves are affected. Skin lesions are sin-
1873, and if the possibility of leprosy enters into gle, o r few in number, and are well defined.They
the discussion of differential diagnosis in the are macules o r plaques which are hypopig-
clinic, the eponymous title of this condition mented in dark skin.The lesionsare anaesthetic,
should always be used, because the fear of lep- sweating is absent, and hairs are reduced in
rosy is so ingrained, even in countries where it is number.Thickened branches of cutaneous sen-
not endemic. sory nerves may be palpable in the region of
Leprosy has a wide distribution throughout these lesions, and large peripheral nerves may
the world, with the majority of cases occurring also be palpable. The lepromin test is strongly
in the tropics and subtropics, but population positive. Histology shows well-defined tubercu-
movements mean that the disease may be en- loid granulomas, and bacilli are not seen on
countered anywhere. modified Ziehl-Nielsen staining.
Leprosy is a disease of peripheral nerves, but When the cell-mediated immune response is
i t also affects the skin, and sometimes other tis- poor, the bacilli multiply unchecked and the pa-
sues such as the eyes, the mucosa of the upper tient develops lepromatous leprosy. The bacilli
respiratory tract, the bones and the testes. Al- spread t o involve not only the skin, but also the
though it is infectious, the degree of infectivity is mucosaof the respiratory tract, the eyes, testes
low. The incubation period is lengthy, probably and bones. Skin lesions are multiple and nodular.
several years, and it is likely that most patients The lepromin test is negative. Histology shows a
acquire the infection in childhood. A low inci- diffuse granuloma throughout the dermis, and
dence of conjugal leprosy (leprosy acquired bacilli are present in large numbers.
from an infected spouse) suggests that adults In between these two extreme,'polar' forms
are relatively non-susceptible.The disease is ac- of leprosy is a spectrum of disease referred t o as
quired as a result of close physical contact with borderline leprosy, the clinical and histological
an infected person, the risk being much greater features of which reflect different degrees of
for contacts of lepromatous cases-the nasal cell-mediated responset o the bacilli.There is no
discharges of these individuals are the main absolute diagnostic test for leprosy-the diag-
source of infection in the community. nosis is based on clinical and histological
The clinical pattern of disease is determined features.
by the host's cell-mediated immune responset o Tuberculoid leprosy is usually treated with a
the organism. When this is well developed tu- combination of dapsone and rifampicin for 6
berculoid leprosy occurs, in which skin and pe- months; lepromatous leprosy with dapsone,
22 Chapter 3: Bacterial and Viral Infections

rifampicin and clofazimine for at least 24 W a r n are benign epidermal neoplasms caused
months.The treatment of leprosy may be com- by viruses of the human papillomavirus (HPV)
plicated by immunologically mediated'reaction- group.There are a number o f different strains o f
al states', and should besupervised bysomeone HPV which produce different clinical types of
experienced in leprosy management. warts. Warts are also lknown as 'verrucae', al-
though the term verruca in popular usage is
usually reserved for the plantarwart.

Common warts
These are raised, cauliflower-like lesions which
occur most frequently on the hands (Fig. 3.6).
They are extremely common in childhood and
early adult life.They may be scattered, grouped
o r periungual in distribution. Common warts in
children usually resolve spontaneously.
Common wartsareusually treated with wart
paints o r cryotherapy. Preparations containing
salicylic acid o r glutaraldehyde are often quite
effective, and a wart paint should certainly be
usedfor atleast 3 months beforeconsideringal-
ternative treatment.
Cryotherapywith liquid nitrogen can be used
on warts which do not respond t o wart paints.
A simple applicator of cotton wool wrapped
around the end o f an orange stick may be used.
This is dipped in the liquid nitrogenand then ap-
plied t o thewart until itand a narrow rim of sur-
Viral infections rounding skin are frozen. Alternatively, a liquid
nitrogen cryospray may be used.This is a painful
Warts procedure, and should n o t be inflicted on small
Fastingspittle isgoodfor warts. (Traditional children -however, most tiny tots will, sensibly,
English remedy) retreat under the desk protesting loudly at first
Viral infections 23

Fig. 3.7 Mosaic plantar warts.

sight ofthe nitrogen evaporating in its container.

Multiple warts usually require more than one
application, and the optimum interval between
treatments is 2-3 weeks.

Plantar warts
Plantar warts may be solitary, scattered over
the sole of the foot, o r grouped together pro-
ducing so-called 'mosaic' warts (Fig. 3.7). The
typical appearance is of a small area of thickened
skin which, when pared away, reveals numerous
small black dots produced by thrombosed
capillaries. Plantar warts are frequently painful.
They must be distinguished from calluses and
corns, which develop in areas of friction over
bony prominences. Calluses are patches of uni-
formly thickened skin, and corns have a painful
central plug of keratin which does not contain
Treatment is with wart paints o r cryotherapy,
after paring down overlying keratin.

Plane warts
These are tiny, flat-topped, flesh-coloured Fig.3.8 Planewarts.
warts which usually occur on the dorsa of the
hands and the face (Fig. 3.8). They often occur
in lines due t o inoculation of the virus into Genital warts (condylomata acuminata)
scratches and abrasions. Plane warts are ex- In recent years the importance of certain types
tremely difficult t o treat effectively, and at- of genital wart viruses in the aetiology of penile
tempts at treatment may do more harm than and cervical cancer has been recognized, and
good.They will resolve spontaneously eventual- this has modified attitudes t o what was previ-
ly, and are best left alone. ously considered a minor sexually transmitted
24 Chapter 3: Bacterial and Viral Infections

lesion between the thumbnails t o express the

central plug-this will often speed their resolu-
tion. In older children and adults molluscum
contagiosum can be treated by cryotherapy.

O r f is caused by a parapoxvirus. It is a disease of
sheep which can be transmitted t o humans.
Those usually affected are people who bottle-
feed lambs, and butchers and abattoir workers
who handle the carcasses of sheep. The typical
clinical picture is of a solitary, inflammatory
papule which rapidly develops into a nodule of
granulation tissue-usually on a finger, but oc-
casionally on the face. The diagnosis can be
confirmed by electron microscopy of smears
from the granulation tissue. O r f lesions resolve
spontaneously in 6-8 weeks, but the disease
may act as a trigger for erythema multiforme.

Hand,foot and mouth disease

This is not related t o foot and mouth disease of
sheep and cattle, but is a harmless disease
Fig. 3.9 Molluscum contagiosum. caused by Coxsackie virus infection, usually
typeA 16. Characteristic small grey vesicles with
a halo of erythema occur on the hands and feet
inconvenience. It is now more appropriate that (Fig. 3.1 O), and the buccal mucosa is studded
patients suffering from genital warts are seen with erosions resembling aphthous ulcers. The
and treated in a department of genitourinary condition resolveswithin 2 weeks, and no treat-
medicine, so that coexisting sexually transmit- ment is required.
ted disease may be detected and treated, and
sexual contacts traced and examined. Herpes simplex
Herpes simplex is caused by herpes virus hominis
Molluscum contagiosum (HSV). There are two antigenic types: type I is
The lesions of molluscum contagiosum are classically associated with the common 'cold
caused by a poxvirus. They are typically pearly, sore' on the lips and face, and type 2 with genital
pink papules with a central umbilication filled herpes. However, neither has rigid territorial
with a keratin plug (Fig. 3.9). The lesions may demarcation, and lesions anywhere may be
occur anywhere on the body, but are most com- caused by either antigenic type.
mon on the head and neck area and the trunk.
They are frequently grouped, and may be sur- Primary herpes simplex
rounded by a mild eczematous reaction. They Initial contact with type I HSV usually occurs in
may be very extensive in children with atopic early childhood, for example adults with cold
eczema. sores kissing children, and any lesions which de-
These lesions resolve spontaneously, and in velop are often so mild that they are not no-
infants and small children are best left alone t o ticed. Occasionally, however, a severe primary
do so. However, if parents of small children are herpetic gingivostomatitis occurs, with painful
anxious they can be advised t o squeeze each erosions on the buccal mucosa and lips. Primary
Viral infections 25

Fig. 3.10 Hand, foot and mouth

disease: vesicles on the hand.

cutaneous herpes simplex may also occur, and virus into a finger, painful episodes of 'herpetic
in atopic eczema this can be very extensive and whitlow' occur. The frequency of episodes of
may be life-threatening (see below). Genital herpes simplex usually gradually declines with
herpes may result from sexual transmission of advancing age.
type 2 HSV o r orogenital transmission of type I Labial herpes simplex is usually a minor cos-
HSV. metic inconvenience, and does not require
Physical contact during sport provides an- treatment. However, if episodes are frequent
other means of HSV transmission-in rugby, and troublesome, topical aciclovir may be of
herpes simplex thus acquired is known benefit. This blocks viral replication-it is not
as 'scrumpox', and in wrestling as 'herpes viricidal, and is not curative.
Followinga primary infection the virus settles Herpes simplex and
in sensory ganglia, and may be triggered t o pro- erythema multiforme
duce recurrent lesions by a variety of stimuli. In Recurrent herpes simplex can trigger erythema
immunodeficient individuals, for example those multiforme. Prophylactic oral aciclovir may be
who are immunosuppressed following organ of considerable benefit in the management of
transplantation, o r in association with human severe cases.
immunodeficiency virus (HIV) infection, herpes
simplex infection may be clinically atypical and Eczema herpeticum (Kaposi's
run a prolonged course. uaricelliform eruption)
This is a widespread herpes simplex infection
Recurrent herpes simplex which occurs in atopic eczema. The head and
Recurrent cold sores on the lips (herpes labialis) neck are frequently affected, but lesions may
are common. Itching and discomfort in the af- spread rapidly t o involve extensive areas of skin
fected area precedes, by a few hours, the erup- (Fig. 3.1 1 ). Lymphadenopathy and constitu-
tion of a group of small vesicles. The vesicle tional upset may occur. If the disease is limited in
contents subsequently become cloudy, and distribution and the patient is seen early in its
then crusting occurs, before resolution in about course, oral aciclovir therapy is appropriate.
I 0 days.The trigger for these episodes is often However, if the lesions are extensive, and the
fever, but exposure t o strong sunlight, and patient is unwell, they should be admitted t o
menstruation are also recognized precipitants. hospital and treated with intravenous aciclovir.
Occasionally, as a result of inoculation of the If the patient is using topical steroid therapy t o
26 Chapter 3: Bacterial and Viral Infections

Fig.3.11 Eczema herpeticum.

Fig. 3.12 Herpes zoster.

treat the eczema, this should be stopped until Shingles usually affects a single dermatome,
the herpes has resolved. Eczema herpeticum most commonly on the thorax or abdomen.
may recur, but in many cases subsequent The eruption may be preceded by pain in the re-
episodes tend t o be less severe. gion of the dermatome, and this occasionally
leads t o an incorrect diagnosis of internal
Herpes zoster (shingles) pathology. The lesions consist of a unilateral
Chickenpox and herpes zoster are both caused band of grouped vesicles on an erythematous
by the varicella-zoster virus. 'Shingles' is a dis- base (Fig. 3.12).The contents of the vesicles are
tortion of the Latin cingulum, meaning a girdle. intially clear, but subsequently become cloudy.
Following an attack of chickenpox the virus There may be scattered outlying vesicles on the
remains dormant in dorsal root ganglia until rest of the body, and these tend t o be more
some stimulus reactivatesit and causes shingles. numerous in the elderly. However, numerous
The middle aged and elderly are most often af- outlying vesicles (disseminated zoster) are also
fected, but it occasionally occurs in childhood. seen in immunosuppressed individuals, and
It is also more frequent in immunosuppressed their presence should prompt further investiga-
individuals. tion of the patient.After a few days the vesicles
Viral infections 27

Sacral zoster
Involvement of the sacral segments may cause
acute retention of urine in association with the

Trigeminal zoster
Herpes zoster may affect any of the divisions
of the trigeminal nerve, but the ophthalmic
division is the most frequently involved (Fig.
3.13). Ocular problems such as conjunctivitis,
keratitis andlor iridocyclitis may occur if the
nasociliary branch of the ophthalmic division is
affected (indicated by vesicles on the side of
the nose), and patients with ophthalmic zoster
should be examined by an ophthalmologist.
Involvement of the maxillary division of
the trigeminal nerve produces vesicles on the
cheek, and unilateral vesicles on the palate.

Motor zos ter

Occasionally, in addition t o skin lesions in a
sensory dermatome, motor fibres are affected,
leadingt o muscle weakness.
Fig. 3.13 Ophthalmic zoster.
Treatment of herpes zoster
dry up and form crusts, and in most cases the Many cases do not require any treatment. How-
eruption resolveswithin 2 weeks. In the elderly, ever, in severe cases, oral aciclovir, valaciclovir
shingles can produce quite severe erosive o r famciclovir are of benefit. In disseminated
changes which take considerably longer t o heal. zoster in the immunosuppressed, intravenous
Even in milder cases there is usually some resid- aciclovir can be life saving.
ual scarring. Pain relief is often difficult t o achieve in post-
The most troublesome aspect of shingles is herpetic neuralgia, and patients with severe dis-
the persistence of pain after the lesions have comfort should be referred t o a pain relief
healed (postherpetic neuralgia). This may be specialist.
severe, and is particularly distressing for the
Fungal Infections

The fungi which may cause human disease in- The dermatophyte fungi are named accord-
clude the dermatophytes (Greek, meaning'skin ing t o their genus (Micmsporum, Trichophyton
plants') and the yeast-like fungus Candida albi- and Epidermophyton) and their species (e.g. M.
cans, which are responsiblefor superficial fungal canis. T. rubrum), and they can be distinguished
infections confined t o the skin, hair and nails, from one another in culture. An experienced
and mucous membranes. Other fungi can dermatologist may be able t o suggestthata cer-
invade living tissue t o cause deep infections, tain fungus is responsible for a particular case of
which may remain localized (mycetoma) o r ringworm, but the only way to establish i t s
cause systemic d~sease(e.g. histoplasmoris). identity precisely is by culture.
The dermatophytes are a group of fungi Some fungi are confined t o humans (anthro-
which are responsible for so-called 'ringworm' pophilic), others principally affect animals
infections. The vegetative phase of dermato- (zoophilic) but occasionally infect humans.
phyte fungi consists of septate hyphae which When animal fungi cause human skin lesions
form a branching network (mycelium). Candida their presence often provoles a severe inflam-
albicans is an organism composed of round o r matory reaction (e.g. cattle ringworm). Der-
oval cells which divide by budding. Apart from matophytes grow only in keratin-the stratum
its yeast form it may produce pseudohyphae corneum of the skin, hair and nails. Infection is
consisting of numerous cells in a linear arrange- usually acquired by contact with keratin debris
ment or, in certain circumstances, true reptate carrying fungal hyphae-for example, the lady
hyphae. who developed ringworm on the buttocks as
a result of her husband's habit of cutting his
toenails with his feet resting on the lavatory
Dermatophyte infections seat.

It is very easyto become totally confused bythe Tinea pedis (athlete'sfoot)

terminology employed in fungal infection, and This is the commonest of the dermatophyte
end up not knowing yourtinea cruris from your infections, and usually presents as scaling, itchy
Trichophyton rubrum. Hence, a novice is best areas in the toe-webs, particularly between the
advised t o stickto simpleterminology.The term third and fourth and fourth and fifth toes, o r on
'ringworm', followed by'ofthefeet, ofthegroin, the soles (Fig. 4.1). I t is usually acquired from
of the scalp', etc.. is a simple way of indicating contact with infected keratin debris on the
the location ofthe infection. If you feel in more floors of swimming pools and showers Some-
classical mood you may use'tinea' (Latin, mean- times there is extensive involvement of the
ing'a gnawing worm') followed by 'pedis, cruris. soles and sides of the feet (so-called moccasin
capitis', etc. tinea pedis, because of its similarity t o the shape
Dermatophyte infections 29

Fig.4.1 Athlete's foot.

Fig.4.2 Tinea cruris.

of that soft leather shoe). The condition

may also spread onto the dorsa of the feet. scratched itchy feet and then scratched groins,
Occasionally, athlete's foot follows a pattern of o r on towels.
episodic vesiculobullous lesions on the soles,
occurring particularly during warm weather. Tinea corporis
The feet are frequently asymmetrically involved Tinea on the body typically has an inflammatory
in fungal infection, in contrast with eczema, in edge with central clearing (Fig. 4.3), but it is
which the involvement is usually symmetrical. relatively uncommon. An annular pattern of
eczema occurs more frequently, and is often
Tinea cruris mistaken for ringworm. Erythema annulare, as
This is common in men and rare in women.The its name suggests, also features annular lesions.
clinical picture is characteristic, and should be If fungal infection is suspected, scrapings should
easy t o distinguish from intertrigo, flexural be examined microscopically for hyphae. In
psoriasis and flexural seborrhoeic dermatitis. A adults, the source of the fungus is usually the
scaly, erythematous margin gradually spreads feet, whereas in children it has usually spread
down the medial aspects of the thighs (Fig. 4.2), from the scalp.
and may extend backwards t o involve the peri-
neum and buttocks.The source of the infection Tinea manuum
is nearly always the patient's feet, so they should Ringworm on the hand is usually unilateral.
be examined for evidence of athlete's foot o r O n the palm the appearance is of mild scaling
fungal nail dystrophy.The fungus is presumably erythema, whereas on the dorsum there is
transferred t o the groins on fingers which have more obvious inflammatory change, with a
30 Chapter 4: Fungal Infections

Fig. 4.3 Tinea corporis.

yellowish streaks in the nail plate (Fig. 4.5), but

gradually the whole nail becomes thickened,
discoloured and friable. The great toenails are
often the first t o be affected, and pressure from
footwear on the thickened nails may produce
considerable discomfort.
Fingernails are less commonly affected. The
changes in the nail plate are similar t o those seen
in toenails (Fig. 4.6).

Tinea capitis
Tinea capitis is principally a disease of child-
hood, and is rare in adults.This is thought t o be
related t o a change in the fatty acid constituents
of sebum around the time of puberty. Postpu-
bertal sebum contains fungistatic fatty acids.The
principal fungi responsible for scalp ringworm
vary in different parts of the world. In the UK,
most cases of scalp ringworm are the result of
M. canis infection, usually acquired from cats,
in the USA the usual causative organism is Tri-
chophyton tonsurans, and in the Indian sub-
continent the commonest cause is Trichophyton
Fig. 4.4 Tinea on the dorsum of the hand. violaceurn.
Trichophyton violaceurn is encountered in
children of Asian families in the UK. A recent
well-defined edge (Fig. 4.4). The source of the development is the occurrence of cases of scalp
fungus is almost invariablythe patient's feet. ringworm caused by T: tonsurans in the UK.
One o r more patches of partial hair loss de-
Tinea unguium velop on an otherwise normal scalp (Fig. 4.7).
Toenail fungal dystrophy is very common in The affected scalp is scaly, and the hair is usually
adults, and is invariably associated with athlete's broken off just above the surface, producing an
foot. The involvement usually starts distally as irregular stubble. In some cases there is little
Dermatophyte infections 31

Fig.4.5 Tinea of the toenails.

Kerion (Greek, meaning'honeycomb') is a term
applied t o severe inflammatory scalp ringworm,
usually provoked by the fungus of cattle ring-
worm, but occasionally by other fungi. It resem-
bles a bacterial infection, with pustules and
abscesses (Fig. 4.8), and affected children are
often given repeated courses of antibiotics on
this assumption.They may also be subjected t o
surgical incision of the areas.

Cattle ringworm
In rural areas, young farm workers often suffer
from cattle ringworm -older farmers have
usually had the disease, and develop immunity
against reinfection. The face and forearms are
the areas most frequently affected. There is a
marked inflammatory reaction t o the fungus,
producing an appearance resembling a bacterial
infection (Fig. 4.9).
Children who visit farms may pick up the
fungus from gates and fences where cattle have
Fig.4.6 Tinea of a fingernail.
left keratin debris containing the organism, and
subsequently develop a kerion.
obvious inflammation, but in others this is
prominent and there is pustule formation. Tinea incognito
Occasionally the area of scalp involved is This term is applied t o a fungal infection whose
more extensive, producing an appearance sug- appearance has been altered by inappropriate
gestive of seborrhoeic dermatitis. Microsporum treatment with topical steroid preparations.
canis fluoresces yellow-green under long wave- Topical steroids suppress the inflammatory re-
length ultraviolet light (Wood's light)-see sponse t o the fungus, and the typical scaly ery-
Chapter 2. thematous margin usually disappears, leaving an
32 Chapter 4: Fungal Infections

Fig. 4.7 Scalp ringworm.

Fig. 4.8 Kerion.

ill-defined area of patchy scaling erythema Diagnosis

studded with pustules. Skin scrapings, nail clippings and plucked hair
can be examined as described in Chapter 2. A
'Ide' reactions little experience is necessary t o distinguish fun-
Patients suffering from the florid vesicular gal mycelium (Fig. 4.10) from cell walls and in-
type of athlete's foot may develop an acute tercellular lipid, o r filamentous debris. Fungal
vesiculobullous eruption on the hands known as mycelium has the appearance of long rows of
an 'ide' reaction. The appearance is indistin- railway wagons which branch periodically. Ma-
guishable from the acute eczematous response terial may also be sent t o the mycology labora-
known as pompholyx (see Chapter 7). The tory for culture.
lesions on the hands do not contain fungus.
The reaction appears t o have an immunological Treatment
basis, but the exact pathomechanism is There are a number of topical antifungal agents
unknown. Occasionally, a more generalized available for the treatment of dermatophyte
maculopapular ide reaction accompanies a infections, including miconazole, clotrimazole,
fungal infection. econazole, sulconazole and terbinafine. These
Candidainfection 33

Although terbinafine and itraconazole are also

effective in scalp ringworm, they are at present
not licensed for use in children in the UK. For
skin and hair infections griseofulvin should be
given for a period of 4-6 weeks. In children the
dosage i s calculated according t o the child's
weight ( I Orngikg): in adults the usual daily dose
is 500 mg.
Skin infections may also be treated with
terbinafine 250mg daily for 2-4 w e e h o r
itraconazole l00mgdaily for 15-30 days.
The treatment of choice for nail infections is
oral terbinafine-250mg daily for 6 weeks in
fingernail infections and for 3 months in toenail
infections. An alternative is itraconazole pulse
therapy-200mg twice daily for 7 days; for
fingernails, repeat once after a 3-week drug-
free interval: for toenails, repeattwice with a 3-
week drug-free interval between each course.

Mycetoma (Madura foot)

In certain parts oftheworld,for example the In-
dian subcontinent, traurnatothefeetmay result
Fig.4.9 Cattle ringwormon the forearmof a in the inoculation of c e m i n soil fungi, which
fanner produce a deep-seated chronic infection with
abscessesand drainingsinuses.
can be used when small areas of skin are affect-
ed, but if a fungal infection is extensive it is
preferable to employ an oral agent such as Candida infection
griseofulvin, terbinafine o r itraconazole.Topical
agents are not effective in scalp ringworm, and Candidiasis (moniliasis; 'thrush') is a term
this should be treated with griseofulvin. applied t o infections of the skin and mucous

Fig.4.10 Fungalmycelium.
34 Chapter 4: Fungal Infections

membranes by yeast-like fungi of the genus with Candida o r staphylococci, and the develop-
Candida. The commonest, Candida albicans, is a ment of prominent creases at the angles of the
normal commensalof the human digestive tract, mouth, either as a normal consequence of age,
where itexists in balance with the bacterial flora. o r in edentulous individuals who do not wear
In its commensal role, Candida is present as dentures o r who have ill-fitting dentures. Saliva
budding yeasts. In a pathogenic role, budding is drawn into the creases by capillary action, and
and mycelial forms are usually present. It only salivary enzymes macerate the skin, producing
becomespathogenicwhen situations favourable sore, moist areas.
t o its multiplication arise.These include topical In denture wearers, modification of the den-
and systemic steroid therapy, immune suppres- tures may help. If Candida is present, the topical
sion of any aetiology (e.g. lymphoma, AIDS), application of nystatin o r an imidazole antifungal
broad-spectrum antibiotic therapy, diabetes such as miconazole will also help. If staphylo-
mellitus, and the apposition of areas of skin t o cocci are isolated, topical fucidic acid may be of
produce a warm, moist environment. benefit.
The diagnosis of candidiasis can be confirmed
by culture of swabs taken from the affected Chronicparonychia
areas. This is a chronic inflammatory process affecting
the proximal nail fold and nail matrix. It occurs
Buccal mucosal candidiasis predominantly in those whose hands are
White, curd-like plaques adhere t o the buccal repeatedly immersed in water -housewives,
mucosa. If these are scraped off, the underlying barstaff, florists, fishmongers. A more wide-
epithelium is inflamed and friable. I t may be spread use of mechanical dishwashers, rather
treated with nystatin pastilles o r oral suspen- than the human variety, may be associated with
sion, amphotericin lozenges, miconazole oral a decline in the incidence of this disorder.
gel o r itraconazole liquid. The clinical appearance is of thickening and
erythema of the proximal nail fold ('bolstering'),
Angular cheilitis (perleehe) and loss of the cuticle (Fig. 4.1 2). There is often
Angular cheilitis is an inflammatory process an associated nail dystrophy. Candida albicans
occurring at the corners of the mouth (Fig. plays a pathogenic role, but bacteria may also be
4.1 I). involved. The absence of the cuticle allows ac-
The main factors involved in its causation, ei- cess of irritant substances, such as detergents,
ther alone o r in combination, include infection t o the area beneath the proximal nail fold, and

Fig.4.11 Angular cheilitis.

Pityriasisversicqlor 35

betes mellitus are predisposingfactors. Balanitis

and vulvitis should be treated with a topical
anti-Candida preparation, and there are several
products available t o treat vaginal candidiasis.
D o n ' t f o r g e t t o t e s t t h e u r i n e for
sugar in anyone w i t h Candida balanitis o r

'Intertrigo' is a term applied t o maceration
which occurs where rwo skin surfaces are in ap-
position-groins.axillae,submammary regions.
o r beneath an abdominal fat apron. Obesity and
poor hygiene are contributory factors. Candida
superinfection is often present, and is suggested
clinically by the presence of creamy 'satellite'
pustulesatrhe marginsofthe affected areas.The
pustules are easily ruptured, leavinga collarette
of scale, and this gives a characteristic scalloped
edge t o the area of intertrigo.
Fig.4.12 Chronicparonychia Therapy with a combined topical prepara-
tion containing an anti-Candida agent; such
as miconazole, and hydrocortisone is usually
this probably contributes t o the inflammatory beneficial, but attention to hygiene is also
process. important.
This condition is quite distinct from acute
bacterial paronychia, in which there is a short
history, severe discomfort and ample produc- Pityriasis versicolor
tion of green pus. Pressure on the swollen nail
fold in chronic paronychia may produce a tiny This condition occurs predominantly in young
bead of creamy pus from underthe nail fold, but adults, and is caused byMalassezia yeasts, which
that is all. are normal skin commensals present in pilose-
Treatment consists of advice t o keep the baceour follicles. It is a common disorder in
hands as dry as possible by wearingcotton-lined temperate zones and is seen even more fre-
rubber o r PVC gloves when worldng, and quently in tropical climates. The reason why
topical anti-Candida therapy, for example with these yeasts multiply and produce skin lesions in
an imidawle. certain individuals is unknown.
On a fair skin, the lesions are light-brown
Balanitis/uulvouaginitis macules with afine surfacescale, and they occur
Small white patches orerodedareasare present predominantly on the trunk and upper arms
on the foreskin and glans o f the uncircumcised (Fig. 4.13). They are usually asymptomatic, but
penis. Predisposing factors are poor penile are a cosmetic nuisance. O n pigmenred s l h ,
hygiene, and diabetes mellitus. Candida balanitis the typical appearance is of patchy hypopigmen-
may be a recurrent problem if a sexual partner ration.The loss of pigment is thought t o be re-
has Candida vaginitis. lated t o production of azelaic acid by the yeasts
Candidavulvovaginitis presents with acreamy which inhibitstyrosinaseand thereby interferes
vaginal discharge and itchy erythema of the with melanin production. The reason for the
vulva. Pregnancy, oral contraceptives and dia- brown colour on pale skin is unknown. The
36 Chapter 4: Fungal Infections

Fig. 4.13 Pityriasis versicolor.

Fig. 4.14 Spores and hyphae of

Malassezia in pityriasis

colour variation, depending on the background few minutes during bathing. This will usually
skin colour, is the reason for the 'versicolor' in clear the organism in 2-3 weeks.Topical imida-
the name. zole antifungal creams and ketoconazole sham-
The diagnosis can be confirmed by micro- poo are also effective against Malassezia, as is
scopic examination of skin scraping in a topical terbinafine. Oral itraconazole is an alter-
mixture of 10% potassium hydroxide and native (200 mg daily for 7 days). Griseofulvin and
Parker Quink ink (Fig. 4.14), when character- oral terbinafine are ineffective.
istic clumps of round spores and short, stubby Pityriasisversicolor tends t o recur, and treat-
hyphae can be seen (an appearance which has ment may have t o be repeated. Hypopigmented
been called'spaghetti and meat balls'). areas may take a considerable time t o repig-
A simple treatment is selenium sulfide, in the ment, and their persistence should not be taken
form of a shampoo (Selsun), left on the skin for a as evidence of treatment failure.
Ectoparasite Infections

Scabies,37 Pedicuiosis.41

itself before the host response developsThere-

Scabies after, life becomes hazardous for the mites be-
cause burrows will be excoriated and mites and
There? osqueak ofpure delight from o motey eggs destroyed. In this way the host ikeeps the
little mite, mite population in check, and in most indivi-
As it tortuouslytunnebin theskin, duals sufferingfrom scabies the avenge number
Singing furrow, foiiy furrow,come ondjoin me in ofadult female mites on the skin is no more than
my burmw, a dozen.
And we'll view the epidermis fmm within
(Guy's Acorus) Clinical features
The patient complains of itching, which is char-
Aetiology acteristically worse at night. Scabies should
Scabies (Latin = the scab, mange, itch) is caused be considered in anyone presenting with this
by small, eight-legged mites (Sorcoptes scobiei). history.
and is acqu~redby close physical contact with There are two principal types of skin lesion in
another ind~vidualsuffering from the disease- scabies-burrows and the scabies 'rash'. Bur-
prolonged hand holding is probably the usual rows are found principally on the hands and
means of spread.Any agegroup may beaffected. feet-thesides ofthe fingers and toes, the web-
It iscommon in children and youngadulu,and in spaces, the wrists, and the insteps. In infants.
recent years has become frequent in theelderly. burrows are often present on the palms o f the
usually in a residential care home environment. hands and soles of the feet, and may also be
Transient contact is not sufficient for spread, present on the trunk and the head and neck.
and anyone encountering ordinary cases of sca- Burrows on the trunk are a common finding
bies in a healthcare setting should n o t be afraid in the elderly, and they may also occur on the
of acquiringthe disease. head and neck. Each burrow is several milli-
The female scabies mite burrows in the epi- metres long, usually tortuous, with a vesicle at
dermis, and lays eggs in the burrow behind her. one end adjacent t o the burrowing mite, and
Male scabies mites have but one function in life. often surrounded by mild eryrhema (Fig. 5.1).
and after the chase and the consummation they Burrows also occur on the male genitalia,
expire. Initially, the host is unaware o f the min- usually surmounting inflammatory papules.
ing activity in the epidermis, but after a period and these papules on the penis and scrotum
of 4-6 we& hypersensitivity t o the mite o r are pathognomonic of scabies. If scabies is
its products develops, and itching begins. The suspected in a male, thegenitalia should always
asymptomatic period is obviously very useful t o be examined.
the parasite because it has time t o establish The 'rash' of scabies is an eruption of tiny
38 Chapter 5: EctoparasiteInfections

Fig.5.1 qpical scabies burrow.

Fig. 5.2 The scabies 'rash'.

inflammatory papules which occur mainly must be found, and this usually requires some
around the axillae and umbilicus, and on the expertise. Look carefully, in good light, at the
thighs (Fig. 5.2).The rash is an allergic reaction hands and feet. A magnifying glass may be of
t o the mites. some help, but myopia is a distinct advantage.
In addition t o these primary skin lesions, Once a burrow, or a suspected burrow, has
there may be secondary changes such as been identified, i t should be gently scraped off
excoriations, eczematization and secondary the skin with the edge of a scalpel blade-
bacterial infection. In some parts of the world, dermatologists sometimes use a blunt scalpel
secondary infection of scabies lesions with known as a 'banana' scalpel for this task (see
nephritogenic streptococci is associated Chapter 2).The scrapings should be'placedon a
with the development of poststreptococcal microscopeslide with afew drops of 10%potas-
glomerulonephritis. sium hydroxide, covered with a coverslip, and
examined under the microscope.The presence
Diagnosis of mites, eggs, or even egg-shells, confirms the
Absolute confirmation of the diagnosis can only diagnosis (Fig. 5.3). Do not attempt t o scrape
be made by demonstrating the mites or eggs any lesions on the penis-the proximity of a
microscopically. In order t o do this, burrows scalpel t o this area leads t o understandable
Scabies 39

Fig. 5.3 Scabies mite and eggs in

potassium hydroxide

apprehension,and is in any case rarely rewarded (crotamiton 10% and hydrocortisone 0.25%)
by the demonstration of mites. can be used on residual itchy areas. I t is not
An alternative t o the scalpel routine is what necessary t o 'disinfest' clothing and soft
might be referred t o as the'winkle-picker' tech- furnishings-laundering of underwear and
nique. If the vesicle at the end of a burrow is nightclothes is all that is required.
opened with a needle, the tip of which is gently
moved around within it, the mite can often be Available treatments
removed, with a theatrical flourish, on the end Malathion 0.5%
of the needle. Aqueous preparations are preferred because
they do not irritate excoriated o r eczematized
Treatment skin. Wash off after 24 h.
Scabies is treated by eatingyoung alligators
and washing the skin with urine. (Mexican Folk 5%Permethrin cream
Medicine) Wash off after 8- 1 2 h.
It is important t o explain t o patients precisely
how t o use their treatment, and written ex- A single application of malathion o r permethrin
planatory treatment sheets are useful.All family is often effective, but a second application 7 days
members, and close physical contacts of an later is recommended.
affected individual, should be treated simul-
taneously. Topical agents should be applied Benzyl benzoate emulsion
from the neck t o the toes, and patients should Three applications in a 24-h period. O n the
be reminded not t o wash their hands after ap- evening of day I apply the emulsion from the
plying treatment. In infants, the elderly and the neck t o the toes.Allow t o dry, then apply a sec-
immunocompromised, in whom burrows can ond coat. The following morning apply a third
occur on the head and neck, it may be necessary coat, and then wash off the benzyl benzoate on
t o extend application t o these areas. Itching the evening of day 2. Treatment is then com-
does not resolve immediately following treat- plete, and this should be stressed t o the patient
ment, but will improve gradually over 2-3 because repeated use will produce an irritant
weeks as the superficial epidermis, contain- dermatitis.
ing the allergenic mites, is shed. A topical anti- Benzyl benzoate is a very effective scabicide,
pruritic such as Eurax-Hydrocortisone cream but it is irritant, and i t has been superseded by
40 Chapter 5: Ectoparasite Infections

more modern preparations in the UK. How- o r paresis. In these circumstances, when the
ever, because it is inexpensive it is still used in host does not itch o r cannot scratch, burrows
many parts of the world. are n o t destroyed and the mite population mul-
tiplies unchecked. Crusted scabies also occurs
neatment of infants more frequently in individuals with Down's
As burrows can occur on the head and neck it syndrome.
may be necessary t o extend application of topi- The crusted skin lesions can contain millions
cal agents to these areas. Malathion prepara- of mites and eggs, and these are shed into the
tions are not recommended for children under environment on flakes o f 1keratin.Anyone com-
6 months and permethrin is not recommended ing into contact with a patient suffering from
in infants younger than 2 months. crusted scabies is at risk of acquiring ordinary
Because of the availability of non-irritant scabies, and undiagnosed cases may be respon-
agents, benzyl benzoate is not recommended sible for outbreaks of scabies in hospitals and
for use in infants, but if it is employed it should residential homes.
be diluted to reduce i t s irritancy.
Clinical features
Weatment in pregnancy The hands and feet are usually encased in a
There is understandable concern about poten- thick, fissured crust and areas of crusting may
tial toxic effects on the fetus of scabicides when be present on other parts of the body, including
used in pregnancy. However, there is no defini- the head and neck.The nails are often grossly
tive evidencethatany ofthe currently employed thickened (Fig. 5.4).The changes may resemble
topical scabicides has been responsible for psoriatic scaling o r hyperkeratotic eczema.
harmful effects in pregnancy following appro- and this can lead t o the diagnosis being missed.
priate use. Hence, in the absence of evidence Burrowrare usually impossible t o identify in the
offetal toxicity, use of malathion or permethrin crusted areas, but may befound on less severely
appears t o be safe. affected parts of the body Microscopy of the
scales reveals numerous mites and eggs.

Crusted (Norwegian) scabies Treatment

The patient should be isolated, and nurses
This is an uncommon type of scabies in which responsible for the patient's care should wear
enormous numbers of mites are present in gowns and gloves. All medical staff and carers,
crusted lesions on the skin. It was called and any other individuals who have been in con-
Norwegian scabies because i t was originally tact with the patient prior t o diagnosis and
described in Norwegian lepers, but the term treatment, should be treated with a topical
'crusted' is now preferred. The mite is exactly scabicide.
the same as that causing ordinary scabies. Mites Crusted scabies is often difficult t o cure with
are present in such huge numbers because of topical agents, and usually requires several ap-
an altered host response t o their presence. plications of a scabicide. Treatment should be
Crusted scabies may develop when itching is applied t o the whole body, including the head
not perceived because o f sensory loss from and neck. Oral ivermectin (Mectizan) given as a
neurological disorders (hence its occurrence in single dose of 2001~gper kg body weight is an
lepers), in individuals who are immunosup- effective treatment, and higher cure rates have
pressed, either as a result of disease (e.g.AIDS) been obtained by givingtwo doses separated by
o r treatment (e.g. systemic steroids; organ an interval of 7 days, particularly in immuno-
transplantation), o r when patients are unable t o suppressed individuals. Some practitioners
scratch because of severe rheumatoid arthritis employ combination therapy with ivermectin
Pediculosis 41

Fig.5.4 Crustedscabiesin a
youth with Dawn's syndrome.

and a topical scabicide. lvermectin is not li-

censed for use in the treatment of scabies in
humans, but may be obtained on a named-
Head lice (Pediculus capitis)
patient basis from the manufacturer.
Herladyship said when Iwent to her house,
That she did not esteem me three skips ofa
Institutional outbreaks of scabies
The huge increase in the number of residential
Ifreely forgave what the deor creature said,
and nursing homer for the elderly in the UK in
For ladies will talk of what runs in their head.
recent years has been associated with numer-
(Theodore Hool<)
ous outbreaks of scabies in these facilities. Al-
Head lice are wingless insects which live on the
though some are related t o cases of crusted
scalp, and feed on blood. Adult head liceare 2-4
scabies, others appear to originate from resi-
mm in length. They are acquired by head-to-
dents who have a la~ge mite population, or from
head contact with another infected individual.
infected carers. Close contact between resi-
dents and carers in these homes is common-
It is still a commonly held belief that head lice
are associated with poor hygiene, perhaps sup-
carers usually hold the hands of residents t o
ported by the findings of surveys in the earlier
provide support when walldng, and this facili-
part of the twentieth century which showed
rates spread of the disease.
that head louse infection was principally a prob-
All residents, their carers and the carers'
lem of the lower classes in large industrial
families should be treated with a topical scabi-
conurbations. However, in more recent years,
cide. Residents who have very numerous bur-
the head louse has climbed the social ladder
rows, o r are suffering from crusted scabies,
and talen up rural pursuits, and is now widely
will require more intensive treatment and
distributed in all socioeconomicgroups.
preferably should be isolated until cured. If such
The adult female louse lays eggs which she
individuals are not identified there is a risk that
cements t o hair shafts (Fig. 5.5). The eggs are
they may only partially respond t o treatment
flesh coloured and are difficult t o see, but once
and therefore provide a source for a further
the louse nymph has emerged (after about 10
outbrealc lvermectin may proveto beofvalue in
days) the empty egg-case (nit) is more readily
dealing with outbreaks of scabies in residential
homes and similar communities.
42 Chapter 5: Ectoparasite Infections

Fig. 5.5 Head louse eggs

cemented to a hair shaft.

Fig. 5.7 The head louse.

Fig. 5.6 Head louse eggs and egg-cases.

shafts may be mistaken for nits, but the distinc-
tion is obvious if the material is examined
microscopically. Adult lice and nymphs will be
Clinicalfeatures found without difficulty in heavier infections
Itching is the main symptom. Nits tend t o be (Fig. 5.7). Impetigo may occur as a result of in-
more numerous in the occipital region of the oculation of staphylococci into the skin during
scalp and above the ears (Fig. 5.6). Occasionally, scratching; the term 'nitwit' is derived from the
flakes of dandruff o r keratin casts around hair substandard performance of children who had
Pediculosis 43

large head louse populations, secondary skin Clothing lice (Pediculus humanus)
sepsis and probably also anaemia, and were The louse
chronically unwell as a result. Has very little 'nous',
Its only pursuit
Tveatment Is the hirsute.
The approach t o treatment has changed in re- (I. Kenvyn Evans)
cent years, and it is now considered appropriate The clothing o r body louse is a parasite which
t o employ a strategy that includes both physical thrives in associationwith poverty and poor hy-
and chemical methods. Chemical means of con- giene. It lives on, and lays its eggs in the seams of,
trol, employing insecticides, have been widely clothing, and only moves onto the body t o feed
used throughout the world. Insecticides are on blood. It is still common in the poorer coun-
easy t o use and convenient, and they have tries of the world, but in an affluent society its
proved very effective. However, there is con- usual hosts are tramps and down-and-outs who
cern about potential adverse effects, particu- have only one set of clothes which are never re-
larly from residual insecticides such as lindane moved o r cleaned. An individual who regularly
(which is no longer used in some parts of changes clothing and maintains a reasonable
the world, including the UK), not only on standard of hygiene will never harbour clothing
humans but also on the environment. In addi- lice because the lice will not survive laundering
tion, there is evidence of widespread resis- and ironing of garments. Clothing lice are vec-
tance of head lice t o malathion and pyrethroid tors of epidemic typhus, a rickettsia1 disease
insecticides. which has been responsible for millions of
deaths over the centuries.


- Malathion.
Clothing lice usually provoke itching, and their
host is often covered in excoriations.

* Carbaryl.
The itching appears t o be the result of an
acquired hypersensitivity t o louse salivary anti-
gens. If clothing louse infection is suspected
there is no point in searching the patient for
lice-you may be lucky and find an occasional
louse at lunch on the body, but it is the clothing
Preparations with an aqueous basis are you should examine (Fig. 5.8).
preferable t o alcoholic solutions because they
are less likely t o irritate an excoriated scalp, Tveatrnent
do not irritate the bronchi of asthmatics, and All the patient requires is a bath. A complete
are not flammable. None of these insecticides change of clean clothing should be supplied
is fully ovicidal, and treatment should there- and the infested clothing either destroyed o r
fore be repeated after 7-1 0 days in order t o laundered at temperatures of 60C o r above.
kill any louse nymphs emerging from surviving D r y cleaning o r use of a tumble drier are
eggs. alternatives.
A simple physical method of treatment in-
volves washing the hair with an ordinary sham- Crab lice (Pthirus pubis)
poo followed by the application of generous It's no good standing on the seat
quantities of conditioner. The hair is then The crabs in here canjump I 0 feet.
combed with a fine-toothed comb with closely lfyou think that's rather high,
set teeth, which removes any lice.This process Go next door, the buggers fly!
is repeated every 4 days for 2 weeks. (Toilet graffito)
44 Chapter 5: Ectoparasite Infections

Fig. 5.8 Clothing lice.

Fig. 5.9 The crab louse.

Crab lice, also known as pubic lice and love bugs, crab's pincers (Fig. 5.9),with which itgrasps hair.
and in France as papillonsd'amour, are usually Female crab lice, like head lice, stick their eggs
transmitted during close physical contact with t o hair shafts with a cement material.
an infected individual, in spite of the above alle-
gation of contagion from hinged lavatory com- Clinicalfeatures
ponents. A t one time they were thought t o be Itching, usually nocturnal, is the symptom which
rather sedentary lice, but experiments subse- draws the host's attention t o these little passen-
quently demonstrated that when its host is gers. Self-examination then reveals the reason
sleeping the crab louse becomes quite active. It for the itch, and the doctor is often presented
is a louse adapted t o living in hair of a particular with a folded piece of paper containing 'speci-
density. It cannot colonize scalp hair, except at mens'. When folded paper is opened it has a
the margins of the scalp, but pubic, axillary, tendency t o flick its contents in all directions,
beard and eyelash hair are perfectly acceptable leaving the unlucky recipient anxiously awaiting
t o it, and in an extremely hairy male most of the signs of personal contamination for weeks
body may resemble a crab louse adventure play- thereafter.
ground.Thecrab louse is so named because of Lice are usually visible on the affected areas,
its squat shape and powerful claws, resemblinga but sometimes their eggs, which are a brown
Papular urticaria 45

Fig. 5.10 Crablause eggson t h e


colour, are easiertosee. Ifthe parasites arevery

numerous the underclothes may be speckled
with spots of altered blood excreted by the lice.
Lice on the eyelids festoon the lashes with their
eggs (Fig. 5.10). Children may acquire crab lice
as a result of normal close physical contact with
an infected parent.The lice will colonize the eye-
lashes and scalp margin. As an isolated finding,
crablouseinfection inachildshould not becon-
sidered indicative of sexual abuse.

Malathion and carbaryl are effectiveagainstcrab
lice. Aqueous preparations should be used be-
cause alcohol basis preparations will irritate the
scrotum. The whole body should be treated.
including the scalp if there is evidence of lice on
the scalp margins. Sexual contacts should also
be treated.The treatment should be repeated
after an interval of7-IOdays.
Eyelash infection may be treated by the appli-
cation of white soft paraftin (Vaseline) three
times a day for 2-3 weelcThis acts by blocking
the louse respiratory system, thereby ruffo- Fig.5.11 Papularurticaria.
cating the insects.

flies, mosquitoes, mites, fleas and bed bugs.The

Papular urticaria lesions are small urticated papules (Fig. 5.1 l),
usually grouped (sometimes in groups of three,
Often referred to as 'heat bumps' by patients, fancifully labelled'brealdast, lunch and dinner'),
papular urticaria is a typical response t o the and they may be surmounted by a tiny vesicle.
bites ofanumber ofarthropodr, including biting They are so itchy that their tops are rapidly ex-
46 Chapter 5: Ectoparasite Infections

coriated.They develop as a result of a hypersen- numbers are small in comparison with the fleas
sitivity response t o antigens in the arthropods' in various stages of development scattered
saliva. Eventually, in many people, immunologi- throughout the household. Flea eggs are not
cal tolerance t o the antigens develops and they sticky, and when laid by fleas feeding on an ani-
subsequently do not react t o the bites. mal they drop out ofthe coat into the surround-
ings -the cat-basket, the carpet, the sofa o r the
Fleas counterpane. Eggs, larvae, pupae and adult fleas
May the fleas of a thousand camels infest your are present in all these areas. Hence, the house
armpits! (Arab curse) should be treated, as well as the pets. A prepa-
The commonest cause of papular urticaria ac- ration designed t o be sprayed around the house
quired in the home environment is flea bites. It on carpets and soft furnishings is a combination
is not the human flea, Pulex irritans, which is of the insecticide permethrin with methoprene,
responsible, but fleas whose natural hosts are a synthetic equivalent of an insect growth regu-
household pets. A familiar clinical picture is of latory hormone, in an aerosol can (Acclaim
multiple lesions, some of which may form blis- 2000).The permethrin kills adult fleas, and the
ters, around the ankles of women (Fig. 5.12). methoprene blocks the metamorphosis of flea
Men are rarely affected, because socks and larvae into adults. I t should be sprayed on car-
trousers deny fleas access t o the ankles. pets and soft furnishings, and the animals' sleep-
Cats and dogs are perambulating quadripedal ing areas, and will confer protection against flea
'meals-on-wheels' for the fleas. However, al- infestation for4 months. Another useful antiflea
though fleas are present on the animals their agent is lufenuron (Program), which is given
orally t o the animal, is ingested by feeding fleas,
and interferes with the production of chitin by
flea larvae, thereby preventing their further
Occasionally, bird fleas will gain access t o
homes from nests under the eaves, and may be
responsible for more extensive lesions of papu-
lar urticaria.

Bed bugs (Cimex leetularius)

The butterfly has wings ofgold,
The firefly wings of flame,
The bed bug has no wings at all,
But he gets there just the same.
Perhapsthis rhyme relates t o the, probably inac-
curate, tale that if one attempts t o stop bed bugs
crawling up bed legs at night by placingthe legs in
bowls ofwater, the cunning bugs climb the walls,
cross the ceiling, and drop on the occupants of
the bed from above.
Bed bugs are not the most appealing of crea-
tures. They live in dilapidated housing behind
peeling wallpaper and rotten skirting boards,
and emerge an hour o r so before dawn t o feed
on the sleeping occupants of bedrooms. They
feed on blood, and although the process offeed-
Fig. 5.12 Flea bites on the ankles. ing does not cause the host any pain, a reaction
Papular urticaria 47

t o the bites of the bugs usually results in papular combings from the animal's coat and demon-
urticaria o r bullous lesions. These insects are strating the mite microscopically. Once the
5-6mm long, dark brown in colour, and can animal has been treated by a veterinary
move quite rapidly. Fortunately, bed-bug infes- practitioner the human skin lesions resolve
tation of houses in developed countries is now spontaneously.
uncommon, but if i t is suspected the local Bird mites may gain access t o houses from
environmental health department should be nests under the eaves, and can cause itchy papu-
asked t o inspect and disinfest the property. lar lesions.

Animal mites Ticks

Human contact with animals suffering from sar- Ixodid, o r hard ticks, are very common, particu-
coptic mange may result in the development of larly in wooded areas where there are deer
scattered itchy papules, often on areas coming populations. They feed on blood, and their
into contact with the animals -for example, the barbed mouthparts are held in the skin of the
abdomen and thighs if a mangy dog sits on its host during feeding by a protein cement ma-
owner's lap. It is highly unlikely that these animal terial. If a tick is pulled off the skin abruptly, its
mites will establish themselves on humans, be- mouthparts may be left in situ, and will provoke
cause they are species specific, but there have a foreign-body reaction.
been a few reports in which i t is said t o have lxodid ticks are vectors of Lyme disease
occurred. which is cause'd by the spirochaete Borrelia
Dogs, cats and rabbits are the natural hosts of burgdorferi. Lyme disease (named after the town
Cheyletiella mites, and these may cause skin le- in Connecticut where its association with ticks
sions in humans. Dogs are the usual culprits. O n was first discovered) affects the skin, joints, cen-
the animal, the mites provoke a heavy scurf over tral nervous system and the heart. It responds
the back (known in veterinary circles as'walking t o treatment with benzylpenicillin, amoxicillin
dandruff'), but hardly bother it otherwise. O n (amoxycillin) o r tetracyclines.
the owner, itchy papules appear principally Probably the best way t o remove a tick is t o
on the abdomen, but occasionally also on the grasp it as close t o the skin as possible with fine
thighs and arms-sites of contact with the ani- tweezers o r forceps, and exert gentle continu-
mal. The diagnosis can be confirmed by taking ous traction.
Acne, Acneiform Eruptions
and Rosacea

Introductian,48 Clinicai features,49 Secondaly acne, 54

Acne vulgaris andits variants, Pathagenesis ofacne, 5 1 Hidradenitis suppurativa.55
48 Treatment of acne. 53 Acneifarm disorders, 55
The psychologicalimpacto f Managementof a patientwith
acnc,49 acne. 54

Out,damnedspot! Out1say! (Shakespeare,

Macbeth v.i)
Acne vulgaris and i t s variants

Acne vulgaris
Introduction About 80% of people develop some spottiness.
Acne may be very mild indeed but at its most
This chapter deals with disorders which cause severe, gross and unsightly changes are seen.
papules and pustules, often lknown in the ver- Acne may be associated with underlying en-
nacular as 'spots' o r 'zirs'. Some are aetiologi- docrinological abnormalities (see below) but
caliy related and can properly be called variants usually it is n o t
of acne (a corruption of the Greek okme-a
point). Others produce lesions closely o r su-
Age of onset and course
perficially resembling'true' acne: the acneiform The first problems are usually encountered in
disorders and rosacea. A summary of the'acne adolescence, although there are exceptions
(see below).
family'isgiven below.
Lesions of acne vary considerably with time.
Most patients notice marked fluctuations in the
number and severity of spots, and in girls this is
often related t o the menstrual cycle.The con-
Acnedgaris dition frequently deteriorates attimes of stress.

- 'Classical'
Infantile andjuvenile onset
* Lateonset
Acne usually gets worse for a while before
gradually settlingafter2-3 years, and disappear-

ing altogether in the majority. The peak of
* Severe (acneconglobata:nodulocystic)
With systemic symptoms (acnefulminans) severity is earlier in girls than boys. In some
individuals the time-course may be much more
Semndqacne prolonged, with lesions continuing t o develop

* Endocrine associated
well into adult life.
There are two groups, described below, in
whom true acne develops outside adolescence.
* Chloracne

i Infantileljuvenile acne
Typical acne is occasionally seen in infants and
Clinicalfeatures 49

children (especially boys), usually at 3-12

months of age. Although lesions subside after
4-5 years, adolescence often heralds a severe
recrudescence. Endocrine abnormalities are
very rare, but should be considered, especially
in agirl with signs ofvirilism.

Late-onset acne
Some women develop acne in their twenties
and beyond, often with marl<ed premenstrual
exacerbations. Endocrinological investigation is
generally unrewarding, but some have polycys-
ticovary syndrome (see p. 54).

The psychological impact of acne

Acne can make life miserable and its predil-

ection for the teens and twenties means that its
effectsare on thoseleastwell equipped t o cope.
The face is prominently involved, and in ado-
lescence the face assumes increasing impor-
tance as self-imaee develo~s.Atthe time when
acne strikes, major relationships outside the Fig.6.1 This girl's [ace shows thetypicalgreasy
family and close circles of same-sex friends are skinoftheacnesufferer,in addition topapules
increasingly crucial. Realize, too, that the psy- andpustules
chological impact of acne is not necessarily re-
lated t o the degree of severity as perceived by
an 0utsider.A young person may spend just as
In severeacne, lesions may extend down the
long staring miserably into the mirror when
arms, across the whole of the central back, and
there are only a few spots as when there are
onto the buttocks.
The appearance of the skin
Thefirst physicalsign t o note is thatthe face and
Clinical features
upper trunl< become very greasy (Fig. 6.1) due
t o increased production of sebum.This is nor-
Physical signs
mal at puberty, but is excessive in those with
The characteristic distribution is as follows
acne. Scalp hair is often very greasy too. Greasi-
ness alone may be bad enough for the patient t o

- Face,anypmofwhichmaybeinvolved
* Neck,esp&allypostenorly
The individual lesions of acne

- Upper back
AnteriarcheJt,inaninvertedv'fmm the
Acardinaifeature is thatthereareseverai differ-
enttypes of lesion at any one time.

* Shoulders
* Ears
50 Chapter 6: Acne, Acneiform Eruptions and Rosacea

PapuIes and pustules

The majority of patients with acne develop
papules and pustules.They are the well-known
* Comedones: little red spots o r pustules on a red base.They
closed (whiteheads)

- open (WaCkheads)
may itch o r be quite painful. Papules develop
rapidly, often over a few hours, and frequently
become pustular as they evolve. They resolve

* Nodules
* Scars
over the course of a few days. N e w lesions may
arise in exactly the same site on many occasions.

Nodules and cysts

W i t h increasing severity, and as the inflamma-
tion extends deeper, the size of visible and pal-
Comedones (singular: comedo) pable lesions increases, resulting in deep-seated
The presence of comedones is an important nodules and cysts (Figs 6.3 & 6.4). Many patients
diagnostic aid. There are two types: closed develop a few, but some have large numbers: a
(or 'whitehead') and open (or 'blackhead'). situation in which the term 'acne conglobata' is
Closed comedones are more easily felt than used.
seen.They are very small papules, with a central Such lesions are often extremely uncomfort-
point o r elevation (Fig. 6.2).They are often most able and last much longer than more superficial
numerous on the forehead and cheeks.There is changes. Some become chronic, and may result
little o r no inflammation. in permanent cyst formation (see Chapter 9).
Open comedones (blackheads) are dilated,
blocked hair follicles, but it is not clear what Scars
causes the characteristic black dots. Burnt-out The final common pathway for the inflamma-
inflammatory lesions may leave multiheaded tory process of acne is scarring, which will re-
blackheads, particularly on the shoulders and main as a lifetime's legacy of adolescent an-
upper trunk. Blackheads are virtually pathogno- guish. Characteristically, small, deep 'ice-pick'
monic of acne in the younger patient (although scars occur, but more severe disease can leave
advanced solar damage may also result in black- gross changes, with atrophy (Fig. 6.5) o r keloid
head formation). formation (see Chapter 9).

Fig. 6.2 Closed comedones.

Pathogenesis of acne 51

Systemic symptoms
Pathogenesisof acne
(acne fulminans)
Very occasionally a young man (almost always)
The aetio-pathology of acne remains t o be elu-
develops severe nodulocystic acne accompan-
cidated fully. However, several key features may
ied by fever, malaise and joint pain and swelling.
contribute t o the final picture (see Fig. 6.6), al-
This is known as'acnefulminans'.
though this does not fully explain every aspect
of the disorder: the occurrence of prepubertal
acne, for example.
As the inflammation subsides, a variable
amount of fibrosis occurs. This may produce
scarring, particularly if repeated episodes occur
in the same site. Sometimes epithelia1remnants
become walled off by fibrosis, producingcysu.

1 Androgens (usuallyin normal amounts)

stimulate increased sebum production
2 Hairfoilicleswithparficularlylarge
sebaceousglands (ontheface,neck,chestand
back)becomeblocked by hyperkeratosis
3 Thisresultsintheclosed comedo
4 WLthin the obligate anaerobe
(Propionibncteriurn nmes) proliferates
5 This organism actson sebum.releasing
6 Theseleakinto the surroundingdeds
7 Thebodymounts anintenseacute
inflammataryresponse.Theresultof this is
Fig.6.3 Acneconglobata.

Fig. 6.4 Severe acne on the

52 Chapter 6: Acne, Acneiform Eruptions and Rosacea

Fig. 6.5 Atrophic scarring in


Fig. 6.6 The pathogenesis of acne.

Treatment of acne 53

Systemic therapies
Treatment of acne
Antibiotics are the mainstay of the treatment of
papulopustular acne. It is not known precisely
Topical therapies
how they w o r k but they reduce bacterial
Many over-the-counter remedies ~ a l yon sulfur
counts, at least initially, and may also have direct
and other astringents which mal<ethe skn flalcy
anti-inflammatory effects.
and unblock hairfollicles.
The most effective are the tetracyclines and
Topical antiseptics such as povidone iodine
erythromycin.To work, antibiotics must be fat
and chlorhexidine are often PI-escribed,but are
soluble, and the penicillins are therefore useless.
of little proven value.
Mosttetncyclinesshould betaken on an empty
Benzoyl peroxide is widely used. It reduces
stomach. Tetracyclines are contraindicated in
comedones (it is 'comedolytic') but must be
the under twelves, and in pregnant o r lactating
used regularly and in the long term.There are
Cyproterone acetate is an antiandrogen
applied once daily, and gradually progress to
which can only be given t o women. I t is given
higher concentrations.
with oestrogen t o prevent menorrhagia and t o
Vitamin A derivatives (retinoids) and
ensure contraceptive cover (it will feminize a
retinoid-lilce agents also have comedolytic
male fetus). Its effect is rather slow.
activity. Preparations in this category include
13-cis-retinoic acid (isotretinoin) is a highly
retinoic acid, isotretinoin (13-cis-retinoic
effective oral vitamin A derivative which dra-
acid) and adapalene. All work well but can be
matically reduces sebum production. It has
several side-effects: dry lips,eyesand skin, nose-
Topical tetracyclines, erythromycin and clin-
bleeds. mild alopecia. aches and pains. It also
damycin are available, and are generally applied
raises blood fat levels and may affect liver
once daily.All have been shown t o be useful in
function tests. The most serious problem is
milder acne.There are some preparations that
teratogenicity. Female patients must not be-
combine antibiotics with other agents such as
come pregnant when taking isotretinoin, as it
benzoyl peroxide.
will produce fetal abnormalities. lsotretinoin is
only available on hospital prescription in the
UK. Over 90% of patients have complete clear-
ance of their acne and in up t o 80% there is no
Topical Steroids can be used intralesionally o r
Benzoylperoxide systemically in severe acne (they are virtually
Retinoidsand retinoid-likeagents always needed in acnefulminans).
* Sulfurandarningents
Topical antiseptics.antibiotiicsand Surgical intervention
Simple measures, such as removing multiple
S p a n i C comedones with a comedone extractor, may

* Antibiotics
* 13-cis-retinoicacid (isouetinoin)
improve the appearance. It certainly gives great
pleasure and satisfaction t o a girl- o r boyfriend
who likes t o pop out blackheads! Large. residual
Steroids cysts may need t o be excised, but there is a risk
of keloid scarring. Plastic surgeons can some-
times help acne scarring by dermabrasion, but
this must not be attempted until the acne is fully
under controL
54 Chapter 6: Acne. Acneiform Eruptions and Rosacea

o r without antibiotics, allowing at least 6

Management of a patient
months fora response.
with acne
Many girls and most young men with severe
or persistent acne eventually require 13-cis-
The approach t o treatment must be tailored to
retinoic acid, usually for 4 4 months.The daily
the individual, but there are some helpful guide-
dose may begin at 0.5 mgkg but may need t o be
lines. Let's first dispel some myths!
raised t o I m d e .
lntralesional steroids are useful for acute
inflammatory lesions. Very rarely, systemic
steroid therapy may be required, especially in
* Acneisduetofattyfoodormts acnefulminans.
* Acneisduetohingdii Surgical imervention may be required later t o

* Acneisdue to'hormonalimbalance'
help overcome the devastation wreal<edby this
degree of acne.


- Dietplays nomle at alkthereisnoneed to

avoidweets.chocolateorchips MM
* Even hourlywashingwouldmakeno Onlycomedonesrmdloroniyafew
difference papulopusrular lesions resuicted to the face

- Hormones arenormalinthevastmajority
anvdifference ithank&ness!l
Morepapulopusrularlesionson the faceor
werawiderarea rmdlw occsaionalnodules

Assessment of the patient Vezywidvad papulopuetularlesions
I t can be useful t o consider acne in three broad andlorncdulocyrticlesions rmdlorsptemic
severity bands: mild, moderate and severe. BYmPtOma
Mild ocne may respond r o topical treatment oracneofmoderateseverity,failjngtosetUe
alone. Begin with benzoyl peroxide, retinoic within 6 months of therapy
or acne ofwhateverseverityeth significant
acid, isotretlnoin or adapalene, andtor a topical
antibiotic.An antibioticlbenzoyl peroxide com-
bination can be a useful option.
Moderateacne should initially be treated with
a combination of a topical agent and oral oxyte- Secondary acne
tracycline o r erythromycin in a dose of 500mg
twice daily. Continue forat least 3 4 months be- Acne lesions may arise as a consequence of
fore reassessing. Alternative tetracyclines have other primary pathological processes. Such
their advocates: some may be better absorbed 'secondary' acne is often monomorphic and
o r tolerated, but most are more expensive and generally mild.
there is generally no indication for their use as An exception is acne occurring in patients
first-line agents. with endocrine abnormo1ities.The commonest is
If the response is not satisfactory, the acne polycystic ovary syndrome, in which acneofany
should be managed as outlined below. severity may accompany hirsutism, menstrual
Severeocne may becontrolledt o someextent irregularities and infertility.Any cause ofabnor-
by systemic antibiotics, but this degree of acne mally high circulating androgen levels (such as
often demands more aggressive treatment. tumours) may also cause quite severe acne.
Girls may respond t o cyproterone acetate with while lesions in Gushing's syndromeare milder.
Acneiform disorders 55

Fig.6.7 Hidradenitis suppurativa.

Fig. 6.8 Keratosis pilaris on the upper arm.

Medicaments such as greasy ointments, po-

mades and topical steroids may induce com-
edones and occasional papules, particularly on sepsis in the apocrine glands of the axillae
the forehead and cheeks. Several drugs induce and groins (Fig. 6.7). Lesions may appear on
acneiform lesions o r make pre-existing acne the breasts (which are modified apocrine
worse, for example systemic steroids, pheny- glands).
toin, isoniazid and lithium. Recurrent painful abscesses and sinus tracks
Oil-induced acne occurs when mineral oils develop. Many patients with hidradeniris have
come into close contact with the skin.This is concurrent severe acne, o r have suffered from
often at unusual sites, such as the lower ab- acne in the past.
domen and thighs. Some patients improve on long-term anti-
Chiaracne is a specific change in which com- biotics, but many require plastic surgery.
edones appear after exposure t o chlorinated
chemical compounds. A famous example was
the release of dioxin from the explosion at Acneiform disorders
Seveso in Italy. Systemic upsets also occur.
Several conditions mimic acne but close exam-
ination will reveal important differences.
Hidradenitis suppurativa Pseudofoiriculitis barboe (shaving rash): pro-
duces small papules in the beard area and is
Although uncommon, this distinctive disorder commoner in those with naturally curly hair.
results in very unpleasant chronic, relapsing especially Afro-Caribbeans. Occasionally small
56 Chapter 6: Acne, Acneiform Eruptions and Rosacea

Fig. 6.9 vpical rosacea.

keloids develop. The process may involve the Fig. 6.10 Rhinophyma.
nape of the neck, when it is usually termed acne
keloidalis.Treatment is unsatisfactory.
Acne excoriee (desjeunes filles) is typically seen
in teenage girls who present with facial excoria- Rosacea is an important differential diagnosis
tions but very few primary lesions.There are no of acne, and is sometimes called 'acne rosacea'.
comedones.This is not true acne but a form of I t most frequently affects middle-aged women,
neurotic excoriation (see Chapter 20),and pa- but it may occur in men, and can occur at any
tients need t o be given a clear explanation.Tran- age. The sites of predilection are the central
quillizers may help. cheeks, forehead and glabellar region, end of
Pityrosporurn folliculitis causes follicular the nose and chin (Fig. 6.9). The eruption
papules and pustules on the trunk, without consists of small papules and pustules arising
other features of acne.The condition responds in crops on an erythematous, telangiectatic
t o antifungal agents such as miconazole. background.
In keratosis pilaris small spiky projections de- There are no comedones. Patientsfrequently
velop at the mouth of hair follicles, especially on complain that their face flushes easily with heat
the upper, outer arms and shoulders (Fig. 6.8). o r alcohol, and migraines are more common. In
Lesions may appear on the face, especially in men, severe involvement of the nose leads t o
children, and are occasionally pustular. A family marked sebaceous hyperplasia known as rhino-
history is common.Topical retinoic acid may be phyma (Fig. 6.10).
helpful. The treatment of choice is tetracyclines,
Acneiform disorders 57

given for several weeks in similar doses t o those Perioral dermatitis (note for strict classical
for moderate acne (see above). Topical metro- scholars: it should really be 'circum-oral') pro-
nidazole may help. It may be possible t o tail off duces a clinical appearance somewhat reminis-
the treatment, but the condition often recurs. cent of rosacea (see Fig. 22.2), and is often
Topical steroids make matters worse. Rhino- associated with topical steroid abuse. (For
phyma is best dealt with by plastic surgeons. more details see Chapter 22.)

Clinical features. 58 Exogenouseczema, 59 Endogenous eczerna,63


To keep three or four spats ofeczemo in o sionally, mostofthe body surface is affected, and
private port ofmy body and now and then to eczema is one cause of generalized exfoliative
scald or bathe them wikh hot water behind dermatitis.
closed doors. Ah,is this not happiness? Other changes in the skin which may
(Tim Shangt'an) accompany eczema include scratch marks
The terms eczema (Greek, meaning 'to boil and secondary bacterial infection. Prolonged
over') and dermatitis are synonymous.'Atopic scratching and rubbing the sldn tends t o polish
eczema' is therefore the same as 'atopic fingernails, and patients with chronic eczema
dermatitis' and 'seborrhoeic eczema' and often have nails which lookas if they havea coat
'seborrhoeic dermatitis'are the same. Eczema/ ofclear nail varnish.
dermatitis is a type of inflammatory reaction
pattern in the sldn which may be provoked by a
number of externai or internal factors. Classification

W e still have a great deal t o learn about the

Clinical features aetiology of certain types of eczema, so any
attempt at classification is based upon our pre-
The principal symptom of eczema is itching. sent state of ign0rance.A commonly employed
The clinical signs depend on i t s aetiology, site system of classification divides cases of eczema
and duration, but usually comprise erythema, into'exogenous', where an externai agent is re-
oedema, papules, vesicles and exudation (Fig. sponsible, and 'endogenous', where the pmb-
7.1). An acute eczema will have all these lem is principally constitutional. There are,
features, and might also have a bullous com- however, frequent cases in which more than
ponent. In a chronic eczema oedema is not one factor may be operating-for example, the
a prominent feature, but the epidermis be- hairdresser with hand dermatitis who suffers
comes thicl<enedand the skin surface markings from atopic dermatitis and also has superim-
are exaggerated (lichenification) (Fig. 7.2). A posed irritant dermatitis from contact with
common feature of eczema of the hands o r feet shampoos. D o notbe t o o rigid in yourattempts
is the formation of painful fissures in the skin t o classifyaparticular dermatitis-it may not fit
overlyingjoints. a recognized category, and it might be prefer-
A phenomenon which is seen with an acute able t o use a more general term such as'prob-
dermatitis, particularly allergiccontactdermati- ably endogenous'. The following classification
tis, is secondary spread o f the eczema t o sites includes most of the types of eczema you are
distant from the originally affected area. Occa- likely to encounter.

Exogenous eczema 59

Fig. 7.1 Ty@caleczema.

* ~malyirritantmntactdennatitir
* Allergiccontactdermatilis

a AtopicMema

] - Discoid eczema

j fiteatati'cenema (enemamquele)

Exogenous eczema

Primary irritant dermatitis

Primary irritants physically damage the skin;
they include acids, alkalis, detergents and petro-
leum products. Some strong irritants will pro-
duce an immediate effect, whereas with weaker
irritants the effects are cumulative.Anyone suf-
fering from atopic dermatitis is more suscep- Lichenifiedeczema.
tible t o the effects of primary irritanrs. The
housewife with a'couch-potato' husband, eight
children and no washing machine o r dish- nanny and a kitchen full of modern appliances,
washer is agood candidate for a cumulative pri- is hardly likely t o inconvenience her epidermis
mary irritant dermatitis, because her hands will t o the same degree.The typical appearance of
be perpetually immersed in washing-up liquid, housewives' hand dermatitis is dryness of the
dirty nappies and soap-powder. However, the palms and fingertips, often with painful fissures
wife of a merchant banker with 2.2 children, a in the skin creases and on the finger pulps.
60 Chapter 7: Eczema

Fig. 7.3 Severe dermatitis of the

hands in a hairdresser.

Occupational irritant dermatitis is common. t o an external allergen.There are innumerable

Duringthe early part oftheir training, hairdress- chemicals which can act as allergens, but most
ing apprentices in the UK spend a substantial rarely cause problems. Some chemicals are such
part of the day with their hands immersed in potent allergens that a single exposure will
shampoo on their clients' heads, and many de- cause sensitization, but many require multiple
velop irritant dermatitis (Fig. 7.3). If they also exposures before sensitization occurs. It is pos-
have atopic eczema their hand problem often sible t o be exposed t o an allergen for years, and
becomes so severe that they are forced t o leave then suddenly develop hypersensitivity.
hairdressing. A similar situation is seen in ma- Frequent causes of contact dermatitis in-
chine-tool operators whose hands are im- clude nickel, colophony, rubber additives, chro-
mersed in cutting fluid. It follows that young mate, hair dyes and topical medicaments- both
people suffering from atopic dermatitis should their active ingredients and components of
be advised t o avoid careers in occupations in- their bases.
volving contact with irritants, such as hairdress-
ing, engineering, vehicle mechanics, nursing and Nickel dermatitis
catering. Nickel is the commonest cause of contact
In theory, the treatment is simple-either re- dermatitis in women, whereas contact allergy
move the patient from contact with the irritant, t o nickel is uncommon in men. Sensitization t o
o r protect the hands against it. In practice it is nickel usually occurs in childhood and early
often impossible t o avoid contact with the irri- adult life as a result of ear piercing and the
tant without changing jobs, and in many occupa- wearing of cheap costume jewellery.The prob-
tions the nature of the work means that wearing lem usually begins with itchy earlobes, but it
gloves is impracticable.The skin can be helped is dermatitis caused by the metallic components
t o a certain extent by the liberal use of emol- of other garments which brings the nickel-
lients (see Chapter 22), but it cannot be re- sensitive patient t o the dermatologist. In the
stored t o normal whilst exposure t o irritants pre-miniskirt era, suspender dermatitis was
continues. What usually happens is that severe the commonest presentation of nickel sensi-
dermatitis eventuallyforces a change of occupa- tivity. Suspender belts were perhaps more
tion, o r individuals with milder problems learn functional than decorative in those days, and
t o tolerate them. the bare metal clips produced patches of
dermatitis on the thighs. W i t h the advent of
Allergic contact dermatitis the miniskirt and tights, suspender dermatitis
This is due t o a delayed hypersensitivity reaction disappeared from the dermatology clinic. A
Exogenous eczema 61

Fig. 7.4 Contact dermatitis to

nickel in jeans stud.

more recent resurgence of interest in the sus- problems from watches can usually be avoided
pender belt has not been accompanied by skin by wearing a'swatch' watch, as the only metal in
problems, because the clips are coated metal o r contact with the skin is the stainless steel bat-
synthetic material. I t is the jeans stud which has tery compartment.
become an important source of nickel, and
a patch of eczema adjacent t o the umbilicus is Colophony
virtually pathognomonic of nickel sensitivity This is a resin which is a component of some ad-
(Fig. 7.4). hesive plasters.
If nickel dermatitis is suspected, look at the
skin on the earlobes and wrists. In spite of being Rubber dermatitis
aware that costume jewellery provokes a skin During the manufacture of rubber, chemicals
reaction, many women continue t o wear a are used t o speed up the vulcanization process
favourite pair of earrings from time t o time, and (accelerators) and t o prevent its oxidation (an-
will have dermatitis on the ears. Nickel dermati- tioxidants), and these can cause allergic contact
tis on the wrists is usually caused by the metal dermatitis.
buckle on a watch-strap. Stainless steel in wrist- In recent years there has been a marked
watches does not cause any problems because increase in the occurrence of reactions t o
although steel contains nickel it is tightly bound natural rubber latex protein in latex gloves.
and does not leach out. Latex protein can provoke an immediate hyper-
The multitude of folk who have adopted the sensitivity response, and reactions range from
'fashion' of having their delicate bodily parts contact urticaria t o rhinitis, asthma and anaphy-
pierced are perhaps fortunate in that the vari- laxis. Individualsaffected are most often health-
ous rings and barbells dangling and protruding care workers who frequently wear latex gloves,
from them are made of steel, and do not pro- but patients who have undergone multiple
voke dermatitis unless further embellished with procedures, most notably persons suffering
costume jewellery. Anyone who is allergic t o from spina bifida, may also be affected. A signifi-
nickel should be advised t o avoid costume jew- cant factor contributingt o this problem appears
ellery (unless it is known t o be nickel free), bare t o have been that the demand for latex gloves
metal clips on underwear, metal buckles on outstripped supply, and some manufacturers
shoes and metal zips. The metal stud on the subsequently produced gloves containing large
front of jeans can be replaced by a button, and amounts of free latex protein.
62 Chapter 7: Eczema

Chromate sponsible for contact dermatitis, a detailed his-

Chromium compounds have a number of indus- tory, including precise information about the
trial applications.They are also used in leather nature of an individual's work, is essential. This
tanning, and are the major sensitizer in cement. should include not only information about pre-
Cement dermatitis is common in building sent occupation but also details of previous em-
workers. p1oyment.A history of significant improvement
of the dermatitis during holiday periods is typi-
Hair dye dermatitis cal of a work-related dermatosis. If someone
Contact sensitivity t o hair dye usually presents tells you he was a 'saggar-maker's bottom
as severe dermatitis affecting the face, ears and knocker', enquire as t o the nature of this exotic-
scalp margin. Hair dyes are also a cause of con- soundingoccupation (it used t o be encountered
tact dermatitis on the hands of hairdressers.A in areas where ceramic ware was produced) -it
chemical present in hair dyes is also used in tem- is common t o encounter terminology which is
porary tattoos, a form of decoration which is specific t o a certain occupation, and is incom-
quite popular at present, and contact dermatitis prehensible t o those outside the trade. Estab-
in the tattoos has been reported. lish what materials are handled at work, and if
there have been any changes which coincided
Topical medicaments with the onset of the dermatitis. It is also useful
Medicament dermatitis is relatively common in t o know if any workmates have the same prob-
dermatological practice, but perhaps not as lem. Seeing a patient in the working environ-
common as might be expected if one considers ment is often important in determining the
the huge quantities of creams, lotions and po- cause of a dermatitis.
tions used in the average household. Open any
bathroom cabinet o r bedside drawer in any Plant dermatitis
house in the land and you will find creams for Plant dermatitis is relatively uncommon in the
dry skin, creams for haemorrhoids, prepara- UK, but Primula obconica is the plant usually re-
tions for cuts and grazes, creams for insect bites sponsible. In the USA the commonest cause of
and stings, and almost invariably a tube plant dermatitis is poison ivy. Dermatitis caused
of a topical steroid-originally prescribed for by plants tends t o present with a linear, vesicu-
Grandma's varicose eczema, but subsequently lobullous reaction on the exposed parts of the
tried on every member of the family. body.
Common causes of contact dermatitis in
topical medicaments include antibiotics, par- Diagnosis of allergic
ticularly neomycin, local anaesthetics (except li- contact dermatitis
docaine (lignocaine),which is a rare sensitizer), I t is important t o take a detailed history cover-
antihistamines and preservatives. Dermatoses ing present occupation, previous occupations,
in which contact sensitivity t o some of these hobbies and the use of topical medicaments.
agents may be a complicating factor include oti- The distribution pattern of the dermatitis may
tis externa, pruritus ani and venous leg ulcers. In suggest a possible allergen, and provoke further
recent years, contact allergy t o topical steroids questions -for example, eczema adjacent t o
has emerged as a problem. It is usually dis- the umbilicus prompts enquiry about previous
covered in patients who fail t o respond o r problems with earrings. Certain patterns are
experience a deterioration in their skin condi- characteristic o f a particular allergen: in the days
tion whilst undergoing treatment with topical when 'strike anywhere' matches were in com-
steroids. mon use contact sensitivity t o phosphorus
sesquisulfide, which is present in the heads of
Occupational contact dermatitis the matches, was responsible for a combination
If occupational factors are thought t o be re- of eczema on the face, in the ears, on the hands
Endogenouseczema 63

andonone orotherthigh.Thefacia1eczema was

Endogenous eczema
caused bv contact with the smoke from the
matches. the hand eczema by handling the
Atopic eczema
matchbox. which had thechemical onthestrik-
The term atopy implies a genetic predisposi-
ing surface, and that on the thigh from carrying
tion to develop eczemq asthma and hay fever. A
the box in a trouser pocket.The eczema in the
family history of atopy is common in patients
ears was the resuit of using matches to clean
with atopic eczema.
them out!
The pathogenesis of atopic eczema i s
However, when the cause is notas obvious it
complex, but involves immunological abnor-
may require considerable detective work to
malities, environmental factors and emotional
trackitdown,and patchtesting(seeChapter2)
influences. Immunological abnormalities in the
is an essential component of the investigative
atopic state include increased serum total IgE
process. Patch testing is quite different from
and specific IgE antibody to ingested or inhaled
prickorscratchresting. ltisadelayed hypersen-
antigens, and preferential activation of theTh2
sitivity response, in which the reaction takes
phenotype C M T cells, which form interleukin
48 h to develop, whereas prick or scratch tests
4 (IL-4) and IL-5.The interleuldns stimulate IgE
elicit an immediate hypersensitivicy response
synthesis by B cells. Staphylococci colonize
which develops within minutes.A standard bat-
the skin of patients with ampic eczema, and
tery of common allergens is used in routine
staphylococcal exotoxins with superantigen
patch testing, but other batteries of allergens,
pmperciesarealsothoughtu, play apathogenic
such as components of topical agents or occu-
pational allergens, are also available.The major-
Atopiceczemais notpresentat birth, butfre-
ity of the allergens used are mixed in white soft
quently appears in the first year of life. In early
parafh to a specific concentration, because
childhood the eczema is often generalized, but
many are irritant in high concentration and
later a characteristic flexural involvement is
might produce false-positive reactions. Patients
seen-wrists, antecubital fossae. popliteal for-
sometimes claim that they areallergic ca ma-
sae and dorsa of feet (Fig. 7.5).The skin is dry
terials they use at work, and these may be pre-
and intensely itchy. As a result of constant
sented to the dermatologisr. often in unmarked
scratching and rubbing, the affected areas be-
jars. Such materials are often irritants. and if
come thickened (lichenification).The course is
used undiluted for patch testing may bore an
typically punctuated by episodic exacerbations.
untidy hole in the patients back.
Atopic eczema often resolves in childhood,
Positive reactions must be interpreted in the
but may persist into adolescence and adult life,
context of the patients presenting problem-
and there is no way of predicting the outcome.
not all positives will be relevant.
Those whose skin has apparently reverted to
Wait until an acute eczema has settled before
normal remain susceptible to the effects of pri-
patch testing-positive reactions may exacer-
mary irritants. which may provoke a recrudes-
bate the eczema.
The commonest complication is secondary
bacterial infection, producing folliculitis or im-
Potent topical steroids (see Chapter 22) should
petigo.Viral warts and moiluscum contagiosum
occur more frequently in atopics. and herpes
ing. Once an allergen has been identified as the
simplex infection may lead to widespread skin
cause of a problem, the patient should be ad-
lesions (see Chapter 3) and a severe illness
vised about its avoidance. If components of
(euema herpeticum; Kaposis varicelliform
medicaments are involved, the patients family
doctor must be informed of what preparations
-. _ r _
the patient should avoid.
64 Chapter 7: Eczema

Fig. 7.5 Flexural involvement in

atopic eczema.

7Yeatment tive antihistamine at night may help t o reduce

An important aspect of the management of a scratching. Ultraviolet light treatment, either
child with atopic eczema is sympathetic ex- UVB o r psoralens and UVA (PUVA therapy),
planation of the nature of the condition t o its helps some atopics, but the eczema often re-
parents. lapses when treatment is stopped. Ciclosporin
Emollients are essential in the managementof (cyclosporin) may be of great benefit t o patients
the dry skin in atopic eczema.There are numer- with severe atopic eczema.
ous emollients available, and it is often neces- The value of dietary manipulation is contro-
sary t o t r y a number of preparations in order t o versial. Some children appear t o be helped by
find one which is suitablefor a particular individ- elimination diets in which dairy products, food
ual. They can be used in combination at bath- additives, nuts, and other foods suspected of ex-
time-for example, one as a soap substitute, a acerbating eczema are excluded, but in many
bath oil in the water, and an emollient cream there is no obvious benefit. Most dermatolo-
afterwards. gists reserve dietary manipulation for severely
Topical steroids are invaluable in the treat- affected children who fail t o benefit from other
ment of atopic eczema. In young children mild treatment methods. It is dangerous t o manipu-
steroids are the mainstay. In older children and late a child's diet without expert advice, as this
adults more potent steroids are required, but can lead t o nutritional deficiencies.
the aim should always be t o use the weakest Chinese herbal therapy is another controver-
preparation sufficient t o control the disease. A sial issue. Undoubtedly some individuals have
topical steroidlantibacterial combination may benefited from Chinese herbal medicines, but
be useful if eczema frequently becomes secon- there is concern about their potential for hepa-
darily infected -obvious secondary infection totoxicity and nephrotoxicity, and some topical
should be treated with a systemic antibiotic 'herbal' medicines have been shown t o contain
such as flucloxacillin o r erythromycin. Emol- steroids.
lientlantimicrobial combinations may also be
useful in reducing bacterial colonization of the Seborrhoeic dermatitis
skin. This is a constitutional disorder whose exact
The wet-wrap technique is useful in the man- pathogenesis is not fully understood, but in re-
agement of severe eczema, and medicated ban- cent years the role of Malasseziayeasts has been
dages such as zinc paste and ichthammol o r zinc emphasized.
oxide and coal tar, applied over a topical steroid, Seborrhoeic dermatitis affects the scalp, face,
are beneficial for eczema on the limbs. A seda- presternal area, upper back and flexures. Scalp
Endogenous eczema 65

involvement presents as itchy, diffuse scaling Mild o r moderate potency topical steroids
on an erythematous background. O n the face, will usually suppress the eczema.
there is scaly erythema in the nasolabial folds
and on the forehead, eyebrows and beard area Endogenous eczema of the palms
(Fig. 7.6). Lesions on the chest are often mar- and soles
ginated. Flexural involvement produces a moist, Some patients develop a symmetrical pattern
glazed erythema. Particularly severe sebor- of eczema affecting the palms and soles which is
rhoeic dermatitis occurs in patients suffering
from AIDS.
Seborrhoeic dermatitis usually requires
treatment over many years, as there is no cure
for this condition. I t is important t o make this
clear t o patients, who otherwise tend t o t r y
many treatments in their quest for a permanent
solution t o the problem.Topical hydrocortisone
is effective, but the problem recurs when treat-
ment is stopped. Steroid lotions o r gels and tar
shampoos will help the scalp.
Ketoconazole shampoo and cream, and imi-
dazole/hydrocortisone combinations are also

Discoid eczema
In this disorder, scattered, well-demarcated
areas of exuding and crusting eczema develop
on the trunk and 1imbs.A potent topical steroid
is usually required t o keep the condition con-
trolled. Its aetiology is unknown.

Varicose eczema
Chronic venous hypertension is frequently as-
sociated with eczematous changes on the legs.
Secondary spread t o the forearms may occur. Fig. 7.6 Facial seborrhoeic dermatitis.

Fig. 7.7 Pompholyx.

66 Chapter 7: Eczema

Fig. 7.8 Eczema craquelh.

ondary bacterial infection is common. It usually

responds t o treatment with potassium perman-
ganate soaks and a systemic antibiotic such as
flucloxacillin o r erythromycin. The trigger for
these episodes is unknown.

Asteatotic eczema
W i t h increasing age, the lipid content of the
stratum corneum decreases, and elderly skin is
particularly susceptible t o 'degreasing' agents.
Asteatotic eczema (also known as eczema
craquele) is usually seen on the legs, but it may
also occur on the lower abdomen and arms, and
occasionally it is generalized. It is common in el-
derly patients admitted t o hospital and bathed
more frequently than they bathe at home. A
crazy-paving pattern develops (Fig. 7.8). and the
skin itches. Treatment with an emollient is
sometimes adequate, but a mild topical steroid
ointment is often necessary.

Juvenile plantar dermatosis

As its name suggests, this condition occurs in
children. It is thought t o be related t o wearing
socks made of synthetic materials and training
shoes. The weight-bearing areas of the feet are
chronic, and does not appear t o be related t o dry and shiny, and painful fissures occur (Fig.
any external factors. Long-term treatment with 7.9). Changing t o cotton o r woollen socks and
potent topical steroids is usually required. leather shoes sometimes helps, as does the
An episodic form of eczema of the palms and liberal use of emollients. Topical steroids are
soles, in which bullaformation occurs, is kt-~own usually ineffective. It almost invariably resolves
as acute pompholyx (Fig. 7.7). This develops by the early teens.
rapidly, and can be severely incapacitating. Sec-
Introduction.67 Treatment ofpsoriasis.72 Arthropathicpsariasis, 75
Pathology,67 Treatment ofclinical patterns Reiter's syndrorne,76
Clinicalpatternsof psariasis.67 ofpsoriasis.74

Dermatologistsdo it an agrandscale. (Anon) of inflammatory cells. The 'epidermal transit

time' is markedly reduced from the normal
8-10 weela t o a few days. There is also
Introduction increased vascularity of the upper dermis.
Figure 8.1 provides a schematic representation
Psoriasis is one of the commonestand most im- of a psoriatic plaque.The cardinal features arc
portant of the inflammatory dermatoses: up to given below.
2% of the population of western countries de-
velop psoriasis during their lifetime. It is also
common in India, the Far East and parts of
Africa. As most of those who develop psoriasis
have lesions for the rest of their lives, it is
* Marked thickeeningofthe epidermis
clearly a considerable problem.
* Absenceofthegranulluelllayer
It is still not known why psoriasis develops. Retention ofnucleiinthehomylayer
There is a strong genetic component in many, (parakeratosis)
particularly if the disease begins in youth or A c c u m u l a t i o n a o f p s i n t h c homy
early adulthood. However,although afamily his- 1-r (microakesses)
tory is common, there is often no clear-cut in- Dilated capillalyloopintheupprdermir
heritance pattern and the'genetic' explanation
may not be readily understood by patients. This basic picture, with some variations
Some well-recognized triggers may induce (e.g. increased size and number of polymorph
psoriasis in susceptible individuals, notablytrau- abscesses in pustular psoriasis), unites all forms
ma and infections. Some authorities also main- of psoriasis and the skin lesions of Reiter's
tain that stress may induce o r exacerbate the syndrome (see pp.76and 164).
condition. However, there is no clear under-
standing of what causes some areas of slcin t o
turn into plaques of psoriasis while others re- Clinical patterns of psoriasis
main normal.
A number of different clinical patterns of
proriasis are recognized.
Pathology Some are common, others are rarer: some
may be seen together o r overlap. However,
The pathological process is a combination of there is some merit in considering them
epidermal hyperproliferation and accumulation separately.
68 Chapter 8: Psoriasis

Fig.8.1 Schematic
representation of a psoriatic

metrical. Involvement of the face is relatively un-

common.The scalp and nails are often affected
(Figs 8.3 & 8.4), and an arthropathy may also
* Classicalplaque occur (see p. 75).
* Scalp psoriasis Plaques tend t o be chronic and stable, with
little day-to-day change (as compared with 'brit-
* Cunate
* Flexural tle' psoriasis-see below). However, they may
* Brittle enlarge slowly, and may merge with adjacent
* EIythrcde-c areas.They may also resolve spontaneously. Oc-
* Acutepustular casionally, psoriatic plaques appear at the site of
* Chronic palmo-plantarpustulosis trauma o r scarring.This is known as the Kobner
Arthropathicporiasis o r isomorphic phenomenon and is a character-

istic, but not pathognomonic, feature. Exposure

Classical plaque psoriasis t o UV radiation and natural sunlight often (but
This is the commonest pattern.There are single not always) improves psoriasis.
o r multiple red plaques, varying from a few mil- I t is often said that psoriasis is not itchy,
limetres t o several centimetres in diameter, but in our experience a significant number of
with a scaly surface (Fig. 8.2). If scraped very patients complain of severe itching, and most
gently, the scale can be seen t o reflect the light, experience some itch at times. In fact, the
giving a'silvery' effect (due t o the parakeratotic Greekpsora actually means itch. Some forms of
horny layer). More vigorous rubbing induces psoriasis (e.g. guttate, flexural) are more prone
capillary-point haemorrhage. t o cause irritation.
The plaques may develop on any part of the
body, but psoriasis has a predilection for exten- Scalp psoriasis
sor surfaces: the knees, the elbows and the base Scalp involvement is very common: indeed the
of the spine. Lesions are often strikingly sym- scalp may be affected alone. It can be difficult t o
Clinical patterns of psoriasis 69

Fig.8.2 Psoriatic plaque on the


Fig.8.3 Scalp psoriasis. Fig.8.4 Nail pits in psoriasis.

distinguish scalp psoriasis from severe sebor- thick and sticks in large chunks t o bundles of
rhoeic dermatitis (see also flexural psoriasis hair. This is known as 'pityriasis amiantacea'.
below), but psoriasis is generally thicker. As a There may be temporary hair loss in severe
rule of thumb, if you can feel scalp lesions as well scalp psoriasis.
as see them, they are probably psoriasis.
Lesions vary from one o r t w o plaques t o a Nail psoriasis
sheet of thick scale covering the whole scalp Nail abnormalities are frequent, and are impor-
surface (Fig. 8.3). Rarely,the scale becomes very tant diagnostic clues if skin lesions are few, o r
70 Chapter 8: Psoriasis

Fig. 8.5 Early psoriatic


atypical. Nail changes are almost always present

in arthropathic psoriasis.
Two common findings may occur together o r
alone: pitting and onycholysis. Psoriatic nail pits
are relatively large and irregularly arranged (Fig.
8.4), compared with those of alopecia areata.
Onycholysis (lifting of the nail plate) initially pro-
duces a dull red area with a salmon-pink rim (Fig.
8.5), but the nail becomes brown o r yellow in
time. It is sometimes painful.These nail changes,
particularly onycholysis, may also occur with-
out other evidence of the disease.
Occasionally, pustular changes occur at the
ends of the digits and in the nail bed (sometimes
known as 'acrodermatitis continua'). Similar
changes may accompany chronic palmo-plantar
pustulosis (see below). In erythrodermic o r
pustular forms of psoriasis the whole nail may
become roughened and discoloured.

G u t t a t e psoriasis
Guttate psoriasis often develops suddenly, and
may follow an infection, especially a streptococ-
cal sore throat. I t is a common way for psoriasis Guttatepsoriasis.
t o present, particularly in young adults.
Gutta is the Latin for 'drop'. Most lesions are totic scale in psoriasis, and the shape of the le-
about a centimetre in diameter (Fig. 8.6), and sions (round in guttate psoriasis; oval in pityria-
usually paler in colour than established plaque sis rosea). Guttate psoriasis may itch.
psoriasis, at least initially. The main differential The lesions of guttate psoriasis often resolve
diagnosis is pityriasis rosea (see Chapter 15), rapidly, but in some patients the patches enlarge
best distinguished by the presence of parakera- and become stable plaques.
Clinical patterns of psoriasis 71

Fig.8.7 Flexural psoriasis.

Flexural psoriasis
Flexural involvement in psoriasis may accom-
pany typical plaque lesions, but is also common-
ly seen alone, o r associated with scalp and nail
changes. Lesions may occur in the groin, natal
cleft, axillae, umbilicus and submammary folds.
Maceration inevitably ,occurs, and the surface
scale is often lost, leaving a rather beefy erythe-
matous appearance (Fig. 8.7). It may be difficult
t o distinguish this from ftexural seborrhoeic
dermatitis, so look for nail changes o r evidence
o f psoriasis elsewhere. Some dermatologists
believe in an overlap state between the two, and
call such changes'sebo-psoriasis'.
Flexural psoriasis is often itchy.Watch out for
a secondary contact sensitivity from the use o f
proprietary anti-itch preparations.

Brittle psoriasis
Occasionally you will see patients whose psori-
asis does n o t consist ofthick, stable plaques, but
o f thin, irritable scaly areas (Fig. 8.8). Lesions
may arise de nova o r develop suddenly in a pa- Fig. 8.8 Widespread 'brittle' psoriasis.
tient whose psoriasis has been stable for years.
One reason for this is systemic steroid therapy
(often for another condition), and potent topi-
cal steroids can also induce stable psoriasis t o Chapter 15) o r even acute pustular psoriasis
become 'brittle'. (see below).
The significance o f brittle psoriasis is that the
lesions may rapidly generalize, especially if Erythrodermic psoriasis
treated with potent agents (see treatment When psoriatic plaques merge t o involve most,
section below), leading t o erythroderma (see o r all, o f the skin a state o f erythroderma o r
72 Chapter 8: Psoriasis

Fig. 8.9 Acute pustularpsoriasis

exfoliative dermatitis results.The effects of this

are discussed in Chapter IS. Fig. 8.10 Chronic palmo-plantarpustulosis
Psoriasis may become erythrodermic by
slow, inexorable progression, o r very rapidly.
Occasionally, erythrodermic psoriasis may ap-
pear de now. Systemic steroids o r potent topi- tween thisconditionand otherformsofpsoria-
cal steroids may precipitatethis, sis. Biopsies reveal psoriasiform pathology, but
it is unusual for patients t o have chronic palmo-
Acute pustular psoriasis piantar pustulosis in association with other
(of von Zumbusch) types of psoriasis.
This is a very serious condition. Patients with The typical changes consist of erythe-
o r without pre-existing psoriasis suddenly matous patches with numerous pustules
develop widespread erythema, superimposed (Fig. 8.10). These gradually change into
on which are pustules. These may coalesce brown, scaly spots and peel off.The condition is
into lakes of pus (Fig. 8.9).The pustules are usually uncomfortable or painful, rather than
sterile. itchy
The patient has a high, swinging fever and Lesions may involve only a small area of one
is toxic and unwell, with a leucocytosis. If handorfoot,orcovertheentiresurfaceofboth
the disease is unchecled, patients become palms and soles.Thir may lead to considerable
increasingly ill and may die, often of secondary disability.

Chronic palmo-plantar pustulosis Treatment of psoriasis

(pustular psoriasisof palms
and soles) Theagentsmostwidelyusedinthetreatmentof
There is somedebateaboutthe relationship be- the skin lesions of psoriasisare:
Treatment of psoriasis 73

topical steroid in a commercially available


Tar has been used for many years, particularly in
combination with UV radiation.The most effec-
* Salicylicaad tive preparations are extracts of crude coal tar.
Topicalsteroids Attempts have been made t o refine tar t o make

* Dithrand(anthralin)
Vitamin D analogues(e.g. calciporriol,
it more cosmetically acceptable, but the most
effective forms still seem t o be the darkest,
smelliest and messiest. Consequently, not many
* VitaminAanalogues patients will use tar forwidespread, routine use.
1 Ultravioletradiation
However, in bath oils o r in ointment mixtures
tar may be helpful, and is very valuable in scalp
PWA (pMralen+ultravioletA) disease.
* Cytotoxics.e.g.methomxate,azathioprine. Topical steroids
hydmxycarbamide(hydmxyurea) Topical steroids do not eradicate psoriasis, but
* Systemicsteroids
may suppress it. Some dermatologists say they
never use topical steroids in psoriasis because
of the risks (they may induce'brittle' psoriasis).
However, if used with care in stable disease, and
It is an old adage that if there are many treat- on the scalp and in the flexures, they can be
ments for a disease, none works perfectly. useful.
This is certainly true of psoriasis.Although each
modality is useful in some patients, all represent Dithranol (anthralin)
a degree of compromise in terms of safety, ef- Dithranol can convert psoriatic plaques into
fectiveness o r convenience. Many patients re- completely normal-looking skin. The mode of
quire a regimen of different agents for different action is unknown. The 'Ingram regime'-
sites at different times. a combination of dithranol, tar and UV radia-
tion -has been used for years: most patients
Topical therapies can be cleared in about 3 weeks of daily treat-
Many agents can be used topically t o induce a re- ment. Originally, the dithranol was left on the
mission o r an improvement. Most are safe, but skin for 24 h, but 'short-contact' therapy is just
they are tedious for patients t o use, as treat- as good.
ment may have t o continue for months o r even Dithranol seems t o work best in Lassar's
indefinitely. paste (starch, zinc oxide and salicylic acid in
white soft paraffin), but is also available in
EmoIlients cream and ointment bases. Always begin with
Some patients are prepared t o tolerate plaques a low concentration (0.1%) and increase as
(especially on covered sites) if scaling can be necessary.
controlled. Emollients such as white o r yellow The main complications are staining (due
soft paraffin o r lanolin may accomplish this. t o oxidation t o a dye) and burning. Skin staining
is temporary, but baths, bedding and clothes
Salicylic acid may be permanently marked. Dithranol burns
Salicylic acid is a 'keratolytic' agent and helps can be very unpleasant, especially around the
t o reduce scaling. It can be used with tar in eyes. Patients must be taught t o use dithranol
mixtures, and is also combined with a potent carefully.
74 Chapter 8: Psoriasis

Vitamin D and vitamin A analogues All cytotoxics have unwanted effects, par-
The vitamin D analogues calcipotriol and tacal- ticularly bone marrow suppression. This is
citol worl<well, and have rapidly found a place in rare with methotrexate, but may occur in an
routine management.Vitamin A analogues are idiosyncratic manner unrelated to dose. The
favoured by some authorities but are generally major problem with methotrexate is hepato-
less effective. There are few local side-effects toxicity, particularly fibl-osis with chronic use.
with either group (although vitamin D ana- Alcohol appears to exacerbate this tendency.
logues may burn on the face and in thcflexures), Younger patients require regular liver biopsies.
but calcium levels may be distul-bed if large Methowexate also inhibits spermatogenesis
quantities of vitamin D analogues are applied and is teratogenic. Its use is therefore irestl-icted
and patients using topical vitamin A may be t o severely affected patients.
advised t o avoid pregnancy because of
teratogenicity. Retinoids
Vitamin A derivatives help some patients with
Ultraviolet radiation psoriasis.The most commonly used is acitretin.
The use of UV light therapy is well established, Retinoids have a number of side-effects, includ-
the most effective wavelengths being in the ing dry lips, nose-bleeds, hair loss, hyperlipi-
medium (UVB) range. UVB must be used with daemia, liver function test abnormalities and
care because it also induces sunburn. Patients teratogenicity.
require doses that just induce erythema but do
not cause burning. The dose is then increased Systemic steroids
gradually Treatment is usually given twice In very severe psol.iasis, steroids may occasion-
weeldy until clearance is achieved. Adjunctive ally be necessary, butshould not be usedalone.
tar may mal<eUVB more effective.
UVB is theoretically carcinogenic (as is tar), Ciclosporin (cyclosporin)
but surprisingly few psoriasis sufferers develop This immunosuppressivedrugworkr extremely
skin cancers. well, even in very severe psoriasis. It is nephro-
toxic and very expensive.
Systemic therapies
Psoralen + ultraviolet A (PUVA)
'Psoralens' form chemical bonds with D N A in Treatment of clinical patterns
the presence of UV radiation.The most widely of psoriasis
used agent is 8-methoxypsoralen, which is
usually taken by mouth 2 h before exposure t o The choiceoftherapeutic regimen in psoriasis is
long-wavelength UV light (UVA), initially twice dictated by the type and extent of lesions, and
weel<ly. Protective glasses are worn to prevent by the effects on the patient's quality of life.
ocular damage.To reduce this risk, some units A balance will often have t o be struck between
now seal< patients' skin in a bath of psoralen the need for improvement and the incon-
solution.There is a significant long-term risk of venience andlor side-effects of the agent(s)
Ikeratoses and epithelia1 cancers with both concerned.
Chronic plaque psoriasis
Cytotoxic drugs Dithranol is a theoretical first choice, but the
The most effective and widely used cytotoxic patient's life-style, o r side-effects, may male it
is methotrexate, a folic acid antagonist. Most impractical. If so, vitamin D analogues o r topical
psoriasis responds t o a once weekly dose of steroids (with o r without tar and salicylic acid)
7.5-20mg. Other drugs include azathioprine are often used. UV radiation may help. If lesions
and hydroxycarbamide (hydroxyurea). become very extensive, o r if there are serious
Arthropathic psoriasis 75

psychosocial problems. PUVA, retinoids o r

cytotoxic drugs may be indicated.

Scalp psoriasis
Tar shampoos are helpful, but will seldom
control thick plaques alone. Tar gels may
help, butthe besttopical remedy is Unguentum
Cocois Co.-a mixture including tar and
salicylic acid. This is massaged in at night and
washed out the following morning. Topical
steroid lotions, with orwithoutsalicylic acid are
also used.

Nail psoriasis
Nail changes do not respond t o topical treat-
ment, and systemic drugs are seldom justified
for nails alone.

Guttate psoriasis
This is most easily treated with UV radiation
together with emollients and a tar-based

Flexural psoriasis
Psoriasis in the flexures poses problems. Mild
taricorticosteroid mixtures may be effective,
but long-term use of topical steroids can cause choice is methotrexate, but ciclosporin (cy-
striae. Dithranol, used in very low concentra- closporin) also works well. When the condi-
tions, can be successful, but burning is common tion is stable, the dose should be gradually
and underclothes are stained. UVB and PUVA reduced and the drug stopped if possible.
generally fail t o ,reach the affected areasvitamin However, many patients relapse and require
D analogues help, but can sting. long-term treatment.

Brittle psoriasis Chronic palmo-plantar pustulosis

Brittle psoriasis requires careful management. Nothing really worls well in this condition.
Avoid potent topical steroids, strong tar and Tar pastes, potent topical steroids or dithranol
salicylic acid preparations. Emollients o r very are often ineffective. PUVA to the hands
dilute steroids may bring the skin into a more and feet may provide control, but relapse i s
stable condition, but PUVA, retinoids o r common.
methotrexate may be needed, at least for a
Arthropathic psoriasis
Erythrodermic and acute
pustular psoriasis One of the most unpleasant complications of
Although both of there states may re& with psoriasis is arthropathy, affecting up t o 10% of
conservative management, it is more l i l d y that psoriaticr.There are four basic patterns.
systemic treatmentwill be 1-equired.Such inter- Most commonly the distal interphalangeal
vention can be life saving. The most common joints are involved, with theother changes listed
76 Chapter 8: Psoriasis

Non-steroidal anti-inflammatory drugs and

methotrexateare used.

- Distalinterphalangealjointinwlvement
Seronegativerheumatoid-likejoint changes Reiter's syndrome
j Spondylitis This disorder. which frequently follows a diar-
rhoeal illness o r non-specific urethritis in HLA
627-positive individuals, is discussed in Chapter
above in descending order of frequency. Psori- 19. Occasionally sldn lesions lknown as'ikerato-
aticarthropathy is erosive and may result in joint derma biennorrhagicum' develop. Palmar and
destruction. plantar lesions may become very gross (Fig.
Psoriadcs who develop the spondylitic form 8.1 I), and lesions elsewhere are clinically very
are usually HLA 627 positive, and there is similartopsoriasis. Histologically.Ikeratoderma
some overlap between psoriatic arthropathy blennorrhagicum is indistinguishable from
and other seronegative arthritides. psoriasis.
Benign and Malignant
Skin ITClmouvs

Introduction and classification General treatment principles Specific tumours.79

ofskin tumours,77 for skin tumours.77

Know ye not that a little leaven leoveneththe The first important principle is that, unless
wholelump?St Paul ( I Corinthians, 5 :6) the diagnosis is certain, some tissue should be
PI-eservedfor histology Failure t o do this will
mean missed malignancies, and is one explana-
Introduction and classification tion for patients who present with mysterious
of skin tumours lymphatic or distant deposits from unlmown
primary rites.
Lumps on or in the skin are extremely common
and the workload associated with them is rising Surgical removal o r biopsy
because: These techniques have already been described
I The age of the population as a whole is in- and illustrated (see Figs 2.1 & 2.2). Removal
cleasing (many sldn tumours are commoner in of small skin tumours is quick, simple and
rheelderly). economical. If the tumour is too large for pri-
2 Skin cancer is increasing in all agegroups. mary excision, take a small incirional biopsy,
3 There is increasing public awareness of the remembering t o cross the edge from normal
importance of skin tumours. t o abnormal tissue. There is no evidence that
Most skin tumours are benign, often repre- such a biopsy adversely affects the outcome,
senting only a cosmetic nuisance. However, it is although it is advisable t o avoid incisional
important t o distinguish these from malignant biopsy of invasive melanomas if possible (see
or potentially malignant tumours quickly and below).
effectively, as decisions about what should be
done about a lesion can only be made after a Curettage andlor
diagnosis t o this minimum level has been made. cautery ('C&C9)
The skin is a complex organ system, with This is a perfectly satisfactory method for the
both benign and malignant tumours described removal of superficial tumours.
for every component. Table 9.1 presents a
simplified version of the wide variety of sl<in

General treatment principles 1 Use a curette (volkmann spoon) to scrape

for skin tumours offlesions
2 Touchtherawbaseafewtimeswiththe
cauteery to controloozing
I t is worth reviewing briefly the techniques used
3 Applya simple dressingandlorantiseptic
to treat skin tumours.This will avoid repetition.
78 Chapter 9: Benign and MalignantSkinTumours

Table 9 1 lhmours,benign or malignant,found in the epidermis and dermis.

An alternative t o cautery is a hyfrecator,

which produces electrical haemostasis and
Pedunculated tumours can be removed by
slicingwith cautery across the base.

The use of cryotherapy for tumours has
become very popular. It is ideal for superficial
skin tumours because it is quick and leaves rela-
tively little scarring. However, histological inter-
pretation of cryobiopsies is not easy and jt
shoul'd be used only if: the tumour is definitely
benign; o r an incisional biopsy has already The patient should be told t o expect
been performed. Cryotherapy is not appropri- blistering, followed by healing with crust for-
ate for melanomas. The best agent is liquid mation. The lesion should separate within 3
nitrogen. weeks.
Specific tumours 79

Radiotherapy cussing the commonest and most important of

Radiotherapy is an effective treatment method there.
for basal and squamous cell carcinomas, and is Some sl<in lumps are hamartomatous mal-
often the most practical option for very large formations. Such a lesion in the skin is termed
tumours in the elderly. However, it is nor ideal a 'naevus'. Naevi are discussed separately in
for some areas of the body, and the choice Chapter 10.
between excision and radiotherapy should be
based on individual circumstances. Benign tumours-epidermal
Radiotherapy can also control secondary Seborrhoeic keratoses (seborrhoeic
tumour deposits. warts; basal cell papillomas)
You are bound to see seborrhoeic lkeratores,
Lasers and photodynamic therapy if only in passing while examining a chest.They
There is an increasing interest in the applica- are most f~equentin the elderly, and may be
tion of laser technology to treating skin disease. solitary o r multiple. Occasionally there are
especially for skin tumours and naevi (see hundreds of lesions (a tendency which may be
Chapter 1 O), butalsoforhirsutirm (see Chapter familial).
13). Many benign epithelia1 tumours will
respond t o ablation by a CO, laser but are Clinical features.A flat-topped area of skin with a
also very easily tl-eated by other, simpler and 'stuck-on' appearance (Fig. 9.1). They may be
cheaper means. Pigmcnted lesions respond t o pale, but are often pigmented, sometimes
several lasers but their place has yet to be fully deeply so.The surface is often said t o be greasy,
established. but a more useful sign is the granular look occa-
Photodynamic therapy (PDT) is a process sioned by small surface pits and irregularities.
involvingthe use of a porphyrin and light, which
destroys superficial lesions such as Bowen'r Sites of predilection. Head and neclc bacl<s of
disease and superficial basal cell carcinomas. hands and forearms; trunk.

Differential diagnosis. Usually straightforward,

Specific tumours but darkly pigmented lesions can be mistalen
for melanomas. O n the face, seborrhoeic lker-
W e shall f i r s t consider benign tumours, and atoses may remain virtually flat, causing diffi-
then dysplastic and malignant processes, dis- culty in distinguishing them from senile lentigo

Fig.9.1 ?!pica1 seborrhoeic

80 Chapter 9: Benign and MalignantSkinTumours

o r lentigo maligna (see below). Another diag-

nostic problem arises if lesions become in-
flamed. There may be crusting and bleeding,
and biopsy for histology may be necessary.

Treatment. If deemed necessary (there is no

malignant potential), the best approach for
smaller lesions is cryotherapy. Larger ones may
be better treated by curettage and cautery o r

Skin tags (acrochordons)

Many people develop these small pedunculated
lesions around the neck and in the axillae. In-
creasing age and obesity are predisposing

Differential diagnosis. Small melanocytic naevi

may looksimilar, and so may small pedunculated
seborrhoeic keratoses.

Treatment.They can be removed very easily with

a cautery.
Fig.9.2 Keratoacanthoma.
Keratoacanthoma (once called
'molluscum sebaceum')
This tumour is an oddity. Some authors classify The main problem is t o distinguish pros-
keratoacanthoma as malignant because the pectively between a keratoacanthoma and a
histology resembles a squamous cell carcinoma squamous cell carcinoma. By definition a kera-
(see below). Keratoacanthomasare much com- toacanthoma should resolve spontaneously, but
moner in the elderly. this cannot be determined in advance. lncisional
biopsies may not help because of the close
Clinical features. Lesions arise rapidly, reaching similarities t o squamous cell carcinoma.
a maximal size over the course of 6-8 weeks
(Fig. 9.2). The tumour is round, with rolled Treatment. I t is reasonable t o wait expectantly
edges and a central keratin plug. The base is for a short while if a lesion is very typical,
often red and inflamed, and may be painful. Ulti- especially in the elderly o r frail. However, if
mately, the tumour begins t o shrink, often al- there is any diagnostic doubt keratoacan-
most as quickly as it enlarged, and disappears thomas are best removed and sent for histology.
completely, leaving a small puckered scar. There is a case for removing such a lesion
early, in order t o avoid the necessity for a
Sites of predilection. Almost invariably on light- more complex procedure if it becomes much
exposed skin. larger.

Differential diagnosis. Differentiation from basal Other benign epidermal tumours

cell carcinoma (see below) can be made on the Viral warts are discussed in Chapter 3, and
basis of the history of rapid growth and on the the other benign epidermal tumours listed are
perfect roundness of the lesion. rare.
Specific tumours 81

Fig. 9.3 Milia around the eyes: a

characteristic site.

Epidermal cysts
There are three common forms of epidermal
cyst-pilar, epidermoid and milium.
I Common scalp cysts are correctly termed
'pilar' o r 'trichilemmal' cysts. There may be
several, and a familial predisposition is usual.
2 Epidermoid cysts may be found anywhere,
but are most common on the head, neck and
trunk.They often follow severe acne; there is a
cystic swelling within the skin, usually with an
overlying punctum.

Treatment. Both types can be removed easily

under local anaesthetic using a linear incision
over the surface.
3 Milia are extremely common keratin cysts,
which may occur spontaneously o r after trauma
o r blistering. In some families there is an inher-
ited tendency t o develop clusters on the cheeks
and around the eyes (Fig. 9.3).

Fig. 9.4 Dermatofibroma(histiocytoma).

Treatment. Milia can be treated by incision,
pricking out o r cautery.
Melanocytic naevi are discussed in Chapter
Benign melanocytic tumours 10.
Freckles (ephelides) and lentigines
Freckles are areas of skin containing melano- Benign tumours -dermal
cytes, normal in number but hyperresponsivet o Dermatofibroma (or histiocytorna)
UV radiation. They are genetically determined: Dermatofibromas (Fig. 9.4) are composed of
we all know the typical freckly red-heads. fibrous tissue and some blood vessels. It is not
Lentigines are flat pigmented areas com- known why they occur, but they may follow
posed of increased numbers of melanocytes. minor trauma.
82 Chapter 9: Benign and MalignantSkinTumours

Fig. 9.5 Campbell de Morgan


Fig. 9.6 Side view of a typical

pyogenic granuloma.

Clinicalfeatures. More common in women; often Pyogenic granuloma

easier t o diagnose by touch than by sight-they Pyogenicgranulomas are benign reactive inflam-
feel like small lentils. matory masses composed of blood vessels and
Sites ofpredilection. Usually found on the legs.
Clinical features. They erupt rapidly, usually have
Differential diagnosis. Occasionally, heavy pig- a polypoid appearance (Fig. 9.6) and a 'collar'
mentation can cause confusion with melanoma. around the base; profuse contact bleeding is
Treatment. Excision may be cosmetically
indicated. Sites ofpredilection. Sites of an injury o r infection,
often on a digit.
Angiomas are collections of aberrant blood Differential diagnosis.They must be differentiated
vessels within the dermis andlor subcutaneous from squamous cell carcinomas and amelanotic
tissues. Some are developmental defects, com- melanomas.
monly present at birth, and these are discussed
in Chapter 10. Others develop during adult life, Treatment. Removal by curettage o r excision
such as the ubiquitous Campbell de Morgan should always be followed by histological
spot (Fig. 9.5). examination.
Specific tumours 83

Others Treatment. Cryotherapy may work o r it can

You may encounter several other benign dermal easily be excised.
o r subcutaneous lumps: neurofibromas, for
example in von Recklinghausen's neurofibro- Hypertrophic scars and keloids
matosis (see Chapter I I); various benign fibro- Scar formation can be very exuberant, espe-
blastic tumours; lipomas, which are readily cially at some sites (see below) and in children,
identified by their soft texture and lobulated young adults and black skin. Some authors only
outline. use the term 'keloid' for lesions which spread
If there is any doubt about any dermal o r sub- laterally beyond the original site (keloids can
cutaneous lump it is best removed for histology. become very large).

Pseudo-tumours Clinicaf features. Protuberant masses usually

Chondrodermatitis nodularis helicis following cuts, ear-piercing, burns, acne and
This curious lesion is not a tumour, but an Bacille Calmette-Guerin (BCG) inoculations (if
inflammatory process. performed high on the shoulder); some appear
t o develop spontaneously; keloids often itch.
Clinical features. A small umbilicated nodule on
the rim of the ear, usually in men (Fig. 9.7); the Sites ofpredilection. Chest, upper back, shoulder,
clue is that it is painful, especially in bed at night. pubic region, ear lobes.

Differential diagnosis. It is often confused with Differential diagnosis. Any soft-tissue tumour,
basal cell carcinomas o r other tumours. especially if there is no preceding history of

Treatment. Excision generally leads t o recur-

rence, and management can be extremely dif-
ficult. lntralesional steroids, cryotherapy, and
radiotherapy before and after excision all have
their advocates.

Dysplastic and malignant tumours

The term 'dysplasia' implies that the skin has
been partly, o r wholly, replaced by cells with
neoplastic features. When this results in inva-
sion of adjacent tissue, the process can genu-
inely be said t o be 'malignant'.
Cutaneous dysplasias and malignancies are
increasingly common, especially in ageing skin
and in skin exposed t o prolonged UV radiation.
Other factors are also associated with dysplas-
tic skin changes:
I Most forms o f ionizing radiation (UV light,
X-rays, y-rays) are powerful inducers of skin
2 There are a number of known carcinogens:
exposure t o some industrial oils, tars and bitu-
men; exposure t o soot used t o result in scrotal
Fig. 9.7 Chondrodermatitis nodularis helicis. cancers in chimney sweeps.
84 Chapter 9: Benign and Malignant SkinTumours

Fig.9.8 Basal cell carcinoma.

Note the telangiectatic vessels.

3 Skin cancers are a feature of some genetic

diseases: a notable example is xeroderma pig-
mentosum, in which the repair of UV-induced
DNA damage is faulty.
4 Skin cancers occur more commonly in im-
munosuppressed individuals following renal
and cardiac transplantation.

Basal cell carcinoma (BCC)
The commonest malignant skin tumour is often
known as a'rodent ulcer'.

Clinicalfeatures. Most begin as a nodule (Fig. 9.8)

which spreads slowly outwards, usually leavinga
central depression (creating the classical 'rolled
edge'); usually skin coloured with a translucent
look (often described as 'pearly'); telangiectatic
vessels on the surface are very characteristic,
and account for the frequent presenting com-
plaint of contact bleeding; metastasis is ex-
tremely rare, but local invasion can be very
~ .and B C C can
destructive ( ~ i9.9) ~ spread along Fig.9.9 Basal cell carcinoma. Such destruction
gives rise to the term 'rodent ulcer'.
bony passages into the skull.

Variants. Several distinctive clinical variants of

the BCC are recognized (see p. 85). the trunk (where the superficial pattern is
Sites of predilection. Predominantly the face,
but BCCs occur on other sun-exposed sites, in Differential diagnosis. Early lesions may be con-
the hair-bearing scalp, behind the ear, and on fused with naevi; superficial BCCs are often
Specific tumours 85

A flat gomh pattern which results in a scar-
like appearance;It anbevery difficultto
know where the tumour begrns and ends,and
localinvasionw more common

centimetres across;usualiysolitary;multiple
tumours may indicate previousarsenic
ingestion;characteristidlxa 'worm-like'

verydark anddense

treated as inflammatory; heavy pigmentation

may suggest a melanoma; morphoeic tumours
can be very difficult t o diagnose.

Treatment. Excision, biopsy and radiotherapy or, Fig. 9.10 Superficialbasal cell carcinoma.
for superficial tumours, curettage, cryotherapy
o r photodynamic therapy; careful assessment
of morphoeic tumours is needed-a technique leading t o confusion with lentigo maligna (see
known as 'microscopically controlled surgery' below).
may be helpful; it is particularly important t o
deal adequately with lesions around the eyes, Treatment. Cryotherapy is best for small num-
nose and ears. bers of lesions; large areas on the face and scalp
can be treated with the topical antimitotic agent
Actinic or solar keratoses 5-fluorouracil; in the very elderly it may be best
These are areas of dysplastic squamous epithe- t o do nothing.
lium without invasion, but actinic keratoses do
have low-grade malignant potential and their Squamous cell carcinoma (SCC) in situ
presence indicates unstable epithelium. (or Bowen's disease)
Bowen's disease is a SCC confined t o the epi-
Clinicalfeatures. Red and scaly patches (Fig. 9.1 I) dermis, and is common in the elderly. Invasive
which characteristically wax and wane with change does occur but is rare.
time; many hundreds of lesions may occur in
heavily sun-exposed individuals. Clinical features. Usually a solitary patch of red
scaly skin, although multiple areas may occur;
Sites of predilection. Light-exposed skin, espe- Bowen's disease is asymptomatic.
cially the face, forearms, dorsa of hands, lower
legs and bald scalp. Variant. Erythroplasia of Queyrat- non-
invasive dysplastic changes may also occur on
Differential diagnosis. Some are pigmented, the penis, where the clinical appearance is of a
86 Chapter 9: Benign and Malignant SkinTumours

Fig.9.11 Multiple solar keratoses.

Fig.9.12 TWOpatches of Bowen's

Specific tumours 87

velvety red plaque. Although given a separate t o regional lymph nodes and beyond (especially
name, it is essentially the same as Bowen's lip, mouth and genital lesions). UV radiation is
disease elsewhere. important aetiologically, but other factors also
play a role: smoking in lip and mouth cancers;
Sites of predilection. Light-exposed skin; may wart virus in genital lesions.
occur on non-exposed areas such as the trunk.
Clinical features. These may be very varied, typi-
Differential diagnosis. There is a superficial re- cally either:
semblance t o psoriasis (Fig. 9.12), but the sur- I a keratotic lump,
face scale is adherent rather than flaky. Removal 2 a rapidlygrowing polypoid mass (Fig. 9.14), o r
of scale leaves a glistening red surface that does 3 a cutaneous ulcer.
not bleed. As arsenic was used in the past t o SCCs are often surrounded by actinic
treat psoriasis, keep an eye out for Bowen's keratoses.
disease in elderly psoriasis sufferers.
Similar changes on one nipple should always Sites of predilection. Sun-exposed sites; SCCs
suggest the possibility of Paget's disease (Fig. also develop on the lips (Fig. 9.15), in the mouth
9.13); a biopsy should be performed as there is and on the genitalia.
always an underlying carcinoma.
Differential diagnosis. Keratotic lesions may
Treatment. Should be treated by excision, curet- closely resemble hypertrophic actinic kera-
tage, cryotherapy o r photodynamic therapy; toses.
very large areas may require radiotherapy.
Treatment. Biopsy of any suspicious lesion;
Invasive squamous cell carcinoma definitive treatment is by surgical removal o r
SCCs are locally invasive, and may metastasize radiotherapy.

Fig.9.13 Paget's disease o f the nipple.

88 Chapter 9: Benign and MalignantSkinTumours

Fig.9.14 A polypoid squamous cell


Fig.9.15 Squamous cell

carcinoma on the lip.
Specific tumours 89

Fig.9.16 Lentigo maligna.

Dysplastic/malignant pigmented actinic keratoses and simple

melanocytic tumours lentigines.
Lentigo maligna (or Hutchinson's
malignantfreckle) Treatment. Biopsy is essential; definitive treat-
The term 'lentigo maligna' describes a patch of ment is a matter of debate; excision is our
malignant melanocytes, in sun-damaged skin, preferred option because of recurrences with
which proliferate radially along the dermoepi- cryotherapy; in the very elderly it may be rea-
dermal junction and deep around hair follicles, sonable t o do nothing and follow the patient
often for many years. An invasive component carefully.
may develop at any time.
Malignant melanoma (MM)
Clinicalfeatures. A flat, brown area with irregular This is the most dangerous of the malignant
pigmentation. skin tumours. Melanomas, other than lentigo
maligna melanoma (see below), occur in a
Sites of predilection. Almost always on the face relatively younger age group than other skin
(Fig. 9.16). cancers. The incidence is rising rapidly, even in
temperate climates, probably due t o the in-
Differential diagnosis. Can be difficult t o crease in intermittent sun exposure that is now
distinguish from flat seborrhoeic keratoses, so fashionable. Rising standards of living have
90 Chapter 9: Benign and Malignant SkinTumours

~ Lendgomalignamelanoma
The appearance of a nodule ofinvasive
- Clinicalfeatures:
rapidly growinglumps (Fig.9.18)
1 melanomawithinalentigomaligna. occasionallywarty (vemcous melanoma)
or non-pigmented (amelanotic melanoma)
Suprhdal s p r e a d i n g m e l a n m (SSM) * Sites ofpredilection:

ThecommonestintheUK; thetumourhasa
- may occur anywhere
Differential diagnosis.
other rapidly growingtumours

* Clinicalfeatures:
mayitchorgive rise tomilddiscomfort
Rare intheUK,butis muchmore commonin
other countries (e.g.Japan); it is virmally the
onlytypeofmelanoma seeninnsian orllfro- I
* Sitesofpredilection: Caribbean patients
most frequentlyon thelegin women and * Clinical features: I
t h e m n k i n men,butmayaccur anywhere apigmentedpatchon the sole or palm oran
* Differentialdiagnosis area of subungual pigmentation
naeviintheyoung * Differentialdiagnosis:
flat seborrhodc keratoses in olderpatients canbeconfused with aviralwan
mustbe distinguishedfmm haematoma
Nodular melanoma Some MMs ariseinpre-eldstingmelanacc
! Thhetumourexhibitsaninvasivegr~ naevialthough estimatesofthefrequencyof '
i pattern from the outset this vary from 5% to oyer50%.
~ ~~ ~~~~~~~~

permitted more sunny holidays abroad (and seems that the incidence of this varies from
at home), during which the most important countryto country.
'activity' is sunbathing. There are four recognized patterns of malig-
Periods of exposure t o very strong sunlight nant melanoma (see table above).
with sunburn are particularly risky and there
is evidence that childhood sun exposure may Treatment ofmalignant melanoma
be important. Some melanomas arise in pre- In understanding MM and its treatment it is
existing melanocytic naevi (see Chapter 10). It important t o realize that the prognosis is re-

Fig. 9.17 Superficialspreading

Specific tumours 91

Fig. 9.18 Large nodular melanoma.

lated t o the depth of tumour invasion at first Encouraging early presentation

excision, regardless of the original type. Most The most effective way of improvingtreatment
centres measure invasion using a technique is t o increase public awareness of MMs and
known as the 'Breslow thickness' (Fig. 9.19). If thereby prompt people t o seek advice about
the tumour is less than 1.5 mm at first excision suspicious lesions. Many doctors now use a
5-year survival is about 90%; if the depth is over checklist (see p. 92).
3.5 mm this falls t o 40% or less.
All types of melanoma should therefore be Prevention of epithelial and
excised at the earliest possible opportunity. melanocytic malignancies
Radiotherapy and chemotherapy have little t o Both types of epithelial skin cancers, and
offer at present in curing the disease. There is melanomas, are more common in those who
some debate about how wide the excision mar- burn easily in the sun: those with fair skin, fair or
gins should be, but margins are becoming red hair and blue or green eyes (skin types I and
narrower. There is certainly no harm in initial Il-see Chapter 12). Melanomas are also more
narrow excision.The urgency is t o remove the common in individuals with many melanocytic
melanoma-further procedures can be con- naevi.
sidered later. It is logical therefore t o recommend that
In acral melanomait may be necessary t o per- those at risk avoid excessive sun exposure:
form a confirmatory biopsy before definitive I N o one should allow themselves t o be
treatment, which may involve amputation. sunburnt.
92 Chapter 9: Benign and MalignantSkinTumours

Fig. 9.19 Breslow thickness.

* isanexistingmolegemnglargeroranew
one growing?Afterpubeqmoles usuallydo
- Is thelesionlargerthan 1 cmindiameter?
* Is thelesionlnfiamedaristhere areddish
not grow.(Thissignessentiallyrefersto
inchildren (seeChapterl0))
Isthelesion bleeding.oozingorcmting?
Does thelesionitch orhurt?
* Doestheiesionhave animegularoutline?
Ordinaryrnolesarea srnooth.regular Any pigment& lesion,whethernemly arising
shape or alreadypresent,which exhibitsthreeor ~

* Is thelesionimegularlypigmented? more ofthe seven listed features, and

~ Particularly,isthere amixtureof shadesof espedallyoneofthe6rstthree,three, shouldbe
~ brown andblack? treated as highly suspicious.

2 It is best t o avoid midday sun (between Differential diagnosis. Difficult t o categorize

I I a.m. and 3 p.m .) or, at least, wear adequate without biopsy.
clothing and hats.
3 Sun-screens offering a high degree of Treatment. Wide excision is generally required;
protection should be used. in one tumour of this kind (dermatofibrosarcoma
Those who tan easily and those with brown protuberans) very wide indeed.
o r black skin need not take such Draconian pre-
cautions, but sun exposure in all children should Kaposi's sarcoma
be restricted. This malignant vascular tumour merits special
mention in spite of its rarity.'Classical' Kaposi's
Dermal malignant tumours sarcoma occurs inAshkenazi Jews and northern
Malignant sarcomas may develop in the skin. Italians. A much more aggressive form is seen in
Africans and in the acquired immunodeficiency
Clinical features. Indolent, slow-growing nodu- syndrome (AIDS).
les, which become fixed t o deeper tissues.
Clinical features. Purplish plaques and nodules.
Specific tumours 93

Fig.9.20 Areas of mycosis fungoides

(cutaneous T-cell lymphoma).

Sites of predilection. Legs in the classical form; Diferential diagnosis. Lesions can be confused
anywhere in the aggressive form. with eczema o r psoriasis.

Diferential diagnosis. Other vascular lesions. Treatment. Biopsy is essential but can be difficult
t o interpret-DNA phenotyping of cells may
Treatment. Biopsy; symptomatic treatment with be of value; definitive treatment varies with the
radiotherapy. stage, but includes radiotherapy, PUVA and
Lymphomatous involvement of the skin may be Extension from deeper tissues
secondary, for example in non-Hodgkin's B-cell and metastases
lymphoma. However, the skin may be the orig- Tumours of underlying structures, such as
inal site, especially in cutaneousTcell lymphoma breast, may invade the skin.The skin may also be
(often called'mycosis fungoides'). the site of metastatic deposits from internal
cancers such as bronchogenic carcinoma (see
Clinical features. Variable; some areas remain Chapter 19).
unchanged o r grow slowly for years; red, well-
circumscribed, scaly plaques and tumours
eventually develop (Fig. 9.20).
Introduction,94 Melanocytic naevi, 96
Epithelial and 'organaid'naevi, Vascularnaevi, 97

Ten thousondraw Iat oglonce (Wordsworth) portant: epithelia1 and organoid naevi, vascular
naevi and melanocvtic naevi.

Epithelia1 and'organoid'naevi
Naevi are extremely common-virtually
everyone has some. However, the word'naevus' These are relatively uncommon developmental
can give rise to confusion. Much of the difficulty defects of epidermal structures: the epidermis
is due t o the term being used in several different itself; hair follicles; sebaceous glands.There are
ways, in addition t o that outlined below. Some two important types, the epidermal naevus and
writers use the word without qualification for the sebaceous naevus.
the commonest cutaneous hamartoma, the
melanocytic naevus (see below). The word is Epidermal naevus
also applied t o lesions that are not congenital at Circumscribed areas of epidermal thickening
all, such as the 'spider naevus' (which should may be present at birth or develop during
be a'rpider telangiectasis').This is complicated childhood; many are linear. Very rarely, there
further by some true'naevi' beingcalled'moles' are associated central nervous system (CNS)
o r 'birthmarks'. Thus, a lump described as a abnormalities.
'mole' may be a melanocytic naevus, but may Becker's noevus is an epidermal naevus that
also be any small skin lesion, especially if presents as a pigmented patch first seen at o r
pigmented,while'birthmark'is accurateenough around puberty onthe uppertrunkor shoulder,
as far as it goes, but many naevi develop after and which gradually enlarges and may become
birth. hairy.
We use the word 'naevus' t o mean a cuta-
neous hamartoma (a lesion in which normal Sebaceous naevus
tissue components are present in abnormal Sebaceous naevi are easily overlooked at birth.
quantities o r patterns). This encompasses They begin as flat, yellow areas on the head and
'naevi' which are not actually present at neck which, in the hairy scalp, may cause Iocal-
birth, because the cells from which they arise ized alopecia. Later, the naevus becomes thick-
are. ened and warty, and basal cell carcinomas may
Any component of the skin may produce arise within it.These naevi are best excised dur-
a naevur, and they may be classified accordingly ingadolescence.
(Table IO.I).We need only discuss the most im-
Epithelia1and 'organoid' naevi 95

Table 10.1 A classification of naevi.

* Epidermalnaevus

* Sebaceousnawus
- Spitznaevus

Mehnorgtic Telangimatic

* Superli6dalcapillarynawus
* Deepcapillarynaevus
Rare telangiectaticdisorders


Sunonshalonaevus Connectivetissue
* Dysplasticnaevus * Mastcell

Fig. 10.1 Giant congenital melanocytic

96 Chapter 10: Naevi

Melanocytic naevi

The commonest naevi are formed from

melanocytes that have failed t o mature o r mi-
grate properly during embryonic development.
Almost all of us have some. Look at your own
skin, o r that of an attractive classmate, t o see
typical examples!
It i s convenient t o categorize melanocytic
naevi by clinical and histopathological features,
because there are relevant differences (Table
10.1). The first is whether they are present
at birth (congenital) o r arise later (acquired).

Congenital melanocytic naevus
One per cent of children have a melanocytic
naevus at birth.
These vary from a few millimetres to many
cenumetres in diameter. There is a rare, but
huge and grossly disfiguring variant, the'giant'
congenital melanocytic o r 'bathing trunli nae-
vus (Fig. 10. I). Fig. 10.2 Mangolianbluespot.
Congeniral melanocytic naevi are more
prone t o develop melanomas than acquired le-
sions. particularly the giant type. Prepubertal
malignant melanoma is extremely rare, but
Acquired melanocytic naeuus
neal-ly always involves a congenital naevus.This
A melanocytic naevus is'acquired' if it develops
leads to a paradox: small, low-risl< naevi are
during postnatal life, a phenomenon that is so
easily removed but larger lesions with higher
common as t o be'normal'. Mostonly represent
malignant potential require extensive, even mu-
a minor nuisance, and'beauty spots' were once
tilating, surgery. Each case must be judged on its
highly fashionable.
own merits, and decisions must involve thefam-
Thetirstthingco understand is thateach nae-
ilyand thechild.
vus has its own life history.This will make the
terms applied t o the different stages in their
Mongolian blue spot
evolution clearer (Fig. 10.3).
Almostall children of Mongoloid extractionand
Thelesion (Fig. 10.4) is first noticed when lm-
many Indian and Afro-Caribbean babies are
mature melanocytes begin t o proliferate at the
born with a diffuse blue-blacl< patch on the
dermoepidermal junction (hence 'junctional').
lower back and buttocks (Fig. 10.2).There are
After a variable period of radial growth, some
melanocytes widely dispersed in the dermis
cells migrate vertically into the dermis ('com-
(the depth is responsible for the colour). The
pound'). Eventually all melanocytic cells are
area fades as the child grows, but may persist
within the dermis ('intradermal'). Different
indefinitely. Unwary doctors have mistaken
melanocytic naevi will be at different stages of
Mongolian blue spots for bruising, and accused
development in the same individual.
parents of baby-battering.
Most melanocytic naevi appear in the first
Vascular naevi 97

Fig. 10.3 The phases afthe

acquired melanocytic naevus:
(a)junctional; (b)compound;
(c) intradermal.These stages
are partofa continuum, and
eachlasts avariable time.

melanocyric naevi is a risk factor for melanoma;

and melanocytic naevi can be confused with
Any melanocytic lesion which behaves oddly
should be excised for histology, but remember
that, by detinition, all melanocytic naevi grow
at some stage. Growth alone therefore is
not necessarily sinister, especially in younger
individuals. Most naevi undergoing malignant
change show features outlined in Chapter 9
(see p. 92); b u t . . . ifin doubt'lop itout'!
There are several variants of the acquired
melanocytic naevus (see p. 98).

Fig.10.4 Acquiredmelanacyticnaevus.
Vascular naevi

Vascular blemishes are common. Some present

20 years of life, but may continue t o develop relatively minor problems, whereas others are
well into the forties. They are initially pig- very disfiguring.Thc classification of these naevi
mented, often heavily, but later may become is confusing and by no means uniform. We have
pale, especially when intradermal. Most disap- adopted a simple approach based on both clini-
pear altogether: very few octogenarians have cal and pathological features.
Their importance (apart from cosmetic) is Telangiectatic naevi
threefold: some malignant melanomas develop Superficial capillary naeuus
in a pre-existing naevus (the chances of this These pinlc. flat areas, composed of dilated
in any one lesion are infinitesimally small); capillaries in the superficial dermis (Fig. 10.7).
the possession of large numbers of acquired are found in 50% of neonates.The commonest
98 Chapter 10: Naevi

Sutmn'shalonaevus increase in melanoma ('dysplastic naevus

A white ringdwelops around an otherwise syndrome')
m i c d melanoytic naevus; thelesion
maybecomered and disappear(Fig. 10.5). Bluenawus
Thisis animmuneresponseofno Thecharacteristicslate-blue colour(fig. 10.6)
sinister significanceand unknown is due to deep dermal melanocytes;they are
cause most commonontheexuemities,head and
Some lesions lookunusual andlorhave Spitrna-
unusual histopathologicalfeatures; this Sometimescalled juvenile melanoma rthe
may affectjust one or two naevi,but some prefix'benign';benignlesion of children
people have many; such individuals may he which has a characteristicbrick-red colour;
partofa pedigreein which thereis a striking SpiQ naevi can beconfusedhistologically
with malignant melanoma

sites are the nape of the neck ('salmon patches' Deep capillary naevi are less common but
o r 'stork marks'), the forehead and glabellar more cosmetically disfiguring than superficial
region ('stork marks' again) and the eyelids lesions. Most occur on the head and neck and
('angel's kisses'). Most facial lesions fade, but are usually unilateral, often appearing in the ter-
those on the neck persist, often hidden by hair. ritory of one o r more branches of the trigeminal
nerve (Fig. 10.8). They may be small o r very
Deep capillary naeuus extensive.
'Port-wine stains' o r 'port-wine marks' are A t birth the colour may vary from pale pink
formed by capillaries in the upper and deeper t o deep purple, but deep lesions show no
dermis. The deeper component gradually ex- tendency t o fade. Indeed they may darken
tends during life. with time, and often become progressively

Fig. 10.5 Sutton's

'halo' naevus.
Vascular naevi 99

If a deep capillary naevus is relatively pale it

may be difficult t o distinguish from the superfi-
cial type, especially in the neonatal period. It is
therefore important t o give a guarded initial
prognosis and await events.

Angiomatous naevi
In some accounts these lesions are classified
with capillary naevi, whereas in others they are
termed 'cavernous'. Most authorities acknowl-
edge that both capillary-derived elements and
larger, so-called 'cavernous' vascular spaces are
usually involved.

Strawberry naevus
Strawberry naevi arise very shortly after
birth. They may appear anywhere, but have a
predilection for the head and neck and the
napkin area (Fig. 10.9). Most are solitary, but
occasionally there are more. The lesion
grows rapidly t o produce a dome-shaped,
red-purple extrusion which may bleed if
traumatized. The majority reach a maximum
size within a few months.They may be large and
Spontaneous resolution is the norm, some-
times beginningwith central necrosis, which can
thickened. Lumpy, angiomatous nodules may look a1arming.A~a rule of thumb, 50% have re-
develop. solved by the age of 5 and 70% by age 7. Some
only regress partially, and a few require plastic
These lesions are most unattractive, and
patients often seek help. The newer types of surgical intervention.
The management, in all but a few, is expec-
lasers can produce reasonable results, and a
tant. It is useful t o show parents a series of
range of cosmetics can be used as camouflage.
pictures of previous patients in whom the lesion
There are three important complications.
has resolved
Specific indications for intervention:
I If breathing o r feeding is obstructed.
2 If the tumour occludes an eye-this will lead
t o blindness (amblyopia).
* An arsaciatedinmnanialvascular 3 If severe bleeding occurs.
malformation may result in fits,long-tract
signs and mental retardation.Thisisthe
4 If haemorrhage within a large tumour leads
SmrgeWeber syndrome t o consumption coagulopathy (Kasabach-
Congenitalglaucoma rnayormrinlaions ~
Merritt syndrome).
involvingtheareaof theophthalmicdivision 5 If the tumour remains large and unsightly


of the uigeminal n e m after the age of 10.

Growth ofunderlyingtissuesmaybe Treatment of complications I 4 is initially
with high-dose prednisolone, which may pro-
limbs- haeman~ectatichvpemophy
duce marked shrinkage. If this fails, and in the
100 Chapter 10:Naevi

Fig. 10.7 Superficialcapillary naevus.

Fig. 10.8 Deep capillary naevus

('port-wine stain').
Other naevi 101

Fig. 10.9 Cavernous

haemangiama on the face.

fifth complication, complex surgical interven- Other naevi

tion may be required.
Naevi may develop from other skin elements.
Rare angiomatous naeui including connective tissue, mast cells and fat.
Rarely, infants are born with multiple straw- For example, the cutaneous stigmata of ruber-
berry naevus-lil<e angiomas of the skin and ous sclerosis are connective rissue naevi (see
inrernal organs. This is known as neonotal Chapter I I), and the lesions of urcicaria pig-
ongiomatosis and the prognosis is often poor. mentosa are mast cell naevi.
Inherited Disorders

The ichthyoses, 102 Hereditary haernonhagic Xerodermapigmentasum, 107

Collodionbaby, 103 telangiectasia(Oslcr- Acrodem~atitisenteropathica,
Palmo-plantarkeratoderma. 103 Weber-Rcndu disease),l05 107
Daricr'sdisease (keratosis Basalcellnaevus syndrome Angiokeratoma corparis
follicularis).l04 (Garlin'ssyndrome).l05 diffusum (Anderson-Fabry
Epidermolysisbullosa,104 Cardner'ssyndrame,106 disease),107
Ehlers-Danlos syndrome, l04 Anhidroticect~demal lncontinenliapigmenti.108
lberous sclerosis. l04 dysplasia, 106 Chromosomalabnormalities,
Ne~r0fibmmato~i~.104 Pseudaxanthomaelasticum. 108
Peulz-Jcghers syndrome. 105 107

There is only one more beautiful thing than a Autosomal dominant ichthyosis
fine healthyskin,and that is a rareskin disease. (ichthyosis vulgaris)
(Sir ErasmusWilson. 180944) This is the commonest, and is often quite
A number of skin conditions are known to be in- mild. The scaling usually appears during early
herited. Many are rare, and will therefore only childhood.The skin on the trunk and extensor
be mentioned briefly.There have been maiorad- aspects of the limbs is dry and flaky, but the
vances in medical genetics in recent years, and limb flexures are often spared. Dominant
the genes responsible for many disorders have ichthyosir is frequently associated with an
been identified and their roles in disease atopic constitution.
Several diseases in which genetic factors play X-linked ichthyosis
an important part, such as atopic eczema, pso- This type of ichthyosis only affects males. The
riasis, acne vulgaris and male-pattern balding. scales are larger and darler than those of dom-
are described elsewhere in the bool<. inant ichthyosis, and usually the trunl<and limbs
are extensively involved, including the flexures.
Corneal opacities may occur, but tliese do not
The ichthyoses interfere with vision. Affected individuals are
deficient in the enzyme steroid sulfatase-the
The term ichthyosis is derived from the Greek result of abnormalities in its coding gene.The
ichthys, meaningfish,astheskin has been likened majority of patients have complete or partial
to fish scales. The ichthyoses are disorders of deletions oithe steroid sulfatasegene.
keratinization in which cheskin is extremely dry Both X-linked ichthyosis and autosomal
and scaly (Fig. I I.I). In the majority of cases the dominant ichthyosis improve during the sum-
disease is inherited, but occasionally ichthyosis mer months.
may be an acquired phenomenon, for example
in association with a lymphoma.There are sev- lchthyosiformerythroderma
eral types of ichthyosis, which have different A bullous form of this condition is dominantly
modes of inheritance. inherited,andanon-bullour form recersively in-

Palmo-plantar keratoderma 103

herited. In both, the skin is scaly and erythema- helpful. The more severe types of ichthyosis
tous, and often has an offensive odour. often require oral retinoid therapy.

Treatment consists of regular use of emollients Collodion baby
and bath oils. Urea-containing creams are also
This term is applied to babies born encased in a
transparent rigid membl-ane resembling colio-
dion (Fig. I I .2).The membrane cracla and peels
offafterafew days. Some affected babies havean
underlying ichthyotic disorder whereas in oth-
ers the underlying skin is normal. Collodion ba-
bies have increased transepidermal water loss.
and it is important that they are nursed in a
high humidicy environment and given additional

Palmo-plantar keratoderrna

Several rare disorders are associated with mas-

sive thicl<eningof the horny layer of the palms
and soles.The commonest type is dominantly
inherited. Many medical texts mention an asso-
ciation of paimo-plantar lkeratoderma (tylosir)
with carcinoma of the oesophagus-in fact this
is extremely rare.

Fig. 11.1 ichthyosis

Fig. 11.2 Collodion baby

104 Chapter I I: Inherited Disorders

Ehlers-Danlos syndrome

There are a number of distinct variants of this

condition, all of which are associated with
abnormalities of collagen, principally defective
production.The most common are dominantly
inherited, but all types of Ehlers-Danlos
syndrome are rare. Typical features are skin
hyperextensibility and fragility, and joint hyper-
mobility In certain types there is a risk of rup-
ture of major blood vessels because of deficient
collagen in thevessel wall.

Tuberous sclerosis

This is a dominantly inherited disorder, but

many cases are sporadic and represent new
mutations. There are hamartomatous malfor-
mations in the skin and internal organs. Charac-
teristic skin lesions include numerous pink
papules on the face (Fig. 1 1.4) (originally called
adenoma sebaceum), which are hamartomas of
Fig. 11.3 Darier'sdiseasc.
connective tissue and small blood vessels (an-
giofibromas): the shagreen patch on the back (a
connective tissue naevus); periungual fibromas
Darier's disease
(Fig. 1 1 .S):and hypopigmented macules (ash leaf
(keratosis follicularis)
macules) which are best seen with the aid of
Wood's light. The hypopigmented macules are
This is a dominantly inherited abnormality of
often presenrat birth, butthe facial lesions usu-
Ikeratinization which is usually first evident in
ally first appear at the age of 5 or 6.Affected in-
late childhood o r adolescence.The characteris-
dividuals may be mentally retarded and suffer
tic lesions of Darier's disease are brown follicu-
from epilepsy. Other features include retina1
lar lkeratotic papules, grouped together over
phal<omas, pulmonary and renal hamartomas,
the face and neclc the centre of the chest and
and cardiac rhabdomyomas.
back, the axillae and the groins (Fig. 1 1.3).The
nails typically show longitudinal pink o r white
bands, with\/-shaped notches at the free edges.
Thereare usually numerous wart-lilke lesionson
the hands (acrokeratosis verruciformis).
There are two main forms of neurofibromato-
Darier's disease responds t o treatment with
sis-type I (NF-I) (von Recldinghausen's dis-
ease) and type 2 (NF-2), both of which are
of autosomal dominant inheritance. NF-I is
characterized by multiple cafe-au-lait patches.
Epidermolysis bullosa
axillary fieclding (Crowe's sign), numerous
neurofibromas (Fig. 11.6) and Lisch nodules
This group of hereditary blistering diseases is
(pigmented iris hamartomas). Other associated
described in Chapter 14.
Basal cell naevus syndrome (Gorlin's synd~ome) 105

Fig. 11.4 Facialangiofibromas

in tuberous sclerosis.

neuromas, as well as other central nervous sys-

tem tumours, and cataracrs.

Peutz-Jeghers syndrome

in this rare dominantly inherited syndrome

there are pigmented macules (lentigines) in the
mouth, on the lips, and on the handsand feet, in
association with multiple hamal-tomatous in-
testinal polyps.

Hereditary haemorrhagic
telangiectasia (Osler-Weber-
Rendu disease)

This is a rare, dominantly inherited disorder in

which numerous teiangiectases are present on
the face and lips, nasal, buccal and intestinal mu-
cosae. Recurrent epistaxes are common, and
Fig. 11.5 Periungualfibrornain tuberous there is also a risk of gastointestinal haemor-
sclerosis. rhage.There is an association with pulmonary
and cerebral arteriovenous fistulae.

abnormalities include scolioris, an increased

risk of developing intracranial neoplasms. Basal cell naevus syndrome
particularly optic nerve glioma, and an (Gorlin's syndrome)
increased risk of hypertension associated with
phaeochromocytoma o r fibromuscuiar hyper- Gorlin's syndrome is a dominantly inherited dis-
plasia of the renal arteries. order in which multiple basal cell carcinomas
NF-2 is characterized by bilateral acoustic on the face and trunk are associated with
106 Chapter I I : Inherited Disorders

Fig. 11.6 Von Recklinghausen's


characteristic palmar pits, odontogenic Ikerato-

Anhidrotic ectodermal dysplasia
cysts of the jaw, calcification of the falx cerebri
and sl<eletalabnormalities.
This is a rare condition in which the eta-ine
sweatglands are absent o r markedly reduced in
number, the scalp hair, eyebrows and eyelashes
are sparse, and the teeth are widely spaced and
conical.The absence of sweating interferes with
This condition is also dominantly inherited.
temperature regulation, and this may lead t o hy-
Affected individuals have multiple epidermoid
perthermia in a hot environment. Anhidrotic
cysts, osteomas, and large bowel adenomatous
ectodel-mal dysplaria is inherited as an X-linl<ed
polyps which have a high risk of malignant
recessive trait.
Angioleratoma corporis diffusum (Anderson-Fabry disease) 107

Fig. 11.7 'Pluckedchicken'

appearanceof the skinin
pseudoxanthorna elasticum

Pseudoxanthoma elasticum Acrodermatitis enteropathica

This inherited disorder of connective tissue, In this recessively inherited disorder there is
which has several modes o f inheritance, affects defective absorption of zinc. The condition is
elastic tissue in the dermis, blood vessels usually manifest in early infancy as exudative
and Bruch's membrane in the eye. The sl<in eczematous lesions around the orifices, and on
of the necl< and axillae has a lax 'plucked the hands and feet. Affected infantz also suffer
chiclen' appearance of tiny yellowish papules from diarrhoea. Acrodermatitis enteropathica
(Fig. 1 1.7). Retinal angioid streaks, caused by can be effectively treated with oral zinc
ruptures in Bruch's membrane, are visible on supplements.
fundoscopy. The abnormal elastic tissue in
blood vessels may lead t o gastrointestinal
haemorrhage. Angiokeratoma corporis
diffusum (Anderson-Fabry
Xeroderma pigmentosum
This condition is the result of an inborn error of
Ultraviolet (UV) damage t o epidermal D N A is glycosphingolipid metabolism. It is inherited in
normally repaired by an enzyme system. In an X-linked recessive manner. Deficiency ofthe
xeroderma pigmentosum, which is recessively enzyme alpha-galactosidaseA leads t o deposi-
inherited, this system is defective, and UV tion of ceramide trihexoside in a number of tis-
damage is not repaired.This leads t o the early sues, including the cardiovascular system, the
development of skin cancers. Basal cell carcin- kidneys, the eyes and peripheral nerves. The
omas, squamous cell carcinomas and malignant skin lesions are tiny vascula~.angiokeratomas
melanomas may all develop in childhood. In which are usually scattered over the lower
some cares there is also gradual neurological trunk, buttocks,genitaliaand thighs.Arrociated
deterioration caused by progressive neuronal features caused by tissue deposition of the lipid
loss. include thefollowing.
108 Chapter I I: Inherited Disorders

ocular, skeleml, denca and central nervous sys-

tem abnormalities.

* Prematureischaemicheart disease
* Rendfailure Chromosomal abnormalities
Swerepain andparaesthesiaeinthehands

and feet
Some syndromes caused by chromosomal ab-
normalities may haveassociated dermatological

lncontinentia pigmenti
An X-linked dominant disorder, incontinentia -
. ..

. - .. . .
pigmenti occurs predominantly in female in-
fants,as itis usually lethalin utero in males. Linear Down's syndrome:increased incidenceof

limbs at birth, o r soon thereafter.The bullaeare

gradually replaced by warrylesions, and these in
bullous lesions are present on the trunk and ' alopeciaareata
mmer's syndrome:primalylymphoedema
Klinefelter'ssyndrome:premature venous
turn are eventually replaced by streaks and i x~~syndmme:premaNrevenous
whorls of hyperpigmenrarion. lncontinentia pig- u1ceration;proncwdevelopswere
menti is frequently associated with a varlecy of nodulocysticacne
~~~~~ .
Pigmentary Disorders

Introduction: normal Hypopigmentation,110 Hyperpigmentation,112


Bold was her face,and fair, and red ofhew, Normal pigmentary mechanisms have al-
(Chaucer, Thewife ofBath) ready been outlined in Chapter I. Humans have
a rather dull range of natural colours when
The complexlan ofthe skin and the colour ofthe
compared with peacocks o r parrots: normally
hair correspondto the colour ofthe moisture
only shades of brown and red. Brown is due t o
which the flesh attracts-white,or red,or black.
melanin, the intensity varyingfrom almostwhite
(no melanin) t o virtually jet-black (lots). The
genetics of melanin pigmentation is autosomal
Red is a bonus: only some people can pro-
pigmentary mechanisms
duce'phaeomelanin'. Red is much commoner in
some races (e.g. Celts) than in others (e.g.
Our sldn colour is important, and there are
many references t o it in prose and poetry. We
Most of human skin pigment is within
all note skin colour in our initial assessment
keratinocytes, having been manufactured in
of someone, and sldn colour has been used
melanocytes and transferred ln'melanoromes'.
to justify all manner of iniustices. Any depar-
There are racial differences in production, dis-
ture from the perceived norm can have
tribution and degradation of melanosomes, but
serious psychological effects and practical
not in the number of melanocytes (see Chapter
I).Thereare, however, importantgenetic differ-
A number of factors give rise to our skin
ences in the ability t o respond t o uh-aviolet ra-
diation, conventionally called'rkin types'.

* Haemoglobin
* 7).peln-bumsoccasionally,tanseasily
7).pe N-neverbums.tans easily
* Endogenouslyproducedpigments(e.g.
* WeV-genetically brown (e.g.Indian) or
Thelastm are themostimportantin
~ Mongoloid
i * 7)rpeVI-geneticallyblack(Congoidor
j Negroid)
dictatingourbasicskincolour . . --
1 10 Chapter 12: Pigmentary Disorders

The first response t o UV radiation is an zyme tyrosinase may be absent (tyrosinase-

incl-eased distribution of melanosomes. This negarive) leading to generalized white sl<inand
rapidly increases basal layer pigmentation-the hair, and red eyes (the iris is also depigmented).
'sun tan'. If stimulation is quickly withdrawn, as Vision is usually markedly impaired, with
typically happens after 2 weeks in the Mediter- nystagmus.
ranean, the tan fades rapidly and peels off with In tymrinase-positive albinism (where the
normal epidermal turnover. If exposure is pro- enzyme tyrosinase is defective), the clinical pic-
longed, melanin production is stepped up more ture is not as severe. and colour may gradually
permanently Tanning represents the skin's ef- increase with age. However, skin cancers are
forts to offer protection from the harmful ef- very common in both forms. Albinism also illus-
fects of UV radiation, such as premature ageing trates the importance of colour: in some
and cancers. societies albinos are rejected and despised, in
We shall now look at states in which these others they are irevered as special.
pigmentary mechanisms appear to be abnor- The biochemical defect in phenyll~etonuria
mal, leading t o decreased (hypo-) o r increased results in reduced tyrosine, the precursor of
(hyper-) pigmentation. melanin, and increased amounts of phenylalan-
ine (which inhibits tymrinase).There is agener-
alized reduction in pigmentation of skin, hair
Hypopigmentation and eyes.
One of the cardinal signs of tuberous sclerosis
Among the most important causes of (epiloia) is hypopigmented macules. These are
hypopigmentation are: often lanceolate (ash-leaf shaped), but may as-
sume bizarre shapes. They are often the first
signs of the disease. Any infant who presents
with fits should be examined under Wood's
light (the macules can be seen more easily-see
also Chapter 2). Identical localized pale areas
may occur withoutany otherabnormality, when
* Albinism

* Phenylketonvria
they are termed hypochromicnaevi.

Acquil-ed hypopigmentation Is common and, in

darker sldn, may have a particular stigma.This

* Pityriasis(tinea)versicolor
is partly because the cosmetic appearance of
patchy hypopigmentation is much worse, but
also because white patches are inextricably
* Fityriasis alba linked in some cultures with leprosy. Histori-
LichensclerPwetatmphinu cally all white patches were probably classified
Drugs and chemicals: as leprosy: Naaman (who was cured of'leprosy'
occupationalleuccdema after bathing in the Jordan (2 Kings 5: 1-14))
self.inflictdiamgenic probably had vitiligo (see below).
Vitiligo is the most important cause of patches
of pale sldn.The skin in vitiligo becomes depig-
Congenital mented and not hypopigmented. although dur-
Some individuals are born with generalized o r ing progression this is not always complete.
localized defects in pigmentation. Characteristically there is complete loss of
Albinism and phenylketonuria are due t o de- pigment from otherwise entirely normal skin
fects in melanin production. In albinos, the en- (Fig. 12. I). Patches may be small, but commonly
Hypopigmentation III

Fig. 12.1 A typical patch of


become quite large, often with irregular out- The organism causing pityriasis versicolor (see
lines. Depigmentation may spread t o involve Chapter 4 ) secretes azelaic acid.This results in
wide areas of the body. Although vitiligo can hypopigmentation, most noticeably after sun
occur anywhere, it is often strikingly symmetri- exposure.
cal, involving the hands, perioral and periocular Pityriasis alba (a low-grade eczema) is a very
skin. common cause of hypopigmentation in chil-
The pathophysiology is poorly understood. dren, especially in darker skins. Pale patches
In early patches melanocytes are still present, with a slightly scaly surface appear on the face
but produce no melanin. Later, melanocytes dis- and upper arms (Fig. 1 2.2). The condition usu-
appear completely, except deep around hair fol- ally responds (albeit slowly) t o moisturizers, but
licles.Vitiligo may be an autoimmune process: may require mild topical steroids.The tendency
there is an increase in organ-specific autoanti- appears t o clear at puberty.
bodies (as in alopecia areata, with which vitiligo Lichen sclerosus et atrophicus (see Chapter 15)
may coexist). usually affects the genitalia. O n other sites it is
Treatment is generally unsatisfactory.Topical sometimes called'white spot disease'.
steroids have their advocates, and PUVA can be Drugs and chemicals may cause loss of skin pig-
successful. Cosmetic camouflage may be help- ment.These may be encountered at work, but a
ful. Sun-screens should be used in the summer, more common source is skin lightening creams,
because vitiliginous areas will not tan. sold especially in areas with Afro-Caribbean and
In some, particularly children, areas may Asian populations. The active ingredient is gen-
repigment spontaneously. This is less common erally hydroquinone, which can be used thera-
in adults and in longstandingareas. Repigmenta- peutically (see below).
tion, when it does happen, often begins with Many inflammatory skin disorders may pro-
small dots coinciding with hair follicles.A similar duce secondary o r postinflammatory hypopig-
appearance occurs in Sutton's halo naevus (see mentation, due t o a disturbance in the integrity
Chapter 10). of the epidermis and its melanin production
Some of the stigma associated with hypopig- system: both eczema and psoriasis often leave
mentation is because tuberculoid leprosy is an- temporary hypopigmentation when they re-
other cause. The (usually solitary) patch of solve. However, inflammation can destroy
hypopigmented skin also exhibits diminished melanocytes altogether: in scars, after burns,
sensation. Pale patches may also be seen in the and in areas treated with cryotherapy (it is the
very earliest stages: so-called 'indeterminate' basis of 'freeze-branding').
1 12 Chapter 12: Pigmentary Disorders

Hyperpigmentation i s prominent in neurofibro-
matosis: cafe-au-lait marks (Fig. 12.3) and axil-
lary frecklingare common. Speckled lenriginous
pigmentation is seen around the mouth and on
the hands in the Peutz-Jeghen syndrome, and
similar but more widespread lentigines may ac-
company a number of congenital defects in the
LEOPARD syndmme (Lentigines, Electrocardio-
graphic abnormalities, Ocular hyperteiorism.
Pulmonary stenosis. Abnormalitiesof thegeni-
talia. Retardation of growth and Deafness.).
Fig. 12.2 Pityliasis albaan thecheek. lncontinentiapigmenti is a rare congenital dis-
order which causes hyperpigmentation in a
whorled pattern, often with blisters and hyper-
Hyperpigmentation keratotic lesions, and sometimes other con-
genital abnormalities.
As with hypopigmentation, there are many
causes of increased skin pigmentation, including Acquired
excessive production of melanin, o r the deposi- Urticaria pigmentoso is most common in chil-
tion in the skin of several other pigments, such dren, but may affect adults. There is a wide-
as beta-carotene, bilirubin, drugs and metals. spread eruption of indistinct brown marks
The maior causes are as follows: which urticate if rubbed.The disorder is due t o
abnormal numbers of dermal mast cells.
Chloasma, o r melasma, i s commoner in
women than men. A characteristic pattern of
hyperpigmentation develops on the forehead.
cheeks and chin (Fig. 12.4). Provoking factors
include sunlight, pregnancy and the oral con-
traceptive pill, but chloasma may occur sponta-
neously. Treatment is difficult. Avoidance of
precipitating factors (especially sunlight and
oestrogens) may help. Topical hydroquinone
preparationsaresomedmes used.
Various drugs and chemicals can cause cuta-
neous hyperpigmentation (see Chapter 21).
In postinflammotory hyperpigmentation dis-
Hyperpigmentation 1 13

Fig. 12.3 CaM-au-laitpatches in


ruption of the lower layers of the epidermis re-

sults in deposition of melanin granules in the
dermis (pigmentary incontinence). Many skin
disorders do this, particularly in pigmented skin,
but lichen planus is particularly troublesome.
There is no useful treatment, but the pigmenta-
tion gradually fades with time.
Hyperpigmentation is an important physical
sign in several systemic diseases:
I Addison's disease-the changes are most
marked in the skin creases, in scratch marks and
the gums.
2 Renalfailure -may cause a muddy-brown skin
3 Haemochromatosis-causes a deep golden-
brown hue, diabetes and liver disease.
4 Some chronic liver diseases-result in deep
Beta-carotene (a yellow pigment) accumulates
harmlessly in the skin in some normal individ-
uals who ingest large amounts of carrots and or-
ange juice (rich sources). The colour is most
marked on the palms and soles. Similar deposi-
Fig. 12.4 m i c a 1 chloasma. tion is seen in some patients with myxoedema
and pernicious anaemia.
Another important, although rare, cause of
acquired hyperpigmentation is acanthosis nigri-
cans. This may o r may not be associated with a
systemic disease (see Chapter 19).
Disorders of the Hair
and Nails

Introduction, 114 Hair abnormalities. 114 Nail abnormalities, 118

Ifo woman hove long hair, it is o glory to her. Changes in physical properties
(St Paul ( l Corinthians. I I : 15)) of scalp hair
Common physical changes which are seen in
The hairtakes root in the headot thesome time
hair are asfollows:
as the noilsgrow. (Hippocrates)


Hippocrates (see above) clearly knew that hair

and nails were intimately connected, but there Pigmentation
are many conditions which affect one o r the * Geneticdiseases.e.g.albinirm,
other alone.We will deal with abnormalities of
hair first and then nail disorders, but there will
be some overlap. pathological.e.g.pmidous anaemia
Abnormalities of hair and nails may be the
result of:
I Local factors.
* Ageing
Alopeaa areata
2 Generalized skin disease.
3 Systemicdisease.
- Brittleness
* Curlineso
Hair abnormalities

Hair is important psychologically. Disturbances

in growth o r physical characteristics, even of Change in colour
minor degree, may bevery upsetting: only Kojak Greying of the hair, whether premature or not.
really liked being bald! Remember that the dir- is permanent, including, usually,thewhite hair in
tress caused is not necessarily proportionate t o scalp vitiligo. Regrowing hair in alopecia areaca
the severity apparent t o an observer. (see below) is often white initially, but often
Patients present with three main hair repigments later.
I Changes in physical properties, such as Textural abnormalities
colour O r texture. Brittleness or coarseness may accompany
2 Thinning or loss of hair. hair thinning in hypothyroidism and in iron
3 Excessive hair growth, including growth in deficiency (see below). Hair may also become
abnormal sites. 'lack-lustre' from hairdressing techniques
Hair abnormalities 1 15

('back-combing', bleaching and drying). In men, Acquired disorders

hair may become curly in the early stages of an- Patients most commonly seek advice about hair
drogenetic alopecia (see below). loss when it is from the scalp, although other
areas may be affected. The most effective ap-
Scalp hair loss proach t o the diagnosis of acquired scalp hair
Congenital disorders loss is t o consider:
Abnormal scalp hair loss is a feature of some I whether the changes are diffuse o r circum-
congenital disorders. scribed, and
2 t o assess the state of the scalp skin.
When combined with some knowledge of
the disorders mentioned below, a preliminary
diagnostic assessment can be made.
1 -- Ectcdermaldysplasias
Telogen effluvium is often triggered by major
illness, operations, accidents o r other stress.

. MonileW
A large percentage of hairs suddenly stop
growing, enter the resting o r 'telogen' phase,
and fall out about 3 months later. Therefore,
* Dioordemofaminoaadmeebolism ask whether there has been any major upset
in the appropriate period. Pull gently on hairs
on the crown o r sides, and several will come
out easily. W i t h a hand lens the bulb looks
much smaller than normal. Telogen effluvium
settles spontaneously, but can unmask andro-
genetic alopecia (see below), and some patients
Very few of these conditions are amenable t o
find their hair never returns completely t o
treatment, but they require careful assessment,
often including microscopic examination of hair
Appropriate tests will exclude the important
S hafts.
systemic diseases listed below, and correct
treatment may restore hair growth.

Telogen effluvium
* Thyroid disease
- %action

* Irondeficiency HairhwithabwrnulsaIpakJn
wlthout m n i n g

* Systemiclupuserythematom
Alopecia totalis
* Severepsoriasisorseborrhoeicdermatitis
nnea capitis (seeChapter4)

Umh mm'ng

* Liehenplanus
Circumsmii hairlossarithnonmldp Pseudopelade

* Alolopeciaareata .
* Cicatricialpemphigoid
1 16 Chapter 1 3: Disorders of the Hair and Nails

Many drugs can induce hair loss. Until recently there was no known treat-
ment, but there is some evidence that early use
of topical minoxidil may help both men and
women, and there are high hopes of a new

--Cytotoric agents
generation of selective antiandrogenic agents.

- Anti-thymid agents,especiallythiouradl
* VitaminAanalogues
Circumscribed hair loss with normal
scalp skin
Alopecia areata
The cause of this disorder is unknown but it is
All of these processes can be confused with probably an autoimmune process. Organ-
alopecia areata (see below) when the latter is specific autoantibodies (to thyroid, adrenal o r
widespread and rapidly progressive. gastric parietal cells) are often found in the pa-
Androgenetic alopecia (or common balding) tients' sera.
occurs in both men and women. It is due t o the
effects of androgens in genetically susceptible History. One o r more areas of baldness sud-
individuals. denly appear on the scalp, in the eyebrows,
In men, the process may begin at any age after beard o r elsewhere. It is most common in child-
puberty; however, it is much more common hood o r early adult life, although periodic recur-
from the thirties onwards, and by age 70 80% rences throughout life may occur.
show some hair loss. Hair is usually lost first
at the temples andlor on the crown, but there Examination. Patches are typically round o r oval
may be complete hair loss, sparing a rim at the (Fig. 13.1); the skin usually appears completely
back and sides.Terminal hairs become progres- normal, although there may be mild erythema; a
sively finer and smaller, until only a few vellus number of areas may develop next t o each
hairs remain.The extent and pace of this varies other, giving rise t o a moth-eaten appearance;
widely. close examination of the edge of a patch of
In women the process is slower and less alopecia areata reveals the pathognomonic fea-
severe, but causes much distress. Up t o half of ture -'exclamation mark hairs' -short hairs
all women have mild hair loss on the vertex which taper towards their bases (Fig. 13.2).
by age 50, and in some more severe thinning
occurs. There may be accompanying hirsutism Prognosis. Most patches regrow after a few
(see below). weeks, although further episodes can occur; ini-

Fig. 13.1 A typical patch of

alopecia areata.
Hair abnormalities 1 17

Fig. 13.2 The edge of the area

seen in Fig. 13.1.Exclamation
mark hairs are visible at the

Fig. 13.3 Traction alopecia.

tial hair growth may be white; occasionally, the In trichotillomania, hair is pulled, twisted o r
process spreads and may become permanent- rubbed out, and affected site(s) are covered in
if this state involves the whole scalp it is broken hairs of different lengths.There may be
termed alopecia totalis and if the whole body is psychologicalfactors (see Chapter 20).
affected, the name alopecia universalis is applied.
The nails may be affected in severe cases (see Hair loss with abnormal scalp skin
below). Psoriasis, seborrhoeic dermatitis and other in-
flammatory processes can cause temporary
Treatment. This is difficult, but intralesional hair loss: an important cause is tinea capitis (see
steroids may help, and topical sensitizers such as Chapter 4).
diphencyprone are also used. In some conditions fibrosis accompanies the
Chronic traction can also cause circumscribed inflammation, and this may result in permanent
alopecia, often around scalp margins (Fig. 1 3.3). damage t o hair follicles, and obvious loss of tis-
It is commonly seen in young girls with tight sue o r atrophy. This is known as scarringo r cica-
'pony tails', Sikh boys and Afro-Caribbean tricial alopecia.
children whose hair is dressed in multiple little Examination of the rest of the skin may pro-
'pigtails'. vide important clues.
ll8 Chapter 13: Disorders ofthe Hair and Nails

Discoid lupus erythemamsus LUPW vulgaris(CutaneoUsTB)

* Prominent pluggingofthehair follicles
Look for lesionson the face
Especially ininternational residents

ltigeminal tmphic syndrome

Lichen planus * Mayfollowherpeszosterbecauseof
* May accompanylichenplanuselsewhere hypoaesthesia andchronic trauma
* Nail involvementis common (see Chapter
15) Pseudopelade
* Smallpatchesofscaningalopedawithout

AloDecia follows blisterinQ
distinguishing feacures

In most of these conditions a biopsy is Treatment includes shaving, waxing, depila-

essential. In cases where lupus erythematorus tory creams and electrolysis.The antiandrogen
o r cicatricial pemphigoid are suspected, im- cyproteroneacetatemay help.
munofluorescence should also be performed.
Generalized h a i r loss Excessive hair growth in a non-sexual distribu-
Generalized hairloss is rare, butmay accompany tion may occur in both sexes.There are several
endocrine disturbances, especially hypothy- causes:
midism o r hypopituitorism. Drugs, particularly
cytotoxics, may induce widespread alopecia. As
has been mentioned, alopeciaareata may lead t o
complete hair loss-oiopeciouniversalis.

Excessive hair and hair in

- Congenitalgenenlized.e.g.Comeliade
abnormal sites Congenitallocalized,e.k'faun-tail'inrpina
This term is applied t o excessive growth of
bifida occulta
minoxidil (nowusedforbaldness-see
hair in a female, distributed in a male secondary above)
sexual pattern. ciclosporin (qclosprin)
A search for more serious causes is indicated hydantoins
if the changes are of rapid onset
- systemicstemids
* Cachexia
* Porphyiaolmneatarda:assodandwith
- Mildhinu~misquitecommoninelderly
Pretibial myxoedema:overlyingplaques

It maybea genetic w i t in younger females,

general reductionin scalphair (see Nail abnormalities


Nail changes may be non-specific, o r character-
polycysticova~~syndmme istic of specific processes. They may occur in
Drugswith androgenicactivitg
isolation, but the nails are abnormal in several
Nail abnormalities 1 19

Congenital Acquired
* Especiallydisordersofkeratinization,e.g.
Darier'sdisease -
* Psoriasis

- Ectodermaldysplasias
Due toscamng,e.g. dystrophic
epidennolysis bullosa
* Alopecia areatdmtalis
* Fungalinfections

0 Seeniniron deficiency(seealsokoi1onychia)
* whitenails-associatedwithcirrhosis
Halfredhalfpale-renal disease
and thyroid disease Sulfuryellow-fungalinfection
* UniformyeIlow-'yellow nail syndrome'

m g k s (Q=b-nychia) (+bronchiectasis andlymphoedema)

* Commonandoftennon-spedfic
May result fiomwidespreadpitting(see
Green-blue-Pseudornonas infection
* Brown-black-melanoma, haematoma

Bean's h e a
Horizontal~~oovesduemmajorillners Nailswithaconcaveuppersurface

* Classicalfeatureofpsoriasis
iron deficiency
* severe alopeciaareata (smaller,morewenly inherited
distributedtianin psoriasis)
* Eczemddennatitis (coarsedentsand Wpshbaardnui
irregular pits) * Habitualpickingofnail foldleads to surface
Ony&olpis(Fig. 13.4)
Liftingofnailplateoffnailbed Onphogryphads
* Causes: * Grossly thickened,distorted nails (Fig. 13.5)
psoriasis often due toneglect
fungalinfection (see Chapterg
thyrotoxicosis wqgium*
space-occupyinglesion(e.g. exostosis or * Damageleadstoepitheliumencroachingon

- tumour)
nail surface
* Cause-lichen planus

Lass of"*
may be familial
* Causes:
scamng, e.g. Stevens-Johnson syndrome
severe inflammation,e.g. pustular
UiSCU1-h psoriasis
120 Chapter 13: Disorders of the Hair and Nails

Fig. 13.4 Onycholysis of the

nails in a woman with no other
relevant findings.

Fig. 13.5 Onychogryphosis.

Fig. 13.6 Myxoid cyst of the

Nail abnormalities 12 1

Common disorders of the trimming nails straight, but surgical intervention

paronychium is often required.
Patients may also complain of disorders of the
area around the nail -the paronychium. Periungual warts
Warts are discussed in Chapter 3. Periungual
Paronychia warts are unsightly and are often extremely dif-
There are two common forms, acute and ficult t o eradicate.
chronic. In acute paronychia, an abscess in the
nail fold forms, points and discharges. It is Myxoid cyst
nearly always staphylococcal. Chronic parony- Small cystic swellings may appear on the proxi-
chia is discussed in Chapter 4. mal nail fold (Fig. 13.6).The nail may develop a
linear depression. Clear gelatinous fluid can be
Ingrowing nails expressed, if the surface is breached. These
Overcurved nails (especially on big toes) dig cysts are commoner in the middle aged and el-
into the lateral nail fold leading t o chronic in- derly and are due t o a degenerative process.
flammation and overproduction of granulation Treatment is with cryotherapy, o r various surgi-
tissue. Sometimes this can be prevented by cal procedures.
Bullous Disorders

All that blisters is not pemphigus This is a fairly comprehensive differential

(Graham-Brown and Burns, 1990) diagnostic list for further reading. Some dis-
orders, such as impetigo and the viral causes,
are mentioned elsewhere. This chapter deals
Causes with the most important remaining causes of
The skin has a limited repertoireofchanges, but
few are more dramatic than an eruption of Physical causesof bullae
blisters or bullae.There are many causes. Burns may result from cold, heat o r chemical


~ccelid Md
* Impetigo Bullae are a major f e a m in:
* Pemphigus

Herper wster
* Bullouspmphigoid
* Cicauicialpemphigoid

-- Herpessimplex

* l5neapediswithpompholyx

* Insectbites

Drags (seealso Chapter 21)


frusemide,nalididcacid (light-induced)
Causes 123

injury and are common causes of blisters, as is 2 Loss of adhesion of epidermal ceHs
extreme friction (e.g. the feet ofvigorous squash ('acantholysis').
players o r joggers). Severe, acute oedema ofthe These changes may be just above the basal
lower legs may also produce tense bullae. layer (pemphigus vulgaris; Fig. 14.1) o r higher in
the epidermis (pemphigus foliaceus; Fig. 14.2).
Arthropods The commonest variant is pemphigus vulgaris,
Remember that insect bites very commonly which presents with flaccid blisters and ero-
present as tense bullae (see Chapter 5). In the sions (Fig. 14.3).These may be anywhere, but in
UK, this is most common in late summer and over 50% of patients the disorder involves the
early autumn (fall). mouth (Fig. 14.4). Perineal lesions are also com-
mon.The blisters rupture easily and the result-
Drugs ing erosions heal very slowly, if at all. A highly
Several drugs cause blistering (see above). Blis- characteristic feature is the Nikolsky sign: skin at
ters caused by nalidixic acid occur on the lower the edge of a blister slides off when pushed by a
legs following sun exposure. Fixed drug erup-
tions may blister (see Chapter 2 1 ).

Skin disorders
Primary skin disorders giving rise t o bullae may
be congenital o r acquired. In some, bullae are an
important o r integral part of the clinical presen-
tation. In others, blisters may occur but are not
the most prominent o r constant feature, and
the reader should consult the appropriate
chapter for further information.

Epidermolysis bullosa
Although very rare, this is an important group of
Fig.14.1 Pemphigus vulgaris: split just above the
disorders. Babies are born with fragile skin that basal layer,with overlyingacantholysis of
blisters on contact. There are several variants, epidermal cells.
with splits at different levels in the skin; all are
unpleasant and some are fatal.
Diagnosis requires electron microscopy t o
determine the level of the blister.
The differential diagnosis of blistering in a
neonate must also include a number of other
I Impetigo (pemphigus neonatorum).
2 Staphylococcal scalded skin syndrome (see
3 lncontinentia pigmenti (see Chapter I I).

The cardinal processes in all forms of pemphigus
are: Fig.14.2 Pemphigus foliaceus: similar changes to
I A split within the epidermis. those in Fig. 14.1but higher in the epidermis.
1 24 Chapter 14: Bullous Disorders

Fig.14.3 Pemphigus vulgaris:

flaccid blisters and erosions.

Fig. 14.4 Pemphigus:oral


finger or picked up with forceps.This sign is vir- 1nvestigations.Theinvestigations performed in all

tually pathognomonic as it is only seen in pem- forms of pemphigus are the same:
phigus and toxic epidermal necrolysis (see I Biopsies from involved skin, preserving a
below). Pemphigus vegetans is a variant of pem- blister intact if possible, for histopathology.
phigus vulgaris in which vegetating masses 2 Perilesional tissue for direct immuno-
occur, especially in the flexures. fluorescence.
Pernphigus foliaceus does not always present 3 Serum for indirect immunofluorescence.
with obvious blisters, because they are even The immunopathology of pemphigus vulgaris
more fragile than in pemphigus vulgaris: there and foliaceus is identical:
may only be non-specific scaly areas, and scalp I Bright staining around epidermal cells with
and face involvement can closely simulate antibodies directed against immunoglobulin G
seborrhoeic eczema. One variant known as (IgG) and C3 (Fig. 14.5).
pemphigus erythematosus remains localized t o 2 Circulati~gantiepithelial antibody (in the
the face, and may be confused with lupus majority of patients).
Causes 125

Fig. 14.5 Pemphigus: direct

immunofluorescence. IgG is
found around epidermal cells.

Fig. 14.6 Bullous pemphigoid:

tense blisters with an
erythematous base arising on
a typical site.

Treatment. Treatment must be vigorous. Before Bullous pemphigoid and cicatricial pemphigoid
systemic corticosteroids were available most Bullous pemphigoid is much commoner than
patients died, often after a long and debilitating pemphigus. More than 80% of patients are aged
illness. over 60.
High doses of prednisolone (60- 120 mg Bullae are the key feature, but are not always
daily) are used. The dose is gradually reduced present initially: the process may begin with a
when new blistering has ceased (usually in about non-specific phase known as 'prepemphigoid',
4-6 weeks). lmmunosuppressiveagents such as characterized by intense irritation and well-
azathioprine, chlorambucil, cyclophosphamide defined, slightly elevated, erythematous areas.
o r methotrexate may be added as steroid- The bullae, which are usually numerous, are
sparing agents. tense and dome shaped, and may be blood filled
Good nursing and metabolic management (Fig. 14.6).They vary from a few millimetres t o
are crucial because pemphigus patients may be several centimetres in diameter and often arise
systemically ill. Widespread erosions cause loss on urticated erythema as described above, but
of protein and fluid, and secondary infection is are also seen on normal skin. Although the le-
common. If the mouth is severely involved, pa- sions may appear anywhere, there is a marked
tients cannot eat and may be severely catabolic. predilection for the limbs. Oral involvement
l26 Chapter 14: Bullous Disorders

occurs in about 30%.When blisters burst, heal- Bullous pemphigoid usually responds rapidly,
ing is usually rapid. Some blisters do not burst, and maintenance therapy with small doses is
and the fluid is simply reabsorbed. Scarring is usually possible. The condition appears t o be
rare, but there is a distinctive variant, charac- self-limiting in some. Cicatricial pemphigoid is
terized by marked scarring, known as cicatricial much less responsive.
pemphigoid. This condition has a predilection
for oral, conjunctival and genital epithelium. Dermatitis herpetiformis and linear IgA disease
The diagnosis in both forms of pemphigoid Dermatitis herpetiformis is uncommon. Its
requires: importance lies in its ability t o cause severe itch-
I A biopsy for histopathology. ing, and i t s association with gluten-sensitive
2 A biopsy for immunofluorescence. enteropathy.
3 Serum for indirect immunofluorescence Clinically, the cardinal features are pruritus
(less valuable). and grouped erythematous papules and ves-
The pathologicalfindings are as follows: icles, found most typically on the elbows
(Fig. 14.9) and extensor surfaces of the fore-

~ * Asubepidemal blister(Fig.14.7)
* Alinearbandoflgc andC3 at thebasement
membrane zone (Fig.14.8)
* Acirculatinglgcantibodytobasement
membranein 70%ofpatien+swith bullous

1 NOcircu~atingantiiyinci&txicial

Treatment. Both variants require treatment with

systemic steroids, in moderate doses, usually
with ~ m m ~ n ~ ~ ~ psuch p raseazathioprine
~ ~ i ~ e ~ Fig. 14.7 ~ ~pemphigoid:
l the
l split~is ~ ~
or chlorambucil. subepidermal.

Fig. 14.8 Bullous pemphigoid:

direct immunofluorescence. IgG
at the basement membrane
Causes 127

arms, knees and shins, buttocks, shoulders Dermatitis herpetiformis should be con-
and scalp. sidered in any patient with atypical eczema
The intense itching may result in excoria- o r pruritus localized t o the areas mentioned
tions, secondary eczematization and lichenifica- above.
tion and it can be difficult t o find intact vesicles The diagnosis requires:
o r bullae. I A biopsy of a blister or, preferably, a new pink
papule for histopathology.
2 A biopsy of normal skin for immuno-
3 A jejunal biopsy.
The main pathological findings are as follows:

* Arubepidermalblisterwhichis
indistinguishable,whenfully formed and
intact,from that seen in bullouspemphipid

vesicle,there are small neutrophil
microabscessesin dermal papillary tips (Fck
14.10).Theseare pathognomonic
GranularlgAin the dermalpapillarytipson

- Therearenocirculatingantikdies
Gutchangesrangefroman increasein
lymphocytenumberstovarious gradesof
villous atrophy

Treatment. Dermatitis herpetiformis responds

dramatically t o sulfones. Dapsone is the drug of
Fig. 14.9 Dermatitis herpetiformis:the elbow is a first choice but it induces haemolysis, especially
typical site. at higher doses. Alternatives are sulfapyridine

Fig.14.10 Dermatitis
herpetiformis: papillary tip
microabscesses as well as a
subepidermal blister.
128 Chapter 14: Bullous Disorders

(sulfapyridine) and sulfamethoxypyridazine diet, because there may be an increased risk

(sulfamethoxypyridazine). Patients in whom a of gut lymphoma (similar to coeliac disease).
gluten-sensitive enteropathy has been demon- Indeed, some patients may respond t o diet
strated should also be started on a gluten-free alone.

Linear IgA disease

Occasionally, patients wirh a pemphigoid- o r
dermatitis herpetiformis-like presentation are
found t o have a linear band of IgA at the base-
ment membrane on immunofluorescence.This
is now generally considered to be a separate
bullous disease, which may be seen in both
children and adults.

Rarer blistering diseases

Porphyria cutanea tarda

This is rare. It presents as small blisters and
erosions on the backs of the hands, and on the
forearms and face, following sun exposure o r
minor trauma. In many patients there is an
underlying liver disorder, and often alcohol

Toxic epidermal necrolysis

This is a term applied CO an acute disorder in
which there is lossofthe epidermis, usually over
Fig. 14.11 Dermatitis herpetiforrnis: wide areas of the body surface (Fig. 14.12),
immunofluorescenceofnormaiskin.~ranu~~~ although localized forms have been described.
depasitsaflgAin derrnalpapillae. Nildsl+ sign is positive. Primary toxic epider-

Fig. 14.12 Severe skinlossin

toxic cpidermalnecrolysis.
Rarer blistering diseases 129

mal necrolysis is usually an adverse reaction t o a Bullouserythema multiforme

drug. (Stevens-Johnson syndrome)
Extensive epidermal loss leads t o severe de- This is a reactional state due t o a wide variety of
hydration and protein depletion. Patients re- triggers (see Chapter 15). In severe erythema
quire intensive care, and are best managed in a multiforme bullae may be the most prominent
manner similar t o those sufferingfrom burns. clinical feature.
Er-ythernatous and
Papulosquamous Disorders
and Light-induced Skin
Introduction, 130 Lichen planus,l34 Pityliasislichenaides,137
Urlicaria and angioedema.130 Lichennitidus.135 Pityliasismbrapilaris, 137
E~ythemamultiforme.132 Lichen sclerosus et atrophicus, Miliaria or'prickly heat', 137
Exfoliativedermatitis 135 Pregnancy rashes, l37
(erythrodema), 133 Pityriasis rosea. l36 Light-inducedskin disease, l38

Misceiioneous:ofmixedcomposition or Afrequent accompanyingfeature of urticaria

choracter;of various kinds;many-sided. is ongioedemo, in which oedema extends into
(Concise Oxford English Dictionary) subcutaneous tissues, especially around the
eyes, the lips and in the oropharynx. The
swelling may be alarming, occasionally resulting
Introduction in complete closure ofthe eyesand compromis-
ing the airway.
This chapter is a mixed bag: it covers a number urticaria and angioedema may be part of a
of common andlor important skin disorders systemicanaphylacticreaction.
that have not found a place elsewhere.

Clinical forms of urticaria

and angioedema
Urticaria and angioedema
Acute urticaria
Attacks last only afew hours o r days. Common
Urticariais theclinical term foragroup ofdisor-
causes include:
derscharacterized bythe formation ofeweals'-
I Contact with plants (e.g. nettles), animal
swellings of the skin which disappear leaving no
fur (e.g. dogs, cats, horses) o r foods (e.g. milk,
visible sign. Most of us have experienced one
common form after falling (or being pushed)
into nettles ('nettle-rash' is used commonly
2 Ingestion of foods, especially nuts, shellfish
and strawberries.
for urticaria). The main pathological change is
dermal oedemadue tovasculardilatation, often
3 Ingestion of drugs, e.g. aspirin and penicillin.
People with atopy (with asthma, eczema o r
in response t o histamine (and probably other
hay fever) are more susceptible.The reaction is
mediators) released from mast cells.
generally triggered by immunoglobulin E (IgE)
Clinical features.The skin itches o r stings; weals antibodies; some reactions, e.g. t o aspirin. are
develop, white at first, then pinl< with a white due t o direct mast cell degranulation.
rim; lesions can become very extensive and ap-
pear in many sites atonce, but always clearspon- Chronic urticaria
taneously within a few hours, even though new Attacks last for weeks, months o r years. Con-
lesions may continue t o develop. trary t o popular expectations, a single causative
Typical lesions of urticaria are shown in factor is seldom found in this form of urdcaria.
Fig. 15.1. Chronic ingestion of food colourings and
Urticaria and angioedema 13 1

preservatives may be important, but in our I Dermographism: weals appear after scratch-
experience (and that of most dermatologists) marks (Fig. 15.2); this may occur alone o r with
this is only true of a minority of patients. other forms of urticaria.
2 Pressure (delayed): weals develop up t o 24 h
The physical urticarias after pressure is applied.
Several physical insults may trigger urticarial 3 Cholinergic urticaria: affects young men;
responses: sweating is accompanied by small white weals
with a red halo on the upper trunk.
4 Cold.
5 Water.
6 Sunlight.
7 Heat.

Hereditary angioedema
In this very rare autosomal dominant
I C I esterase inhibitor is lackingo r defective.
2 There are sudden attacks of angioedema
which can be life-threatening.
3 The gut may be affected, giving rise t o spasms
of abdominal pain.

Urticaria pigmentosa
Abnormal accumulations of mast cells result in
multiple pigmented macules that urticate on
being rubbed (see also Chapter 12).

Urticaria in systemic disease

An urticarial eruption may be part of a systemic
disorder, especially hepatitis B.

Fig. 15.1 Urticaria.

Fig. 15.2 Dermographism.

132 Chapter 15: Miscellaneous Disorders

Treatment of urticaria
Erythema multiforme
If a possible trigger
.. can be elicited from the his-
tory, it should be avoided. Aspirin should be
Theclassiclesion oferythemamultiforme isthe
banned in anyone prone t o urticaria.
'iris' or'target' lesion (Fig. 15.3): a round or oval
Mosttypes of urticariarespond t o H, antihis-
area of erythema, with a dusky, purplish centre.
tamines although some of the rarer, physical
Sometimes the centre becomes paler and a
forms do n0t.A large rangeofagents is available.
many of which cause CNS depression, but
several newer antihistamines have litde o r no
History. Lesions appear suddenly, enlarge over
sedative effect (e.g. loratidine, cetirizine, fexo-
the course of afew days, and fade (often leaving
fenadine).Theseare now drugs offirst choice. It
pigmentary disturbances). The whole process
may help t o add an H, antagonist (cimetidine,
settles in about 3 week. Repeated episodes are
rare, but can be triggered by herpes simplex
It is sometimes necessary to use other
(see below)
agents, such as systemic steroids and adrenaline
(epinephrine) (see below)
Aetiology Erythema multiforme may occur out
of the blue, but there are several recognized

Acute attacks-afewdays'treatmentis

usually sufficient
antihistamine which suppresses the eruption * Herpessimplex-the
campletely,maintainthisdosefo~several herpes maybe recurrent,so may herpes-
months;graduallywithdrawtreatment related erythema multifonne
Angiwdema-may requireparenteral * Othervirures-orf. hepatitis,mumps
therapywith adrenaline (epinephrine).

an~israminesand steroids
Anaphylaxis-adrenaline (epinephrine)is
- Radiotherapy
* Connectivetissuediseases
required * Awidevarietyofdrugs
fHereditavangioedema-does not respond
to antihistaminesor steroids; danazol works
by increasinglevelsofthe missingenzyme;
purified enzyme preparationsare available Examinotion. The distribution characteristically
includes extensor surfaces of arms and legs, but

Fig. 15.3 'Target'lcsions of

erythema multiforme.
Exfoliative dermatitis (erythroderma) 133

most important diagnostically is involvement of and cause ocular scarring. Patients occasionally
palms and soles. die of severe bronchopulmonary involvement
o r renal failure.
Pathology.Theprocesrisavarculitisand themore
serious the vascular damage, themore dramatic Treatment. Close attention must be given t o
are thechanges.When really severe, theepider- fluid balance and nutrition. The role of sys-
mis becomes necroticand bullae may form. temic steroids is controversial, because the
morbidity from steroids may outweigh that of
T-eotment. Erythema multiforme is self-limiting. the disease.
and treatment is not usually required.

Stevens-Johnson syndrome
A t is mostextremeerythema mulriformecaus-
es a major systemic disturbance. There is an
acute onset, with severe inflammation of con- These terms (either will do) are used to de-
junctivae, mouth and genitalia (Fig. 15.4). which scribes state in which most of theskin becomes
may prevent normal eating, affect micturition red, inflamed and scaly (Fig. 15.5).

Fig. 15.4 Erasionson thelips in

bullour clythema multiforme.

Fig. 15.5 Exfoliativedcrmalitis

134 Chapter 15: Miscellaneous Disorders

The correct management depends on the

underlying disease process, because the opti-
mum treatment is different. The four most
important causes of exfoliative dermatitis
Drug reactions.
Lymphomas (especially cutaneous Tcell

Clinicol features.The skin is red, hot and scaly;

there may be generalized lymphadenopathy;
there is a loss of control of temperature regula-
tion, and there are bouts of shivering as the
body anemprs to compensate for heat loss by
generating metabolic heat.

Efefens. Cardiac output is increased: protein is

lost from the skin (and the gut); water loss
from the skin is increased: patients radiate
heat into their surroundings: there is a rise in
metabolic rate, with mobilization of energy
sources and increased muscle activity; the Fig.15.6 Typicalpapules oflichen planus
body cannot compensate for long, especially in
the elderly.
fectlng I% of new referrals t o a dermatologist.
Compiications. Cardiac failure; renal failure; The commonest pattern is an acute eruption of
sudden death due t o central hypothermia. itchy papules (Fig. 15.6).

Treatment. Sites ofpredilection.Wrists, anldes and the small

I Stop any potential causative drugs (see of the back; lichen planus may affect the mouth
Chapter 2 I). andgenitalia.
2 Nurse the patient in a warm room.
3 Attend to secondary medical problems (e.g. Clinical features:
dehydl-ation,heart failure and infections). I SMn lesions:
4 Biopsy skin t o obtain definitive diagnosis. (a) flat-topped;
5 Give short course of systemic steroids (if (b) shiny;
the diagnosis i s known t o be psoriasis from the (c) polygonal (Fig. 15.6).
outset, commence systemic antipsoriadc drugs 2 Surface-fine networkof dots o r lines called
inrtcad-see Chapter 8). 'Wickham's striae'.
6 Initiate appropriate treatment for underlying 3 Colour-'violaceous' (reddish-purple).
diagnosis. 4 Oral-lacy, reticulate streaks on the cheek,
(Fig. 15.7),gums and lips.
In the majority of patients, the eruption set-
Lichen planus tles over a period of a few months.There are a
number of variants, some of which are more
Lichen planus is a rather variable disorder, af- persistent.
Lichen sclerosur et atrophicus 135

Fig. 15.7 Orallesions inlichen


Lichen sclerosus e t atrophicus

Lichenscierosusetatrophicus (oftenshortened
to lichen sclelnsus o r LS et A) is a disorder of
unknown aetiology.

Sites of predilection. The genitalia, especially in


Clinical features:
I White, atrophic patches on the vulva.
perineum and perianal skin, or glans penis and
Aetiology. Lichen planus i s aT-cell-mediated at- 2 Similar plaques may develop elsewhere.
tack on the epidermis, similar changes being 3 Purpura and blistering may appear.
seen in graft-versus-host reactions. However. 4 Vulval lichen sclerosus easily becomes
the cause of lichen planus in most instances eroded and haemorrhagic, with severe sore-
remains a mystery. ness and irritation.

Treatment. Potent topical steroids usually sup- Complications. Vulvo-vaginal stenosis; develop-
press irritation; very extensive o r severe oral ment of rquamous cell carcinoma.
disease may need systemic steroids o r
ciclosporin (cyclorporin). Childhooddisease. Lichen sclerosus in prepuber-
tal girls often presents with dysuria and pain on
defaecation. It may be mirdiagnosed as sexual
Lichen nitidus abuse, but lesions are usually easy t o diagnose
(Fig. 15.8), and parents and child can be reas-
Probably avariant of lichen planus, this uncom- sured. The prognosis of childhood disease is
mon disorder produces clusters of tiny, asymp- good, as many clear at puberty.
tomatic papules.
Disease in moles. Lichen sclerosus may be seen
on theglans and prepuce (sometimes called'bal-
136 Chapter 15: Miscellaneous Disorders

Fig. 15.9 Pityriasisrosea.

Fig. 15.8 Lichensclerasusina prepubertalgirl (often misdiagnosed, especially as ringworm!).

3 Afew days later, there is a sudden eruption of
anitis xerotica obliterans'), and can give rise t o pink, oval patches on the trunk, upper arms and
phimosis and meata stenosis.Asignificantnum- thighs.
ber of boys undergo circumcision because of Thereare three especially notablefeatures:
phimosis due to lichen sclerosus. Extragenital I O n the trunk, lesions tend t o lie with their
lesions may also occur. long axes in lines sweepingfrom the back to the
front (almost as if they were following spinal
Treatment. The disease in adults generally pur- nerves). This is said t o loolk like an 'inverted
sues a chronic, relapsing course. Very potent Christmas tree' (Fig. 15.9)-but that depends
topical steroids provide symptomatic relief in on whether you are lookingat the patient's back
vulval disease. Patients should be [kept under o r front and on your concept of a Christmas
surveillance because of the risk o f neoplastic tree! However, once understood, this sign will
change. never be forgotten and no otherdisorderpmduces
2 The scale on the surface of each lesion
Pityriasis rosea exhibits a tendency t o peel from the inside
towards the edge, resulting in a so-called
Pityriasis rosea is a self-limiting disorder, pre- 'peripheral collarette' (Fig. 15.10).
dominantly affecting children and young adults. 3 If none of this has resulted in the diagnosis
Clinical features: being made, it becomes clear when the rash
I There may be a mild prodromal illness. disappears (as it always does) in 6-8 weeks.
2 One o r more 'herald patches' appear. A
herald patch is large, red, oval and scaly, and Treatment. Usually unnecessary, but mild topical
usually appears on the trunk o r upper arm steroids may help t o relieve irriration.
Pregnancy rashes 137

Fig. 15.10 Pityriasisrosea: t h e

'peripheral cailarette'afscaie.

Atypical pityriasis rosea be confused with polymorphic light eruption

There may be no gap between the herald patch (see below) in which the lesions are induced
and the generalized rash; the eruption may ex- by light not heat. The condition is also seen in
tend down the arms and legs, and occasionally infants. particularly In the napkin area.
spares the trunk altogether; lesions may be so
numerousthatthedistribution described above
is not apparent; the inflammation may be so Pregnancy rashes
intense that it causes blisters.
Pregnancy may alter the course of a number of
skin disorders, such as acne, eczema, psoriasis
Pityriasis lichenoides and vulva1 warts, and it may trigger erythema
Small brownish-red papules surmounted by a There are also three important conditions
'plate' of scale appear on the trunl< and limbs. related t o pregnancy itself.
Some patients have more acutely inflamed
lesions that heal t o leave pockmarks.

Pityriasis rubra pilaris

i * UptoZO%ofwomen:maybeduem
Pityriasis rubra pilaris (PRP) occurs in locali-
zed o r generalized forms. All types are rare.
Lesions are reddish-orange,and hairfolliclesare
prominently involved. Generalized change is a , Blotchy,udcarialandpapularrarhwith
very rare cause of exfoliatlve dermatids (see intense itching
above). * Onset in third himester
Lesions favour abdomen
* Parficularp~dedil~onforrhiae(fi~15.11)
Miliaria or'prickly heat'
This is the exotic name for little red bumps that
some peopledevelop in h o t humid conditions. It
is due t o sweat duct obstruction. It should not
* Blister3onwticated background
138 Chapter 15: Miscellaneous Disorders

Fig. 15.11 Polymorphic


Light-induced skin disease Sunburn

Most of us are familiar with sunburn, even if
But yet the light that led astray only in others. Excessive medium wavelength
Was iight from Heaven UVR induces erythema and, if severe, blister-
(Robert Burns. The Vision) ing. The dose required depends on skin type
Sunlight is generally thought t o be beneficial: (see Chapter 12). and the intensity of the
advertsfor sunbeds, solariaand foreign holidays UVR: s h types I and II are very prone t o
all bear witness to this twentieth-century myth. sunburn; sunlight around midday is the most
However, ultraviolet radiation (UVR) can intense.
initiate, wholly or in part, many unwanted Treatment of established sunburn is difficult,
skin changes: but calamine lotion and topical steroids may
Some are chronic: cancers and keratoses helpsymptomatically. Prevention ismuch better
(see Chapter 9); the yellowing, coarsening and than cure.
wrinlding known as 'photo-ageing'. Note that Sun care should include avoidance, hats
most oftoday's tanned beauties are tomorrow's and clothing, sunglasses and sun-screenr.All ex-
'wrinlded prunes'! posed surfaces need to becovered. Sun-screens
Some are moreacute: sunburn; reactions t o a come in a range of potencies, graded by 'Sun
combination of plants o r drugsand light Protection Factor' (SPF) number.This number
S o m e are due t o metabolic disturbances. indicates the approximate multiple o f time t o
whereas in others the cause is quite unknown. redness that the agent will provide: if the expo-
UVR may also exacerbate certain pre- sure time t o redness i s normally IOmin. SPF 6
existing slcin disorders (see below). sun-screenwill prolongthis toaboutan hour.

- Sunburn
* Photosensitiveeczema
* Porphyrias
* Solarurticaria(seeabave) * Pellagra

* ACtiniCpNrigO
* JuvenileSpringeNpion
- Xerodennapigmentosum
Light-induced skin disease 139

Polymorphic light eruption Treatment. This is very difficult. Barrier

This common disorder is frequently misdiag- sun-screens containing titanium may help, and
nosed as 'prickly heat' (see above). Women are azathioprine has been shown t o be of benefit.
affected more often than men, and typically
trouble starts in adolescence o r early adult- Porphyrias
hood. This miscellaneous group of disorders is due t o
enzyme defects in the haem production
Clinical features. An eruption develops on light- pathways. Some, but not all, are associated with
exposed surfaces, most commonly the face, photosensitivity.
arms, legs and the 'V' of the neck. Individual The commonest in northern Europe is ery-
lesions vary from papules t o plaques. Blisters thropoietic protoporphyria, in which burning in
are sometimes seen. The reaction may only the sun (even through glass) develops in early
occur in very strong sunlight, but even mild childhood. A form known as 'variegate por-
British summer sunshine can be the trigger. phyria' is seen in some Dutch and South African
Treatment. Preseason PUVA is helpful. Anti- It is perhaps worth mentioning that one of
malarial~may be of some benefit, and sun- the rarest (congenital erythropoietic porphyria
screens and clothing will help prevent the o r Gunther's disease) may be the origin of the
eruption in some. werewolf legend. Sufferers become disfigured,
hairy and anaemic (hence the werewolf's
Actinic prurigo craving for blood). They avoid sunlight because
Actinic prurigo is a rare disorder of childhood of severe photosensitivity (the werewolf
in which eczematous areas develop on the prowls at night when the moon is full -a logical
face and backs of the hands every summer, time t o prowl if there is no other source of
and disappear in the winter. The cause is illumination).
unknown and attempted treatment is often
ineffective. Pellagra
A photosensitive rash in the malnourished
Juvenilespring eruption should suggest pellagra. The classical triad of
Little boys occasionally develop blisters on the diarrhoea, dermatitis and dementia is only
ears in spring, and this is given the grand title of seen in western societies in alcoholics and
'juvenile spring eruption'. It is probably a variant recluses.
of polymorphic light eruption.
Xeroderma pigmentosum
Photosensitive eczema and This rare disorder often presents with
chronic actinic dermatitis photosensitivity in early childhood (see also
Some individuals develop eczema of light- Chapter I I).
exposed surfaces. In others a pre-existing
eczema becomes much worse on exposure t o Phytophotodermatitis
light. Every summer, we see patients who have de-
One cause is a contact dermatitis t o airborne veloped a rash following contact with plants
chemicals, such as perfumes o r plant extracts on sunny days. Linear, streaky dermatitis (Fig.
(e.g. chrysanthemums). A similar picture may 15.1 2) results, and residual pigmentary distur-
occur with certain drugs. bances are common. One important cause is
The changes tend t o become more intense giant hogweed, but there are several others.
until the skin is permanently thickened and in-
flamed. This state is termed 'chronic actinic Light-induced drug reactions
dermatitis'. Several groups of drugs are associated with
140 Chapter 1 5: Miscellaneous Disorders

Fig. 15.12 A

photoallergic and phototoxic reactions (see

Chapter 2 l).

Disorders exacerbated by light

- Lupvscrythernatosus
* Rosacea
A number of disorders may show a deteriora- Pwriasis
tion or provocation on exposure t o light. The
mechanisms for this are unclear. - Dariers disease
Herpes sirnpla
Vascular Disorders

Legulcers, 141 Vasculitis, l43

the transudate is converted t o fibrin, which

Leg u l c e r s forms cuffs around blood vessels.The pe~kapil-
lary fibrin impairs transfer of oxygen and nutri-
By far the commonest type of leg ulcer is the ents, and the relatively ischaemic tissues are
venous ulcer. Other causes of leg ulceration susceptible t o ulceration, either spontaneously
include ischaemia, vasculitis, skin neoplasia o r following minor trauma.
and certain haematological disorders. Problems caused by venous hypertension
usually present in middle or old age, and
V e n o u s leg u l c e r s women, particularly the obese, are pre-
Venous retulnfrom the legs is dependent on the dominantly affected. The clinical features are
deep and superficial venous systemsand activity as follows.
of the calf muscles.When the calf muscles con-
tract they pump blood in the deep veins to-
wards the heart against gravity. Valves in the
deep veins prevent reflux of blood when the
muscles relax. During relaxation of the calf
muscles blood parses from the superficial veins
into the deep veins via the sapheno-femoral
- Varicoseveinr
* Oedema
and sapheno-popliteal junctions and numerous * tipodematoxlemrip
perforating veins. If the valves in the deep veins * Hyperpipentation
are incompetent the calf muscle pump cannot * Eczema
function effectively and venous hypertension Anophie blanche
develops. Congenital abnormalities of the Ulceration
venous system, and valve damage following
deep vein thrombosis, contribute t o incompe- Lipodermatosclerosis. This term refers t o areas
tence. Genetic factors are important as certain of induration, caused by fibrosis, on the lower
racial groups have a low prevalence of venous parts of the legs, above the ankles (Fig. 16.1).
hypertension and venous ulcers. There is initially an area of erythema, and
The high pressure in thedeepveinsofthe legs this subsequently becomes purple-brown
is tl-ansmittedviaincompetentperforatingveins in colour. On palpation. affected areas feel
t o the superficial venous system (resulting in indurated.When the process is circumferential
'varicose' veins), and eventually t o the capillary the tissues around the anlde are constricted
network. Skin capillaries become dilated and and the leg above is oedematour, producing
tortuous, and there is increased transudation of the classical 'inverted champagne bottle'
fluid into the surroundingtissues. Fibrinogen in appearance.

142 Chapter 1 6: Vascular Disorders

Hyperpigmentation. Haemosiderin, derived from Atrophie blanche.This term is applied t o areas of

red cells extravasated from dilated, leaky cap- scar tissue within which are prominent dilated
illaries, produces areas o f brown discoloration. capillaries. Scattered pink dots are seen on a
white background (Fig. 16.2). Such areas are
Eczema. Areas of 'varicose' eczema are very prone t o ulcerate, and the ulcers are
common. usually extremely painful.

Ukers.The commonest site for a venous ulcer is

the medial aspect of the leg, just above the
medial malleolus (Fig. 16.3), but the lateral
malleolar area may also be affected.
Rarely, a squamous cell carcinoma may
develop in a longstanding venous ulcer
(Marjolin's ulcer).

Varicose eczema may be treated with mild po-
tency topical steroids. Duplex sonography is a
valuable investigation, and assessment by a vas-
cular surgeon is an important aspect o f manage-
ment, as some patients benefit significantly from
surgery on incompetent superficial veins. In ad-
dition, it is essential t o assess the arterial supply
in patients with leg ulcers because they may have
a remediable arterial abnormality. It is n o t un-
common t o discover both venous and arterial
pathology in individuals with leg ulcers.
Another important aspect of the manage-
ment o f venous ulcers is reduction of venous
hypertension and oedema by compression
bandaging. It is vital, however, t o establish that
Fig.16.1 Lipodermatosclerosis. the arterial supply t o a limb is adequate (by

Fig.16.2 Atrophie blanche.

Vasculitis 143

Fig. 16.3 Venousulcer

Doppler studies) before using compression ders, including rheumatoid arthritis and sys-
bandaging. temic lupus erythematosus (SLE), may produce
Secondary infection, often with a mixed bac- legulcers.
terial flora, occurs in the majoricy of venous
ulcers. However, systemic antibiotic therapy Neoplastic ulcers
is not necessary unless there is associated Basal cell carcinomas and squamous cell carci-
cellulitis (see Chapter 3). nomas arisingon the legs may resemble, and be
There are numerous agents which have been mistaken for, venous ulcers. However, they usu-
marketed as topical therapies for leg ulcers. ally occur above the ankle region. If there is any
including alginate, hydrogel and hydrocolloid suspicion that an ulcer is neoplastic, a biopsy
dressings, but a simple regimen of regular should be performed.
cleansing with saline, followed by the applica-
tion of a topical antibacterial dressing such Haematological disorders and
as chlorhexidine gauze, is often adequate if leg ulcers
combined with compression bandaging. Uncommon causes of leg ulcers include heredi-
When a venous ulcer has healed i t is impor- tary spherocytosis, sickle cell anaemia and
tanttomaintain compression by wearingagrad- thalassaemia. The mechanism of ulceration in
uated compression stocking. these conditions is related t o tissue hypoxia
due to blockageof sl<incapillaries by abnormally
lschaemic ulcers shaped red cells.
lschaemic ulceration is usually a manifestation
of atherosclerotic peripheral vascular disease.
Typically, ischaemic ulcers occur on the dorsum Vasculitis
o r the sides of the foot, between the toes, o r
on the heel. Pedal pulses are reduced o r absent. Classification of vasculitis is difficult, but a sys-
and Dopplel- studies will demonstrate im- tem based on the size of vessel involved and the
paired blood flow. lschaemic ulcers are usually role played by neutmphils, lymphocytes and
painful. g~ranulomatousprocesses is relatively straight-
The advice of a vascular rulgeon should be forward.Triggers of vasculitis include immune
sought. complexes, bacterial and viral disease, and
drugs. Heat and cold damage are also respons-
Vasculitic ulcers ible for vascular changes. Clinically, vasculitis
Vasculitis associated with a number of disor- may present as urticaria, livedo reticularis,
144 Chapter 1 6: Vascular Disorders

purpuric papules, nodules, haemorrhagic bullae Clinical presentations of vasculitis

o r ulcers.
Small vessel
'Allergic'vasculitis(leucocytoclastic vasculitis)
Typically, the patient presents with numerous
palpable, purpuric lesions on the legs, pre-
dominantly below the knees (Fig. 16.4). Some
lesions may develop into haemorrhagic vesicles

'Auer& vasculitis (leukocytadastic
o r bullae.
Histologically, there is fibrinoid necrosis of
small blood vessels, and a perivascular infiltrate
* Behcet'sdisease composed predominantly of neutrophil poly-
morphs. The perivascular tissues also contain

extravasated red cells, and fragments of poly-

* Erythemanodosum
morph nuclei (nuclear dust).These changes are
initiated by deposition of immune complexes in
small vessels, complement activation, and pro-

Wepener's granulomatosis
duction of polymorph chemotactic factors.
Polymorphs attracted t o the area release en-
zymes which damage the vessel wall. Drugs,
bacterial o r viral infections may act as the anti-
Large-1 genic triggering factor, but often the initiating
* Polyarteritisnodosa factor is not discovered.
The joints, kidneys and gastrointestinal tract
Lymphocyfic may be affected, and it is important t o check the
* Lupuse@ematosur urine for microscopic haematuria. Henoch-
Schonlein purpura (anaphylactoid purpura)
Gmnulommous is the name which has been given t o a systemic
* Giant-cellarteritis
allergic vasculitis occurring predominantly in

Fig. 16.4 Allergic vasculitis.

Vasculitis 145

children. Palpable purpuric lesions on the but-

tocks and legs are associated with arthralgia,
gastrointestinal symptoms and proliferative
glomerulonephritis. Viral upper respiratory
tract infections may trigger some cases.

Treatment. If a trigger can be identified, then it

should be eliminated. A period of bed rest may
result in complete resolution of the skin le-
sions.Treatment with dapsone may be helpful in
some cases. If there is evidence of damage t o
internal organs treatment with systemic cor-
ticosteroids o r immunosuppressive agents is

Behqet's disease
The principal features of this disorder are recur-
rent, severe oral and genital ulceration, and
uveitis. Skin lesions include erythema nodosum
and pustules at sites of minor trauma such as
venepuncture sites.

Drug eruptions (see Chapter 2 1 )

Fig. 16.5 Erythema nodosum.
Erythema nodosum
This condition usually affects children and young
adults, and is characterized by the development Investigation of a patient suffering from ery-
of multiple tender, erythematous nodules, usu- thema nodosum should include culture of a
ally on the shins (Fig. 16.5), but occasionallyalso throat swab, antistreptolysin titre, chest X-ray,
on the forearms. As each nodule regresses i t and Mantoux test.
changes colour from red t o purple t o yellow-
green -like a fading bruise. Pathologically, ery- Treatment. In most cases bed rest and simple
thema nodosum is an inflammatory process of analgesia is all that is required.The lesions will
fat (panniculitis) associated with a lymphocytic gradually resolve over a period of a few days.
Causes of erythema nodosum include: Perniosis (chilblains)
Chilblains are painful, inflammatory lesions
provoked by exposure t o cold (Fig. 16.6). The
commonest sites for chilblains are the fingers
and toes, but they may also occur on fatty pro-
minence~such as the fat pads on the medial
aspects of the knees, and on the thighs. A
characteristic type of chilblains occurs on the
lateral aspects of the thighs of horse riders-
this is related t o the chill factor produced
by galloping along in the middle of winter.
'Chilblains' is a rather unimpressive word, so
146 Chapter 16: Vascular Disorders

Fig. 16.6 Chilblains.

dermatologists call this disorder 'equestrian fat, occur on the legs. These may ulcerate.
cold panniculitis'. Other organs are not involved.
Treatment for chilblains is not very satisfac-
tory. The best management is prophylaxis, by Large vessel
wearing warm gloves and thicksocks, and, in the Polyarteritis nodosa
case of the equestrian, thermal underwear and Polyarteritis nodosa (also known as periarteri-
thick jodhpurs. tis nodosa) is an uncommon type of necrotizing
vasculitis which affects small and medium-sized
Wegener's granulomatosis arteries throughout the body. Immune com-
This is a rare form of necrotizinggranuIomatous plexes appear t o cause this disorder, and the
vasculitis affecting principally the small arteries triggering antigens may be infections o r drugs.
of the respiratory tract, and associated with Manifestations include pyrexia, weight loss,
glomerulonephritis. Skin lesions take the form arthralgia and myalgia. The most significant
of a nodular vasculitis, sometimes with ulcera- clinical sign is the presence of cutaneous o r
tion. I t is associated with the presence of anti- subcutaneous nodules along the course of
neutrophil cytoplasmic antibodies (ANCA). superficial arteries. Vessel damage results in
aneurysm formation. Livedo reticularis and
Nodular vasculitis skin ulceration are other features. There may
This is an ill-defined condition, predominantly be renal, gut, cardiac and nervous system
affecting middle-aged women, in whom inflam- involvement.
matory nodules, produced by a granulomatous There is a type of polyarteritis nodosa which
vasculitis in the deep dermis and subcutaneous affects the skin alone. Livedo reticularis and
Vasculitis 147

cutaneous nodules occur on the legs, usually Lupus erythematosus (see Chapter 17)
below the knees.
Temporal arteritis (giant cell arteritis)
Treatment. Polyarteritis nodosa is treated with Skin changes are rare, but ulceration may occur
systemic steroids and immunosuppressive on the temporal and parietal regions of the
drugs. Purely cutaneous polyarteritis usually scalp.
responds t o small doses of systemic steroids.
Connective Tissue Disorders

LUPUS crythemarosus,l48 Dermatomyositis, is0

Lupus erythematosus

Lupus erythematosus is an autoimmune disor-

der which occurs in two main formr-systemic -
Arthralgia and arthritis (usuallynon-
lupus erythematosus (SLE), which affects both
the skin and internal organs, and discoid lupus
erythematosus (DLE), in which the slcin alone is
affected. A small proponion of patients suffer-
ingfrom DLE may subsequently develop SLE.A
third variant, subacute cutaneous lupus erythe- Central ne- .gmmimkent
matosus (SCLE), is characterized by distinctive * Psychosis andconwlsions
skin lesions which may be associated with
systemic features. Haemopoictic abnormnlities
* Normochrornic.norm@c anaemia
* Coombs'positive haemolyticanaemia
Systemic lupus erythematosus
* Leucopenia
This is a multisystem disorder which may affect * Thrombocytopenia
the s h , joints, heart and pericardium, lungs,
I~idneys,brain and haemopoietic system. Typi- ppda,"eightlosr and generald i s c
cally, the disease affects women.. ,~articularlvof
childbearing age, and progresses in a series of
exacerbations and remissions. Its aetiology is
Antinuclear antibodies (ANA), also lknown as
antinuclear factor (ANF), and D N A antibodies
Mucocutaneour lesions include oropharyn-
are found in most patients with SLE. Antibodies
gcal ulceration, diffuse alopecia, Raynaud'r
t o double-stranded D N A are characteristic.A
phenomenon and photosensitivity. Often there
number ofother autoantibodies may also occur,
is facial erythema in a 'butterfly' distribution
including anti-Ro and anti-La, lymphocytotoxic
(Fig. 17.1). The 'butterfly' is represented by
antibodies, antiplatelet antibodies and the lupus
erythema on the cheeks linlcsd by a band of
anticoagulant. A positive rheumatoid factor and
erythema across the nose. However, b y far
biological false-positive serological tests for
t h e c o m m o n e s t cause of this p a t t e r n of
syphilis may also be found.
facial e r y t h e m a is rosacea.
Histology of a biopsy from clinically involved
Systemic manifestations include the
skn shows distinctive features, and direct im-
munofluorescence study demonstrates linear
deposition of immunoglobulin G (IgG) o r im-
Lupus erythematosus 149

Fig. 17.2 Discoid lupus erythematosus.

Fig. 17.1 Facial erythema in systemic lupus

erythematosus. most frequently implicated in its provocation
include hydralazine, procainamide, anticon-
munoglobulin M (IgM) and C3 at the dermoepi- vulsants (phenytoin, primidone), isoniazid and
dermal junction. chlorpromazine.

ll-eatment Discoid lupus erythematosus

Systemic steroids and immunosuppressive Classically, DLE affects light-exposed areas -
agents are the mainstay of treatment. Light- principally the face and neck, but also the dorsa
exposed areas of skin should be protected by of the hands and the arms. Lesions may be
sun-screens with a high sun protection factor precipitated o r exacerbated by sunlight. Indi-
(SPF). vidual lesions consist of scaling, erythematous
plaques, with prominent follicular plugging. If
Antiphospholipidsyndrome the scale is lifted off, follicular plugs may be seen
This syndrome may be primary, o r occur with on its undersurface -the so-called'carpet-tack'
SLE. The main features are the occurrence of sign.There may be only a few lesions, but exten-
recurrent miscarriage, venous thromboses, sive, cosmetically disfiguring involvement of the
cerebral infarcts, thrombocytopenia and livedo facial skin can occur. Lesions heal with scarring,
reticularis. These clinical abnormalities are and the typical picture is of an active, erythema-
associated with the presence of lupus antico- tous scaly margin enclosing a central area of
agulant and anticardiolipin antibodies. scarred, hypopigmented, atrophic skin (Fig.
17.2). The scalp may be involved, producing
Drug-induced systemic areas of scarring alopecia in which follicles
lupus erythematosus are permanently destroyed. Occasionally, the
Drug-induced SLE is uncommon. The drugs buccal o r nasal mucosae are affected.
150 Chapter 17: ConnectiveTissue Disorders

Investigations Skin
The diagnosis can be confirmed by skin biopsy The skin changes are as follows:
Histology shows a periadnexal lymphocytic
infiltrate. liquefaction degeneration of the basal
layer of the epidermis, follicular plugging, and
hyperkeratosir. Direct immunofluorescence
* Wolaceouserythema afthe face andv-area
of lesional skin reveals the same pattern of aftheneck(Fig.l7,3).Thisissaidtoresemble
immunoglobulin deposition seen in SLE (see the colour of the heliotrope flower,and is
above). referred to as 'heliotrope erythema'

Potent fluorinated topical steroids are helpful
* Periorbitaloedema
Erythemaon thedorsaofthehands,and
linear erythema on thedorsa ofthe fingers
(Fig. 17.4). Erythematous papules (Gcmon's
in many cases, but ifthey are ineffective, intrale-
papules) over the knuckles
sional injection of triamcinolone, o r oral the- * Prominent,raggedcuticlesand dilated
rapy with the antimalarial chloroquine may be capillariesin the proximal nail folds (Fig. 17.5)
required. Light-exposed areas should be * EIythemaoverknees and elbows
protected by a sun-screen with a high SPF. * In childhood.cutaneousMsculitisleads to
Where there is extensive involvement of facial ulcerationof the skin.particularlyinthe
skin, the use of cosmetic camouflage can be of
axillae and groins

Subacute cutaneous lupus

Non-scarring, papulosquamous o r annular
lesions occur predominantly on light-exposed
areas. Associated systemic features may occur,
but are usually mild.


A flower resemblingthepole violet,
Which, with the Sun,though rooted-fost,doth
And,beingchooged,yet changeth not her love
Dermatomyositis is an autoimmune inflamma-
tory diseaseof sldnand muscle which may occur
in childhood or in adult life. There are differ-
ences in the manifestations of the disease in
these two agegroups.Vasculitisand the late de-
velopment of calcinosis are features ofthe child-
hood disease which are n o t seen in adults. In
some adults dermatomyositis is associated
with systemic malignancy, whereas there is no Fig. 17.3 Faciale~ytl~emaindermatomyositis
association with malignancy in the childhood
Dermatomyositis 15 1

Fig.17.4 Linear erythema on

the dorsa of the hands in

Fig.17.5 Hypertrophic cuticle,

and nail fold telangiectasia in

Muscles t o screen for malignancy in an individual patient.

In some cases there is little evidence of any The preferred approach appears t o be perfor-
muscle disease, whereas in others there is mance of limited screening, in the form of
profound muscle weakness. Typically, there is careful history, thorough physical examination
proximal, symmetrical weakness and wasting (rectal, lymph node, breast and pelvic examina-
of the limb girdle muscles. Pharyngeal and tions), full blood count, stool occult blood,
oesophagealmuscles may also be involved, lead- cervical smear and chest radiograph. In female
ing t o dysphagia. patients, ovarian computed tomography o r
Other features include pulmonary fibrosis, magnetic resonance imaging is advisable.
and arthralgia andlor arthritis. Further investigations should be carried out
There have been a number of studies of the in anyone with specific symptomatology.
association between adult dermatomyositis and
systemic malignancy, and these have produced Investigations
some controversy about the prevalence of this Electromyography and biopsy of affected mus-
association. However, there is no doubt that cles, measurement of serum creatine phospho-
some patients have an underlying malignancy, kinase, and a 24-h urine creatine level will help
and this raises the question of how extensively t o confirm the diagnosis.
152 Chapter 17: ConnectiveTissue Disorders

Fig. 17.6 Aplaqueof morphoea.

'ITeatment Morphoea
In dermatomyositis associatedwith malignancy, This isadisorderofunknownaetiologyin which
there is usually marked improvement when the there is sclerosis of the skin. I t may be sub-
neoplasm is excised.A relapse of the dermato- divided clinically into the following types:
myositis signals a recurrence. I Circumscribed.
The mainstay of therapy is oral corti- 2 Linear.
costeroids. If the response t o steroids is poor, 3 Frontoparietal (en coup de sabre).
immunosuppressives such as azathioprine, 4 Generalized.
methotrexate o r cyclophosphamide may be of
benefit. Where there is severe muscle involve- Circumscribed
ment, physiotherapy is an important adjunct t o This is the commonest clinical presentation
drugtherapy, in o~.dertominimizecontractures. of morphoea. Solitary 01-multiple indurated
plaques develop, predominantly on the trunlc
Initially, affected areas of skin have a violaceous
hue, but gradually become thickened and ivory
in colour (Fig. 17.6).The surface is smooth and
Scleroderma means'thiclening of the sl<in', and shiny. Eventually,usually after many months, the
is a term applied t o agroup of diseases in which sclerosis I-esolves, leaving atrophic, hyperpig-
there is sclerosis of the skin and destruction of mented areas.
hair folliclesand sweatglands. Scleroderma may
be an isolated cutaneous phenomenon, when it Linear
is called 'morphoea', o r a cutaneous compo- Linear morphoea usually affecrs one limb, often
nent ofa multisystem disorder. extending i t s full length. In childhood, it can sig-

[ c;A;.-.>zLrw. r-r.x.&
, , I , . . , j
, Z::+*'Q;';'&:"&&

- Morphoea: sclerosis oftheskinwithout * Chemicallyinduced sclerodema: sclerosis


systemic involvement
Systemicsclerosis mtaneoussclemsisin
associationwith a vasculopathy of small
oftheskinas amanifestalionofthe toxic
effectsof certain chemicals
* Pseudoscleroderma:sclerasiooftheskin
arteries produangmultiorgansystemic associateZwithanumberofdiseasesother
disease than morphaea orsy~temicsclemsis
1- ~~ ~ ~- ~ ~~
Scleroderma 153

nificantly impair the growth of the limb, and pro- Systemic involvement
duce severe flexion deformities of large joints Gastrointestinal. In the oesophagus, damage t o
and digits. the myenteric plexus leads t o hypomotility of
smooth muscle and later t o atrophy and
Frontoparietal (en coup de sabre) fibrosis, resulting in impaired peristalsis. The
Resemblingasabrecut acrossthe scalp and fore- gastro-oesophageal sphincter mechanism is
head, this type of morphoea is a considerable also impaired, leading t o gastro-oesophageal
cosmetic problem. A linear, depressed, sclerotic reflux, oesophagitis, and eventual stricture
area extends from the face into the scalp, and is formation. Symptoms of oesophageal reflux
associated with loss of hair along its length. are common. Dysphagia usually indicates the
development of oesophageal stricture.
Generalized Atrophy and fibrosis of the smooth muscle of
There is extensive sclerosis of the skin on the the small bowel leads t o impaired peristalsis,
trunk and limbs. Flexion contractures restrict and the resultant relative stagnation of small
limb movement, and if the chest is severely bowel contents predisposes t o bacterial over-
affected breathing may be impaired. growth as colonic bacteria move upstream into
the small intestine. Gut bacteria deconjugate
Deatrnent bile salts (which are essential for micelle forma-
There is no effective treatment for morphoea. tion) and this leads t o fat malabsorption and
In linear morphoea on the limbs, physiotherapy steatorrhoea. Occasionally, patients pre-
is essential t o maintain joint motility, and sent with a picture simulating acute intestinal
othopaedic surgery may be necessary. Plastic obstruction.
surgery can be of benefit in frontoparietal Similar pathology affects the large bowel
morphoea. and leads t o the formation of multiple wide-
The natural history of morphoea is gradual mouthed pseudodiverticula.
spontaneous resolution.
Pulmonary. An inflammatory alveolitis is fol-
Systemic sclerosis lowed by pulmonary fibrosis, and disease of
This is a condition of unknown aetiology in small pulmonary arteries leads t o pulmonary
which sclerotic changes in the skin occur as one hypertension and cor pulmonale.
component of a multisystem disorder associat-
ed with a vasculopathy of small arteries. The Renal. Fibrinoid changes in arteries and arteri-
skin changes affect predominantly the face and oles are associated with proteinuria and hyper-
hands. tension. Renal involvement is usually mild, but in

Face HandS
The fadal skin is sclerotic andbound to
underlyingsrmmues.pmducingatight, -
* Raynaudkphenomenon
abeaked nose,andresoictionofmouth
mntramres ofthe digits (sclercdactyly)
painfululcerr (Fig. 17.8).Theseinfarctiw
* Perioralfunwiing(pme-stringrnouth) changeslead topropssive pulpatrophyand
Facial telangiectasia resorptionofthe underlyingterminal
* Lossoflipvermilion phalanges
~~~ . .... .
154 Chapter 1 7: ConnectiveTissue Disorders

some cases it is severe and rapidly progressive, Cardiac. Myocardial fibrosis, conduction dis-
and leads t o renal failure. orders and a variety of electrocardiographic
(ECG) abnormalities have been described.
Nervous system. Neurological involvement is
uncommon, but carpal tunnel syndrome and Hepatic. There is a significant association
trigeminal neuropathy have been reported. between systemic sclerosis and primary biliary

Musculoskeletal. Arthralgia and arthritis occur in

some patients, and myopathy and inflammatory
myositis may also occur.

N o therapy is known t o alter the overall course
of systemic sclerosis, but many components of
the disease may be helped significantly by speci-
fic measures. Digital ischaemiamay be helped by
electrically heated gloves and socks. Calcium-
channel blockers may help relieve Raynaud's
phenomenon. Patients with oesophageal reflux
should avoid lying flat, and treatment with
proton-pump inhibitors may be very effective.
Broad-spectrum antibiotics are helpful in treat-
ing patients with gut bacterial overgrowth and

Severe pulmonary o r renal involvement are
poor prognostic factors, but the majority of
patients suffering from systemic sclerosis live
for many years.

Chemically induced scleroderma

Fig.17.7 Facial appearance in systemic sclerosis. Polyvinyl chloride (PVC) can induce a disorder

Fig.17.8 Finger pulp ulcers and

scars in systemic sclerosis.
Scleroderma 155

resembling idiopathic systemic sclerosis, and

'vinyl chloride disease' has been described in
workers in the PVC industry, particularly reac-
t o r cleaners. A number of other chemicals may
induce diseases mimicking systemic sclerosis,
including perchlorethylene and trichlorethyl-
ene (solvents used in dry-cleaning), and bleo-
mycin. A disorder similar t o systemic sclerosis
occurred in 198 1 in people poisoned by con-
taminated rape-seed oil sold as cooking oil in

Scleroderma-like changes may be seen in a
number of unrelated conditions, including
porphyria cutanea tarda, carcinoid syndrome
and phenylketonuria.

Fig. 17.9 Calcinosis in systemic sclerosis.


Mechanisms ofpruritus, 156 Causes oipruritus. l 5 6

There was a young belle ofold Natchez The sensation that we call itch is produced.
Whose garments were always in patches conditioned and appreciated at several levels
When comment arose in the nervous system: stimulus; mediators
On thestate afher clothes and receptors; peripheral pathways; central
She drowied:'When oh itchez,ah scratchez!' processing; interpretation. A wide variety of
(Ogden Nash. Requiem) stimuli can induce an itch, and a number o f
Pruritus means itching. Please note the correct chemicals may be involved, especially his-
spelling: it is not spelt pruritis as often appears in camine, prostaglandins and some proteinases.
student examination papers, clinical notes and However, the details remain obscure: although
referral letters! histamine can induce itch without weaiing, non-
Pruritus varies in duration, localization, and sedative antihistamines have no effect on simple
severity. Everyone hasexperienced short-term, pruritus.
localized, itch, and there is a perverse joy in hav- More complex, central mechanisms may also
ing a really good scratch. However, some indi- be important in modulating and appreciating
viduals suffer from distressing chronic irritation pruritus. Many itch-provoking stimuli induce
lasting for years. ltching may be restricted t o pain if applied at higher intensities. Indeed.
one o r more sites, o r cover virtually the whole scratchingappears to induce pain and toabolish
body surface. It may creep about, appearingtirst irritation. However, other sensory stimuli can
on an arm and later on the back, o r in more than also abolish itching, and more complex mecha-
one site simultaneously. ltching can be mild nisms have been proposed.One theory involves
o r appallingly severe, constant and distressing. a complicated filtering system controlling input
Chronic pruritus can completely ruin the qual- pathwaysto further stimuli and passing informa-
ity of life. tion on t o higher centres.
Pruritus is prominent in many skin diseases. ltching can certainly be affected by higher
Especially itchy are the eczemas, lichen pianus, centres. It is much less apparentwhen the mind
insect bites and infestations, urticaria and der- is fully occupied and much worse when bore-
matitis herpetiformis. However, the skin may dom sets in.4tressSandother psychologicalfac-
also itch when there is no rash. tors can induce orworsen pruritus.

Mechanisms of pruritus Causes of pruritus

W e d o not clearly understand why skin diseases The term'pruritus', used without qualification,
itch, and we know very little about irritation in implies that there i s itching without a primary
otherwise apparently nol-rnalskin. skin disorder However, in many instances there

Causes of pruritus 157

are considerable secondary skin changes from this is scabies (see Chapter 5). A full history
scratching (e.g. excoriations, scars and and a careful examination of the skin are there-
prurigo -see below). fore important in all patients complaining of
But watch out! Subtle changes are easily itching.
obscured by scratching: a classical example of In considering causes, we shall look sepa-
rately at localized itching, generalized states
and so-called'senile' pruritus.

Localized pruritus
Localized irritation of the skin is common.The
skin may be normal, but it is more common t o
find some abnormalities.
Two very important and troublesome
forms of localized pruritus are lichen simplex
chronicus and prurigo, and anogenital pruritus.

Lichen simplex chronicus and prurigo

This difficult problem is sometimes called
'neurodermatitis'. Constant irritation leads t o
constant scratching which, in turn, leads t o
thickeningofthe skin.This may occur in plaques,
known as lichen simplex chronicus (Fig. 18.1),
or in nodules, which are given the name prurigo
(Fig. 18.2).The areas are intensely irritable, and
a self-perpetuating itchlscratch cycle develops.
Patients who develop this kind of localized itch-
ing are often rather tense.

Sites of predilection. Lichen simplex chronicus-

classical sites include shins, forearms, palms and
Fig.18.1 Lichen simplexchronicus. the back of the neck (sometimes known as

Fig.18.2 Nodular prurigo.

1 58 Chapter 18: Pruritus

'lichen nuchae'); perianal and vulva1 skin may

also be affected (see below). Prurigo nodules-
may accompany areas of lichen simplex o r ap-
pear separately almost anywhere; they are
frequently multiple.
Lesions are often asymmetrical.

Treatment. Potent topical steroids (sometimes

under occlusive bandages) may help, but the
problem often recurs.

Anogenital pruritus
Two very common (and least talked about)
forms of localized itching are pruritus vulvae
and pruritus ani. They may be encountered
Treatment of perinea1 irritation depends
Pruritus ani is often attributed t o haemor-
upon the cause.
rhoids. However, although haemorrhoids and
tags are often present, their treatment alone
does not always relieve the symptoms. The
problem is also often dismissed as psycho-
logical, but only rarely is this the complete
* C0odhygiene.a highfibrediet.and
Anal itching may continue for years. Irritation lmeatmentwith topicalsteroids areuseful
is often spasmodic and extremely intense. The * 'IYeatingconcornitanthaemorrhoids may
majority of patients are male. reduce discharge

Clinical features. Examination often reveals little SkindhorderandaIleqiccmmctde-titia

abnormality; there may be some excoriation Mostwillrequiretopicalsteroids
and thickening of anal and perianal skin; 'tags'
are often present; occasionally gross changes
amounting t o lichen simplex are seen; there may diabetes
be an associated fissure.

Aetiology. Pruritus ani is probably largely a

low-grade irritant reaction t o faeces, sweat
Fatientsseldomrespond toantipruritics

and discharge; sedentary occupations make

matters worse. Contact allergy t o medica-
ments may be a factor, especially allergy t o local Generalized pruritus
anaesthetics and preservatives. Psoriasis of Generalized pruritus is extremely unpleasant,
the natal cleft and perineum may give rise t o and can either affect most of the body surface
pruritus ani. continuously o r involve several different areas.
Pruritus vulvae can be very distressing. There By definition, a primary skin disorder has been
are a number of causes t o consider. excluded.

Clinical features. Skin changes vary consider-

ably-nothing t o see at all; mild flakiness of
Causes of pruritus 159

the skin, with a few scratch marks; o r the other features of cholestatic liver disease, espe-
skin may be covered in excoriations, scars and cially in primary biliary cirrhosis.
nodules.The skin is often dry, especially in the
elderly. Chronic renalfailure
Although there may be no identifiable under- Unfortunately the intractable itch is largely un-
lying disorder, all patients with generalized irri- affected by dialysis. Parathyroidectomy is said t o
tation should be investigated because a number help, but the benefit is short-lived and is hardly
of potentially remediable systemic disorders justified in most patients.
may be responsible.
Thyroid disease
Both thyrotoxicosis and myxoedema may pre-
sent with pruritus. In myxoedema the general
dryness of the skin may be responsible.
Haematological disorders

- * Iron deficiency
Lymphoreticular malignancies are particularly
prone t o cause itching, but pruritus may also
Chdestpticliverdisease occur in association with a variety of carcin-

-- * Extrahepatic obstruction
omas. Up t o 30% of Hodgkin's disease patients

I- Hepatitis suffer from generalized pruritus.


I aronicrenaltaiiure
Various agents induce itching, but the mechan-
isms are poorly understood. Opiates appear t o
Thymiddisease act centrally and on mast cells. Oestrogens and

- * Thptoxicosis
phenothiazines induce cholestasis.

1 --
Malignancy Diabetes mellitus
Lymphomas andleukaemias You may come across lists quoting diabetes as a
Carcinomas cause of itching, but we do not consider that this
is the case.


Psychological factors
When everything else has been excluded,
psychological factors may be considered.
The most common underlying problem is an
Haematological disorders anxiety neurosis, but patients with mono-
Chronic iron deficiency may be due t o blood delusional psychoses such as parasitophobia
loss (e.g. from menorrhagia o r agut carcinoma). also itch.These individuals, however, offer their
Many elderly patients and some vegans are iron own explanation only too readily! (see Chapter
deficient for dietary reasons. Polycythaemia 20).
rubra vera is characteristically associated with
itching triggered by bathing.

Liver disease
The itch is probably related t o bile salts in the
skin. Irritation may precede the development of
160 Chapter 18: Pruritus

Screening procedures for generalized pruri- very severe and generalized. The patients (and
tus are as follows: their carers) are often anxious and miserable,
but this is usually secondary t o the irritation
rather than a primary cause.This state is called
'senile pruritus'. It is not known what causes
ageing skin t o itch.
! * A full history and geneal examination
, * Full blood count Examination
* ESR (orplasmaviscosity)
Liver functiontests
* Blood redurea niuogedcreatinine
The skin is texturally either'normal' or'dry'. Ex-
coriation~,secondary eczematization and areas
of infection are common. Localized areas of
* Ironstudies
Serum thyroxine
'eczema craquele' may develop (see Chapter 7).

* Chestx-ray neatment
Ifthese tests arenegativeinitially,mdifthe Treatment is extremely difficult. Sedative anti-
pruritus persists,repeatatintervals histamines often cause excessive drowsiness
and confusion, and topical steroids are of
limited use. If the skin is texturally 'dry', liberal
ITLeatment use of emollients may be helpful. Care has t o be
Treatment of generalized pruritus is that of its taken, however, as these agents can make both
cause. When no apparent underlying reason can the patient and their surroundings very slippery!
be found, a topical steroid and a sedative antihis- Increased frequency of washing o r the use of
tamine, such as hydroxyzine, may help. harsh soaps and detergents makes matters
worse, both by removing surface lipids and
Senile pruritus and xerosis acting as direct irritants. Soaps should therefore
ltchingwith no apparent cause is common in the be used sparingly and emollients employed
elderly. It may be mild and localized, but can be instead.
Systemic Disease
and the Skin

Endocrinedisease, l 6 1 Inflammatolyboweldkease, Cutaneousmanifestations

Thyroid disease, l62 164 ofsystemicmalignancy.
Adrenaldisease.163 Hyperlipidaemia,165 166
Rheumaticdkeases.l 6 3 Amyloidosis.16S Leukaemiaandthe skin, 168
Vitamin de~iciency.164 Sarcoidosis,165 Purpura,l68
Liverdiseaseand the skin, 166 AIDS and the skin, 168

Theskin may beinvolved directly o r indirectly in sions of necrobiosis lipoidica characteristically

anumberofsystemicdiseaseprocesses,andcan occur on the shins, although they may develop
providevisible diagnosticclues which could lead elsewhere. The lesions are initially erythema-
r o the discovery of internal disease. rous, but subsequently become yellowish-
brown and atrophic, and underlying blood
vessels are easily seen through the chinned skin
E n d o c r i n e disease (Fig. 19.2). Occasionally the lesions ulcerate.
N o t all patients with necrobloris lipoidica are
Diabetes diabetic. Good diabetic control does notappear
There are a number of cutaneous manifesta- t o influence the skin lesions.
tions of diabetes, includingthe following: Potent topical steroids and inwalerional
steroid injections are used in the treatment of
necrobiosis lipoidica, but results of treatment
are not very impressive.
1 1 CertainNtaneousinfecti'ons
2 Neuropathiculcen
3 Necrobiorirlipoidicadiabeticomm
I 4 Diabeticdermopathy.Thistermisapplied
t o small, brown, scar-like lesions seen on the
shins in some diabetics.The lesions are thought

4 Diabetic dermopathy
5 Diabeticbullae
6 Xanthomas I t o be associated with diabetic rnicroangiopathy.
5 Diabetic bullae. In this uncommon blister-
ing disorder of diabetics subepidermal bullae

7 Acanthosisnigicans
8 Lipoatrophy
9 Cheiroanhropathy
~~ ~~~
I occur on the hands and feet.Their aetiology is
6 Xanthomas. Hyperlipidaemia in uncon-
trolled diabetes may be associated with the
I Cutaneous infection. Mucosal candidiasis. development o f multiple small, yellow, erup-
particularly balanitis and vulvovaginitis, and tivexanthomas.
carbuncles, occur more frequently in diabetics. 7 Acanthosis nigricans. In association with
2 N e u r o p a t h i c ulcers. Impaired sensation. insulin resistance.
as a result of sensory neuropathy, predisposes 8 Lipoatrophy. Insulin-resistant diabetes as-
t o the development of neuropathic ulcers on sociated with partial o r genealized cutaneous
the soles ofthe feet (Fig. 19. I). lipoatrophy,
3 Necmbiosis lipoidica diabeticorum.Le-
162 Chapter 19: Systemic Disease and the Skin

Fig.19.1 Diabetic ncurapathic


Fig. 19.2 Necrabiosislipaidica


9 Cheiroarthropathy. A scleroderma-like
thickenine of the skin o f the hands in insulin-
dependent diabetics
Thyroid disease

The skin is typically dry, and feels thickened due
Granuloma annulare
t o subcuraneous mucin deposition-hence the
There is no significant association between
designation myxoedema. A malar flush on an
classical granuloma annulare and diabetes, but
otherwise pale face produces what has been re-
in a much rarer generalized form of granuloma
ferred to as a'rtrawberries and cream' appear-
annulare there is an association with diabetes.
ance.There may be a yellowish tinge t o the skin,
Typically, lesions of granuloma annulare are
said t o be due t o the deposition of carotenes.
groups of firm, skin-coloured papules, often
There is often periorbital oedema Scalp hair is
arranged in rings, and commonly occurring on
diffusely thinned and there is loss of the outer
the dorsa of the hands and feet (Fig. 19.3).The
part of the eyebrows. Sirting close t o the fire t o
natural history of granuloma annulare is
keep warm may produce severe erythema ab
eventual spontaneous resolution, but persis-
igne ('granny's tarran') on the shins.
tent lesions may be treated with intralerional
triamcinolone o r cryotherapy.
Rheumatic diseases 163

Fig. 19.3 Granulomaannulare.

Cutaneous changes which may accompany thy-
rotoxicosis include excessive sweating, palmar
erythema, diffuse alopecia, generalized hyper-
pigmentation and thyrotoxic acropachy (digital
clubbing). The nails may show onycholysis.
Some patienrs develop pretibial myxoedema.
which is produced by subcutaneous deposition
of excessive amounts o f mucopolysaccharide,
and ischaracterized by erythemaand thicl<ening
ofthesofttissues overthe shins and dorsaofthe
feet (Fig. 19.4).
Vitiligo may accompany autoimmune thyroid
disease, and generalized pruritus may be a fea-
tureof both hypo-and hyperthyroidism.

Adrenal disease

Cushing's syndrome
The cutaneous effects o f Cushing's syndrome
include thinning of the skin, spontaneous bruis-
ing, prominent striae on the trunl< and limbs.
diffusealopecia,acneand hirsutism. Fig. 19.4 Pretibialmyxoedema

Addison's disease
Diffuse hyperpigmentacion is the main cuta-
neous manifestation of Addison'r disease. The Rheumatic diseases
pigmentation is particularly prominent on the
buccal mucosaand in the palmarcreases.Vitiligo Gout
may also accompany autoimmune Addisonb In addition t o tophaceous deposits around af-
disease. fected joints,goutytophi may occuron theears.
164 Chapter 19: Systemic Disease and the Skin

Still's disease
Vitamin deficiency
This is a disorder of childhood, although it may
rarely occur in adults. Accompanying the
pyrexial episodes of Still's disease is a diffuse
The classical pictureofvitamin C (ascorbicacid)
maculopapular eruption which characteristi-
deficiency is rarely seen nowadays in developed
cally develops In the late afternoon and evening.
countries, but scurvy may be encountered in
and usually resolves by the following morning.
theelderly and in alcoholics,as a result of nutri-
Some slander-mongers claim that derma-
tional self-neglect. The typical appearance is
tologists never see this eruption because its
of perifollicular purpura, easy bruising, poor
periodicity is outside their normal working day!
wound healing and bleedinggums.

Rheumatoid arthritis
Dermatological features o f rheumatoid arthri-
Pellagra is the result of nicotinic acid deficiency.
tis include the following:
Classically, it has three major manifestations-
dermatitis, diarrhoea and dementia. The der-
matitis affects light-exposed areas, and there is
often a well-demarcated margin t o the affected

- Rheumatoid nodules.Subcutaneous
nodules overbany prominences,particularly
areaon the neck(Casal's necldace). Pellagramay
occur in alcoholics as a result of nutritional self-
neglect. A similar dermatitis may be provoked
on theextensoraspectofthe forearmsandthe
by isoniazid in individuals who are slow acetyla-
* Vasculitic lesions.Digitalvasculitismay tors ofthis drug.
produce smallinfarctsamundthenail folds
ulceration and even gangrene.Vasculitic Inflammatory bowel disease
lesionsmayalro m r o n thelegs,and

contribute to the dwelopmentoflegulcers
Fyoderma gangrenosum
* Palmarelytherna
Ulcerative colitir and Crohn's disease may be
associated with a number of mucocutaneous
manifestations includingthefollowing:

Rheumatic fever
Almost extinct in developed countries,
rheumatic fever may be accompanied by a
characterisitc eruption, erythema marginatum.
* Fyodenna gangenosum.The lesions may
be singleor multiple.Theyinitiallyresemble
Reiter's syndrome boils.whichsubsequentlybreakdown to form
Predominantly a disease of young adult males. necroticulcerswithund=-n~dpurple edges
Reiter's syndrome 1s usually precipitated by (Fig. 19.5).Fyodermagangrenosum may also
non-specific urethritis, but occasionally by accurin asrodationwith rheumatoid
bacillary dysentery. In addition t o urethritis. arthritis,myelomaand IeukaemixThe
matment ofchoiceis systemic steroids.but
conjunctivitis/uveitis and arthritis, there may
azathioprine, minocycline.clofazimineand
be an eruption which is indistinguishable from
psoriasis. O n the roles o f the feet the skin le-
sions may become extremely thickened, pro-
ciclosporin(cyc1osporin)may alsobeeffective
Perianalandbuccal mucosallesions.In
ducing so-called'keratoderma blenorrhagicum'. clohns disease.analexaminationmayrevea1
The buccal mucosa may show scattered ero- fleshytags.fissuresandperianalfistulae.The
sions, and superficial circumferential erosive buccal mucosamaybeoedematousand
changes on the penis are ~eferredtoas'circinate
resultof agranulomatouscheilitis
Sarcoidosir 165

Fig. 19.5 Pyoderma


Fig.19.6 Xanthelasma

lipidaemia (broad beta disease; dysbetalipo-

Hyperlipidaemia proteinaemia). Eruptivexanthomas arecrops of
yellowish papules which occur in association
Both primary and secondary hyperlipidaemic with marked hypertriglyceridaemia.
states may be associated with lipid deposits in
the skin, known as xanthomas. There are
several different clinical types of xanthomas. Amyloidosis
Orange-yellow lipid deposits in the eyelid skin
are known as xanthelasma (Fig. 19.6).Tuberous In systemic amyloidosis, amyloid deposits in the
xanthomas occur as yellowish nodules, usually tongue produce macroglossia, and cutaneous
over bony prominences (Fig. 19.7). Tendinous deposits are visible as yellowish, waxy, purpuric
xanthomas, as their namesuggests, are deposits plaquesaround theeyesand in the perianal area.
oflipid in associationwitYl tendons,often involv-
ing the Achilles tendons and extensor tendons
on the dorsa of the hands. Deposits of iipid Sarcoidosis
in the skin creases of the palms of the hands
(striate palmar xanchomar) appear t o be par- Sarcoidosis is a multisystem granulomatous
ticularly associated with primary type Ill hyper- disorder of unknown aetiology There are a
166 Chapter 19: Systemic Disease and the Skin

Fig. 19.7 Tuberaus xanthomas.

number of patterns of skin involvement in sar-

coidosis, including the following:
of systemic malignancy

Cutaneous metastases
Malignant tumours may metartarize t o the skin,
* Erythema nodosum.Tender.erythematous and tumours of renal, ovarian, gastrointestinal,

ncdulesanthelegs (seeChapterl6)
is involved in the granulomatousprocess. and
breast and bronchial origin are those most
likely to doso (Fig. 19.8). Cutaneous metastases
becomes swollen and purplish in colour usually presentas pink nodules, and occur most
* Scarsarcoid.Sarcoidgranulamas localizein frequently on thescalpand anteriortrunk. Scalp
oldscartissue,makingthe scarsprominent metasrases may produce areas of alopecia
Fapules.nodules and plaques.Theseoften (alopecia neoplastica).
have anorange-browncolour Lymphatic extension of carcinoma t o the skin
may ~ r o d u c ean area of ervthematous indura-
tion resemblingcellulitis,and lknown as'carcino-
Liver disease and the skin
. .
ma ervri~eloides'.
Metastasis of ovarian or gastrointestinal car-
Changes in the skin and nails which occur in as-
cinoma via the ligamentum terer can present as
sociation with chronic liver disease include the
an umbilical nodule (Sisterjoseph's nodule).

* Palmareqhema jaundice,patientswithlongstanding

* P~ritus:incholestaticliverdisease
cholestatic liver disease may also have
marked melaninpigmentation.Patients
sufferinefrom haemochramatosishave
* Xanthelasma:inp"marybiliarycirrhDsis generalized bronze-brownpigmentation
whitenails (Terry's nails) which isproducedbva combinationof iron
Pigmentary changes:in addition to and melanin
Cutaneous manifestations of systemic malignancy 167

Fig.19.8 Cutaneous metastasis

from carcinoma of the

Fig.19.9 Acanthosis nigricans.

Miscellaneouscutaneous signs of 4 T h r o m b o p h l e b i t i s migrans. This is

underlying malignancy particularly associated with carcinoma of the
I D e r m a t o m y o s i t i s (see Chapter 17). pancreas.
2 A c a n t h o s i s nigricans. This is a warty, hy- 5 A c q u i r e d ichthyosis. lchthyosis develop-
perpigmented thickening of skin in the flexures ing for the first time in adult life may be asso-
(Fig. I9.9).The palms ofthe hands may also be af- ciated with a lymphoma.
fected, producing an appearance known as'tripe 6 P y o d e r m a gangrenosum.This may occur
palms'. The commonest associated malignancy with myeloma and leukaemia.
is an adenocarcinoma of the gastrointestinal 7 N e c r o l y t i c m i g r a t o r y e r y t h e m a . This is
tract. However, 'malignant' acanthosis nigricans a distinctive eruption associated with pan-
is rare, whereas flexural acanthosis nigricans is creatic glucagonoma.
common in the obese and is unrelated t o sys- 8 Flushing and a rosacea-like eruption are
temic problems. cutaneous features of the carcinoid syndrome.
3 G e n e r a l i z e d pruritus. Generalized itch- 9 E r y t h e m a g y r a t u m repens.This rare skin
ing, without a rash, may be associated with a marker of malignancy is a bizarre eruption
wide variety o f systemic malignancies. whose appearance resembles wood grain.
168 Chapter 19: Systemic Disease and the Skin

10 Acquired hypertrichosis lanuginosa. I Oral candidiasis extending into the

The sudden growth of profuse vellus hair over oesophagus.
the face and body is a rare sign of underlying 2 Oral 'hairy leul~oplal&-white ridges along
neoplastic disease. the sides of the tongue, caused by Epstein-Barr
3 Seborrhoeic dermatitis: this is often severe,
and is probably related t o an altered host re-
sponse toMalassezia yeas-.
There are numerous cutaneous changes which 4 Itchy folliculitis: the aetiology of this non-
may accompany leul~aemia,o r be provoked by specific pruriticfolliculitis is unknown.
the drugs used in i t s treatment. 5 Staphylococcal infection, shingles, mollus-
Common presenting features of acute cum contagiosum and dermatophyce fungal in-
leulkaemia include purpura, bruising, and bleed- fection occur more commonly in AIDS patients.
ingfrom the gums, and the skin may be directly 6 Episodes of herpes simplex are more fre-
involved in the form of leulaemic infiltrates. quent and more severe, and lesions may be-
Disseminated herpes zostcr (herpes zoster come chronic.
with numerous outlying vesicles) may accom- 7 Perianal warts tend t o be more florid and
pany leukaemia, as may a severe bullous form of more difficult t o treat.
pyoderma gangrenosum, and Sweet's disease 8 Kaposi's sarcoma: a tumour which is thought
(acute febrile neutrophilic dermatosis). t o arise from vascular endothelium and is
related t o infection with human herpesvirus
type 8 (HHV-8) infection. Lesions are usually
Purpura multiple, and may affect any part of the sldn, as
well as Internal organs. It is rarely the cause of
Purpura is produced by extravaration of red death in AIDS patients, who usually succumb
cells intothe sl<in,andhas numerous causes.The to intercurrent infection. It is a radiosensitive
lesions do not blanch on pressure. tumour.
Causes of purpura include vasculitis 9 Preexisting psoriasis may become severe
(see Chapter 16). quantitative or qualitative and extensive in AIDS patients.
platelet abnormalities, drugs, amyloidosis, 10 Bacillary angiomatoris. Caused by the
dysproteinaemias and infections (e.g. bacillus Bartanella henselae, these angioma-like
meningococcaemia). lesions affect the skin, mucosae and inter-
nal organs. They respond t o treatment with
AIDS and the skin I I Drug-associated problems.The antiretrovi-
ral treatment now widely employed in treating
Patients sufferingfrom the acquired immunode- HIV infection may provole rashes and cause nail
ficiency syndrome (AIDS) are at increased risk pigmentation.
of developing a number of mucocutaneous
Skin and the Psyche

Dematitisartefacta.169 Delusions ofparasitosis Obsessive-compulsivehabits,

Dematologicalpathomimicry. (parasitophobia), 172
171 171
Dermatological non-disease
(dysmoqhophobia),l 7 1

lfyou happento have a wart on your nose or have marked psychological effects. Patients feel
forehead,you cannot help imaginingthat no one unclean, and these feelings can persist long after
in the world has anything else to do but store at the problem has been eradicated.
your warr laugh at it,and condemn you for it, There are some skin disorders which are
even though you hove discoveredAmerica. directly related t o psychological problems, and
(Fyodor Dostoevsky, The Idiot) these are described below.
Patients with skin disease who often ask'ls it
caused by nerves doctor?, are usually trying t o
establish if they can attribute their sldn condi- Dermatitis artefacta
tion to'stress'. In fact, very few skin disorders
are directly related t o psychological distur- Patients with dermatitis artefacta produce skin
bance. However, there is evidence that psoriasis lesions t o satisfy a psychological need, but what
and atopic eczema may be exacerbated by benefit they derive from their actions is usually
stress, and other conditions in which emotional not obvious. There is no rational motive for
stress has been claimed t o play a part in some their behaviour.They will vehemently deny that
cases include alopecia areata and acute the lesions are self-induced if challenged. As a
pompholyx. group they are distinct from malingerers, who
There is no doubt chat skin disease has psy- consciously imitate o r produce an illness for a
chological effects on the patient, and can signifi- deliberate end.
cantly adversely affect their quality of life. Skin Dermatitis artefacta iscommoner in women.
disease is visible t o others. it carries the raint of and most o f those affected are adolescents o r
contagion, and affected individuals often feel youngadults. However, there is a subgroup with
sdgmatized and have poor self-image and low an older age o f onset who are more likely t o be
self-esteem.They will be aware that their skin is male. Many have some connection with the
being scrutinized, and that any form of physical health professions, either directly o r via family
contact, such as shaking hands o r collecting members.
change, may provol<eapprehension in others. In Artefactual sldn lesions may be produced in a
certain ethnic groups where marriages are number of different ways including rubbing.
arranged,thepresenceofskin diseasemaycom- scratching, picking,gouging, puncturing, cutting.
promise marriage prospects, and cause con- sucking, biting, the application of heat o r caus-
siderable emotional distress. tics, o r the injection of milk, blood and faecal
Infections with ectoparasites sometimes material. Limb oedema may be simulated by the
170 Chapter 20: Skin and the Psyche

intermittent application of constricting bands treatment, relatives and friends of the patient
(Secretan's syndrome). Lesions tend t o have quite naturally rally t o their support, and may be
bizarregeometric shapes which do not conform somewhat vocal in their criticism of a seemingly
t o those seen in naturally occurring disease- inept medical profession. Other doctors caring
no dermatosis has square, rectangular o r trian- for the patient may also be convinced that their
gular lesions. Often the lesions are more disease is naturally occurring. This situation, in
numerous on the side of the body opposite the which other individuals'support' a patient with
dominant hand. If a caustic material such as an dermatitis artefacta, is known as 'folie a deux',
acid has been used t o induce lesions this may and is also encountered in delusions of parasito-
trickle off the main area of damage t o produce sis (see below).
the'drip-sign' of tell-tale streaks a t the margins. The psychopathology of patients who pro-
Even when suspected artefactual lesions duce artefactual lesions is not uniform, but in
are covered with occlusive dressings, patients some cases there is a demonstrable personality
will often manage t o insert knitting needles disorder, and in others significant depression.
under the dressings, o r push sharp instruments It requires considerable expertise t o be able
through them, in order t o continue damaging t o make a confident diagnosis of dermatitis
the skin. The history obtained from these artefacta, but even experienced dermatologists
patients is devoid of any useful information see cases in which they suspect the lesions are
about the evolution of their lesions.The impres- self-induced, but they cannot be certain. Alter-
sion conveyed is that one minute the skin was natively, there are occasional cases in which
normal, and the next it was blemished. This there is a strong suspicion of dermatitis arte-
so-called 'hollow history' is characteristic, as is facts, but the lesions are discovered t o be the
a striking complacency about what are often result of naturally occurring disease.
extremely disfiguring lesions ('10 belle indifer- Treatment is extremely difficult in many
ence'), sometimes accompanied by an enigmatic cases. Confronting the patient with the diagno-
'Mona Lisa' smile. One patient we have seen, sis is counterproductive, in that it usually
who had extensive suppuration of the left arm, produces a categorical denial that they are
probably produced by the inoculation of faeces producingthe lesions, and subsequentfailure t o
(Fig. 20. l), said about her arm 'Yes, it is rather attend for follow-up.
unpleasant isn't it. I wonder if you could arrange Strict occlusion of the traumatized areas may
for someone t o take it off'. allow healing, but the lesions will reappear as
When they see the severity of the lesions and soon as occlusive dressings are removed.
an apparent lack of progress in diagnosis and An alarming consequence of occlusion may be

Fig.20.1 Dermatitis artefacta-

in this case probably the result
of inoculation of faeces into the
Delusions of parasitosis (parasitophobia) 17 1

the appearance of lesions elsewhere, o r the altered texture of scalp hair; the belief chat
development of other 'symptoms', as if t o they are the source of an offensive odour, usu-
compensate for inability t o reach the usual ally from the axillae o r feet. These delusional
sites. Antidepressants will help those who are beliefs o r perceptions of abnormal sensations
depressed. Psychiatric referral is often unhelp- are a consuming preoccupation for the patient.
ful and, unfortunately, many patients refuse Perinea1symptoms in men includecomplaints o f
assistance from a psychiatrist. The situation a red, burning scrotum, and the female equiva-
often remains at stalemate. As long as the lent is a burning discomfort in the vulva (vulvo-
doctor's suspicions are n o t voiced, the patient dynia). There is nothing abnormal t o see on
appears content t o continue attending for examination.
follow-up. In some cares depression is part of the
The course of this disorder is often pro- picture; others may be suffering from mono-
tracted. Recovery often has little t o do with symptomatic hypochondriacal psychosis.There
successful medical treatment, but occurs be- i s a significant risk of suicide. Management is
cause of increasingmaturity, marriage o r having d i f f i c u l ~but some patients respond t o treac-
children. ment with antidepressantsand psychotherapy.

Dermatological pathomimicry Delusions of parasitosis

Dermatological pathomimicry is distinct from
dermatitis artefacta. Patients with this condi- The individual with delusions of parasitosis
tion either deliberately perpetuate their sLin has an unshakeable conviction that their skin
disease, or reproduce a pre-existing skin disor- is infested with parasites, An experienced
der. Having been appraised o f the aetiology of dermatologist will recognize this disorder from
their skin disease, they use this knowledge t o information supplied in the referral letter, and
reproduce the lesions when under emotional willoften arrange tosee the padentattheend of
stress, t o obtain sympathy, o r in an effort to a clinic, because the consulration is usually ex-
avoid an unpleasant situation with which they tremely lengthy. However, prior t o seeing a
cannot cope. Examples of the type of illness dermatologist the patient has often consulted
used by patients for pathomimicry include their local university department of zoology or
allergic contact dermatitis, drug reactions and a medical entomologist in an attemptto identify
chronic leg ulceration. the'parasites'.They will also probably be known
Sympathetic discussion with the patient will t o companies specializing in pest eradication.
usually solve the problem. who will have been asked t o disinfest their
home.They may have isolated themselves from
family and friends because of their fear o f pass-
Dermatological non-disease ing the'infestation'on t o them.
(dysmorphophobia) Because of their absolute conviction that
they are infected they may have convinced their
In this condition patients complain of severe family, friends, and even the family doctor, ofthe
symptomatology localized t o certain parts of reality of the problem (shared delusion; folie a
the body, most commonly the face, scalp and deux).
perineum, but without any objective evidence Patients often describe a feeling of itching.
of disease.The complaints includedysaesthesias biting o r 'crawling' in the skin, and state that
such as burning, itching o r throbbing pain; too when this occurs they areabie t o remove asmall
much o r too little hair on the face o r scalp, o r 'insect' o r 'worm' from a skin 'lesion'. When
172 Chapter 20: Skin and the Psyche

Fig.20.2 Trichotillomania

asked to demonstrate typical sl<in'lesions', they thing. Patients often refuse psychiatric help be-
will often point t o Campbell de Morgan spots. cause they do notacceptthatthey havea mental
frecl4es or other minor blemishes. Typical illness, and cannot see how a psychiatrist could
'specimens' are presented to the doctor possibly help with what, t o them, is a physical
wrapped in pieces of paper o r adhesive tape. disorder.The neumleptic drugpimozide may be
and kept in a matchbox. These should always of benefit, if the patient can be persuaded t o
be examined under the microscope, because take it.
they just might contain parasites, but usually
they contain fragments of cotton o r sl<indebris.
I t is impossible t o persuade there patients Obsessive-compulsive habits
that parasites are not responsible fortheir con-
dition. If they are shown that their'specimens' Trichotillomania
are simple debris they remain unconvinced, and Trichotillomania means compulsive plucking of
may even suggest that the parasites are so small hair.The scalp is involved most often, but the
that an electron microscope will be required t o eyebrows and eyelashes may be affected.A mild
demonstrate them. In this situation the most form of trichotillomania may be obsel-ved in
lucid, eloquent discourse will fall upon deaf libraries, where engrossed students compul-
ears-the patient's beliefs remain unshaken, sively twist locks of hair around their fingers,
and the doctor usually retires from the conflict but they rarely pull it out unless examinations
feeling more than somewhat jaded. areapproaching!The clinical picture is of patch-
Deiusionsofparasitosismayoccurin associa- es of hair loss containing hairs ofvarying length.
tion with organic brain disease such as senile Often the crown of the head is affected, and the
dementia and cerebral arteriosclerosis, and has hairatthemargins is ofnol-mal length (Fig. 20.2).
been described in pellagra,vitamin 6,deficiency The undel-lyingscalp is usually normal, but may
and following coronary bypass surgery. The be excoriated.
term 'monosymptomatic hypochondriacal psy- Trichotillomania in childhood is often tran-
chosis' may be applied t o patients with a single. sient. However, it may bea manifestation of sig-
fixed delusion, and most pacients with delusions nificant psychopathology. particularly in adults.
of parasitosisfit into this category.
Effective treatment is very difficult, and many Neurotic excoriations
patients continue with their delusion for years. This disorder is encountered predominantly in
A confrontational approach rarely achieves any- middle-aged women.The lesions are produced
Obsessive-compulsivehabits 1 73

by picking and gouging, and are usually scattered Patients with this problem have obsessive-
over the arms, upper trunk and face. More re- compulsive personalities, and picking the skin
cent lesions are usually interspersed with scars appearst o provide relief of unconscious aggres-
from previous excoriations. Acne excoriee is a sion and tension.
variant of this condition in which minimal acne Antidepressants and supportive psychother-
lesions are repeatedly picked and gouged, leav- apy may be of benefit.
ing scars when the lesions heal.
Cutaneous Drug Reactions

Introduction, l 7 4 Drugreaction patterns, 174 Conclusion,l79

There are only two types ofdrug-those thot

don't work and those thot have side-efects.
(Bruno Handel FRCS)
* Antiiioties (especiallypenicillin,

Introduction -
semisyntheticpenicis and sulfonamides)
Non-stemidal anti-inflammatolydmgs
* Hypnoticr I1
The skin is one of the commonest sites for un- * Tranquillizers
wanted drug effects (a better term than 'side-
effects'), although estimates of the frequency isn't), atest may n o t be appropriate because the
vary considerably. Cutaneous drug reactions reaction is n o t t o the drug itself, but to a drug-
are probably under-reported and often go un- complex o r metabolite that is produced in viva
recognized. Note, though, that skin disorders after ingestion.
wholly unrelated t o drug ingestion can be la- The only definitive test is direct challenge
belled erroneously. It is important not t o jump with the suspected agent, but this may be im-
t o conclusions:we have seen viral infections and possible o r unethical in many circumstances.
scabies labelled as drug reactions. There are For these reasons, proving that a specific erup-
many people who state they are 'allergic t o tion was due t o a specific drug is difficult, and
penicillin', butwho are not. judgements usually have t o be made on clinical
Unfortunately there are no reliable in vitro grounds alone.
testsforestablishingthatarash is duetoadrug.
Simple in vivo tests, such as prick testing and
patch testing, havealimited placeinspecificsitu- Drug reaction patterns
ations, butusually yield no useful information.
Cutaneous drug reactions may be due t o However, all is not lost! Some drugs are much
several different mechanisms. more prone to induce cutaneous drug reac-
Note, however, that even ifthe mechanism(s) tion patterns than others. Common offenders
for a particular reaction is known (and it often include:
Drug reaction patterns 175

Furthermore, there are a number of well- and maculopapular (Fig. 2 1.1): there is often a
defined clinical drug reaction patterns. strong resemblance t o a viral exanthem. The
Some of these patterns are more drug time relationship is variable: in most instances
specific, and therefore recognition of them as the rash begins afew days after startingthe drug,
drug reactions may help t o identify the culprit. but it may begin almost immediately, o r be de-
layed for a few weeks. Exanthematic eruptions
Exanthematic eruptions usually fade a week or so after stopping the
The commonest cutaneous drug reactions are drug, but exfoliative dermatitis may develop
itchy, widespread, symmetrical, erythematous (see below and Chapter 15).

Fig. 21.1 A typical exanthematic

eruption due to an antibiotic.
1 76 Chapter 2 l :Cutaneous Drug Reactions

Common causes. Non-steroidal anti-inflamma- (lignocaine)); topical antihistamines; topical an-

tory drugs and antibiotics, particularly tibiotics, especially aminoglycosides, in creams
ampicillin, other semisynthetic penicillins, sul- and drops; increasingly,topical steroids.
fonamides and gentamicin.
Rarer causes. Gold, barbiturates and Drugs are one of the four important causes of
phenothiazines. exfoliative dermatitis (see Chapter 1 5).

Urticaria and anaphylaxis Common causes. Prominent offenders are sul-

(see also Chapter 15) fonamides and sulfonylureas, gold, phenytoin,
Drug-induced urticaria may be due t o a direct allopurinol and barbiturates.
pharmacological action on mast cells, o r t o a
type Io r type Ill hypersensitivity reaction. Vasculitis (see Chapter 16)
Occasionally, drugs may trigger a major ana- Drug ingestion is a common trigger for
phylactic reaction, with o r without urticaria, vasculitis.
which can be fatal unless treated very rapidly.
Unfortunately, there is no known way of pre- Common causes. Thiazides, captopril, cimeti-
dicting this disaster. dine, quinidine, sulfonamides.

Common causes. Aspirin, opiates (direct), peni- Fixed drug eruptions

cillin~, cephalosporins, pollen vaccines and Fixed drug eruptions are one of the most
toxoids (immune). curious events encountered in dermatological
practice. The reaction occurs in the same
Eczema place(s) every time the offending drug is taken.
Type IV hypersensitivity reactions t o topical They are often misdiagnosed as recurrent
medicaments are common, and give rise t o a eczema o r ringworm.
contact dermatitis (see Chapter 7). Figure 2 1.2 A round o r oval patch of dusky erythema de-
shows a woman who was given eye drops velops, often with a purplish centre (Fig. 2 1.3),
containing an aminoglycoside. Occasionally, a and sometimes a central bulla. This fades t o
topically sensitized patient may receive the leave postinflammatory hyperpigmentation.
compound (or a closely related chemical) sys- There may be only one lesion o r multiple sites.
temically. The result is a severe, widespread, Fixed eruptions can occur anywhere, but the
eczematous reaction. limbs and genitalia are favoured sites.

Common causes. Lanolin in creams and bandages; Common causes. Laxatives containing phenolph-
preservatives (parabens, ethylenediamine) in thalein, sulfonamides, dapsone, tetracyclines,
creams; topical anaesthetics (not lidocaine barbiturates.

Fig. 21.2 Contact sensitivity to

Drug reaction patterns 177

Fig. 21.3 Fixed drug reaction to

a sulfonamide.

Lichen planus-like eruptions Some drugs also exacerbate pre-existing acne

Lichen planus-like (sometimes known as (see below).
'lichenoid') reactions are rare, but can be se-
vere.The eruption is occasionally indistinguish- Hair abnormalities
able from idiopathic lichen planus, but more As discussed in Chapter 13, drugs may be re-
commonly there is an eczematous element, and sponsible for hair loss o r excessive hair growth.
there is much more scaling. In severe cases an
exfoliative dermatitis may develop (see above Pigmentary changes
and Chapter 15). Several drugs cause pigmentary changes
(Table 2 l.l).
Causes. Antimalarial drugs; some beta-blockers; Heavy metals such as silver may be deposited
sulfonylureas; gold. Thiazides may cause lichen in the skin following industrial exposure o r
planus-likeeruptions on light-exposed surfaces. ingestion (e.g. in antismoking lozenges).

Erythema multiforme (see Chapter 1 5) Bullous reactions

So many things seem t o be able t o trigger There are several ways in which drugs may
erythema multiforme that it is usually difficult t o induce blistering.
be certain whether a drug is responsible.

Suggested drug causes. Barbiturates; long-acting

sulfonamides; cotrimoxazole; rifampicin.

Acneiform eruptions . InfueddNgeNptions

* D N ~ Smay induce pemphigusand
Skin changes resembling acne vulgaris occur pernphipid (see Chapter 14)
with several drugs. The changes tend t o be * D ~ g may
s exacerbateporphyriacutanea
monomorphic, consisting largely of papulo-
pustules.There are seldom comedones present. -
phototoxicbullous reaction
Causes. Corticosteroids (both topical and * Barbituratesmaybeassodatedwithbullae
onbony prorninences.usuallyin patients
systemic), adrenocorticotrophic hormone
unconscious due to overdose
(ACTH), androgenic drugs, lithium and iodides.
178 Chapter 2 l : Cutaneous Drug Reactions

Table 21.1 Drugs causing

cutaneous pigmentary
Characteristic Clofazirnine(usedin leprosy)
YellOW Mepanine
Furplish Chlorpmmazine
Blue-black Chlomquine(npciallyoshins)
Minoqcline (iihigh dosage)
Cwmgem (= chloasma)
~ ~~~ ~~

There are three main types of reaction.

* Exacerbationofunderlyingdisease
1 Photoalleraicreadon

In phototoxic reactions, the dose of the drug

and the intensity of ultraviolet exposure may
both be important: if critical levels are not
reached the reaction may not develop.This can
be confusing if the drug has been taken on a
number of occasions.
Patients complain that exposure t o the sun
causes a burning sensation followed by ery-
thema, swelling and, later, eczematous changes
on light-exposed areas (Fig. 2 1.4).

Common causes. Phenothiazines; sulfonamides; Fig. 21.4 Photosensitivityto a sulfonamide.

tetracyclines; thiazides. Demethylchlortetracy-
cline can cause photo-onycholysis. Bullaedue t o Causes. Many agents have been incriminated,
nalidixic acid have been mentioned above. including hydralazine, isoniazid, penicillin, mino-
cycline, procainamideand griseofulvin.
Lupus erythematosus-like
syndrome Exacerbationof
A rare but important drug reaction is the induc- pre-existing disease
tion of a syndrome closely resembling systemic Some drugs may produce a deterioration
lupus erythematosus. in certain skin disorders. Notable examples are:
Conclusion 179

1 Acne-androgenic drugs (e.g. danazol, drug provoked, and what the causitive agent
stanozoloi), oral contraceptives and cortico- might be.
2 Porphyrias-all clinical features, including
cutaneous photosensitivity. may be worsened
by drug ingestion, particularly barbiturates and

I --
3 Psoriasis-lithium, antirnaiarials. * Acarefulexamination
4 Systemic lupus erythematosus-penicillin
and sulfonamides may produce deterioration.

- Elimination ofother skindiseases

Recognition of the clinical reaction pattern
* Matchingthe reactionwiththemostlikely

Conclusion -offenderand
Tests,where appropriate (possiblyincluding
If you use all the clinical informatio'n at your dis-
posal it is often possible t o determine if a rash is
Tveatrnent of Skin Disease

Bases,180 Communicationand patient Quantitiesprescribed,181

cornpliance,l8l Topicalsteraids,182

Ifit's dry, wet it qit's wet dry it.Congratulations, gredient (or ingredients) and a material in which
you ore now a dermatologist! (Anonymous) this is suspended-a base.These components
The above witticism is oft-quoted by non- must be compatible.There is little point in dis-
dermatologists as an assessment of the scope covering a new base which penetrates the sldn
of dermatological therapeutics. An alternative lile a hot lknife through butter if it completely
calumny relates t o a dermatologist murmuring inactivates everything suspended in it.
an unintelligible Latin name as a diagnosis and The stratum corncum forms a natural pro-
then prescribing a topical steroid, for every- tective barrier t o penetration of externally ap-
thing. Apart from being deeply offensive to plied agents. Hence, t o facilitate penetration by
sensitive skin doctors, both these quips are far a drug, this barrier function must be breached.
from the truth, as dermatologists have an enor- and this can be achieved by hydration of the
mous therapeutic armamentarium at their dis- stratum corneum-for example, penetration
posal. In days of yore, itmust beadmitted, many of a topical steroid may be markedly enhanced
of the available topical therapies resembled by occluding an area of sldn with polyrhene.
witches brews containing'Eye of newt and toe Unfortunately, if large areas of rldn are occluded
of frog, wool of bat and tongue of dog'. They in this way the amount ofsteroid absorbed may
were often cosmetically unacceptable and mal- be sufficient t o produce systemic effects. Bases
odorous-if the sldn disease did n o t render the containing urea also hydrate the stratum
patient a social pariah, the treatment could be corneum and enhance penetration of their
relied upon t o do so. However, in recent years, active ingredients. Dimethyl sulfoxide (DMSO)
topical therapies have n o t only become more is a solvent which penetrates sldn extremely
effective, but also cosmetically much more rapidly, and is used as a vehicle for the antiviral
acceptable. agent idoxuridine.
The treatment of individual disorders has
been dealt with in preceding chapters, and this
chapter is designed t o provide an overview oi Bases
the principles oftopical therapy.
An ideal topical preparation for the manage- Bases include creams, oily creams, ointments,
ment of skin disease would penetrate well, but lot~onr,gels and pastes. A cream is an oil-in-
remain localized within the skin, thereby avoid- water emulsion which is relatively non-greasy
ing potential problems from systemic effects. In and has only limited emollient activity. Creams
practice this is extremely difficult t o achieve, a l r cosmetically acceptable and can be used t o
and any agent which penetrates the stratum treat either moist o r dry skin conditions. Oily
comeum is absorbed t o some extent. creams are water-in-oil emulsions which com-
Topical preparations consist of an active in. bine good emollient properties with cosmetic
Quantities prescribed 18 1

acceptability and are tllerefore of benefit in dry oughtto be matched by the provision of precise
skin conditions. Ointments aregreasy prepara- Instructions by the doctor.Verbal instructions
tions which have emollient and occlusive arenotsufficientif multiple topical therapiesare
properties.The occlusive effect of an ointment prescribed. For example, a patient suffering
results in hydration of thestratumcorneumand from psoriasis might be given a tar shampoo, a
enhanced penetration o f the active ingredientof steroid scalp lotion.amild topical steroid cream
the ointment.The benefits of ointments are off- t o use in the flexures, and a dithranol prepara-
set by a lack of cosmetic acceptability. Oint- tionfor short contact therapy t o plaqueson the
menrsare messy and stickto clothing. If used on trunk and limbs. If the patient has only recently
the hands theytransferto everythingtouched- developed psoriasis, and is not familiar with its
an obvious disadvantage to someone employed treatment, the pmvision of multiple therapies
in clerical work, for example. Lotions are fluid without clear instructions could easily lead t o
preparations which have a cooling effect due t o confusion.
evaporation.They are useful in the management D o not expect patients who depart for work
of moist, exudative skin lesions, and also in der- at the craclc of dawn t o adhere strictly t o in-
matoses affecting the scalp. Clear, non-greasy structions to wash their hair every morning
gels are designed for use on hairy parts of the and use a topical medication twice daily Modify
body, where they are cosmetically acceptable. the treatment schedule t o suit the individual.
Pastes are powders, usually mixed with soft If you are prescribing a preparation which is
paraffin, and are protective-for example, In messy t o use andlor malodorous, warn the pa-
the prevention of maceration ofthesldn around tient about this. For example, dithranol stains
adischarging ulcer. and benzoyl peroxide bleaches, and lack of
Thechoiceofa particular base should be de- prior warning could lead t o ruined clothingand
termined by the cype o f skin problem and the bed-sheets.
sites affected. It is, for example, wholly inappro-
priate t o prescribe a steroid ointment for day-
time use on the scalp, because it is too messyA Quantities prescribed
gel o r lotion preparation should be used in-
stead. Similarly, a lotion is not the correct base It is importantwhen prescribing topical therapy
for ichthyotic skin, where an oily cream o r oint- t o consider the area t o be covered and thefre-
ment are more appropriate. qucncy of application before assessingthe quan-
Bases are mixtures of several components. tity of a topical agent required by the patient.
formulated t o provide stability and freedom For example, there is little point in prescribing
from microb~alcontamination. Random dilution a 30-g tube of an emollient t o be used over the
of a topical preparation will dilute the preserva- entire body surface after bathing-a repeat
tives in the base and significantly shorten its prescription would be required after one appli-
shelf-life. cation, because this is the approximate amount
necessary for a single application over the
whole body surfaceofan adukTopical therapies
Communication and are available in a variety of container sizes.You
patient compliance will need tochecktheavailablesizes before pre-
scribing, as they vary from product t o product.
Most non-topical medication involves popping Topical steroids, for example, may be marketed
pillsofvarious colours into the mouth atcertain in 15-. 25-, 30-, 50- or 100-g tubes, depending
times of the day, requiring a minimum of effort on the manufacturer and the steroid. Most
and only a minor feat o f memory.Topical ther- emollients are available in 50- and 100-g tubes
apy demands a great deal more of the patient. and 500-g tubs o r dispensers.
and the increased effort required ofthe patient Underprescribing and overprescribing are
182 Chapter22 TreaunentofSkin Disease

both common. One does n o t require IOOg of psoriasis of the scalp, flexures, hands and feet.
cream t o treat a small patch of eczema on the and discoid lupus erythematosus. In general,
leg-most of the tube will languish in a drawer a severe dermatosis should be treated with a
o r bathroom cabinet until its shelf-life is long potentsteroid, and a mild condition with a weak
expired, o r it may be inappropriately used by steroid. In the case o f a chronic dermatosis
another member of the family. subject t o periodic exacerbations a mild t o
moderate potency steroid can be used when
the condition i s quiescent, and a potent pre-
Topical steroids paration when it worsens.
There are regional variations in the absorp-
A t first sight the huge number of available topi- tion of topical steroids through the skin and
cal steroids is bewildering t o the uninitiated. their potential for local adverse effects. These
butwith a little knowledgeand experiencetheir variations are determined by the thickness of
use is quite straightforward. They are divided the stratum corneum, occlusion, for example in
into several groups according t o potency. the flexures where skin surfaces are in apposi-
Hydrocortisone preparations are the weakest. tion, and the vascuiarity of the area. Most facial
However, hydrocortisone in a base contain- dermatoser should only be treated with mild
ing urea, which enhances penetration of topical steroids, although a few conditions such
the stratum corneum, is moderately potent. as discoid lupus eryrhematosus will require po-
Modification of the basic steroid skeleton by tent preparations. Sldn disease affecting the ax-
fluorination (fluorinated steroids) o r esterifica- illae, groins and submammary areas should also
tion produces steroids of much greater potency betreated with mild topical steroids. Converse-
(Table 22.1). ly, dermatoses ofthe palms and soles, where the
stratum corneum is extremely thick, require
Choice of preparation potent steroids, and a greater benefit is often
The most appropriate topical steroid for a obtained if polythene occlusion is used t o en-
given situation should be determined by the hance penetration.
typeandseverity ofthecondition beingtreated, There is agreater riskofadverse systemic ef-
the sites affected,and theage ofthe patient.The fects from the use of topical steroids in c h i i d ~ m
skin disorders which are steroid responsive because of the high ratio of skin surface area t o
have been delineated in previous chapters, and bodyvolume,particularly in infants Forthis rea-
include various types of eczema, lichen planus, son mild topical steroidsshould be used in small

Table 22.1 Steroid potency

Mild 1% Hydroconisone
Moderately potent Clobetasonebutyrate (Eumovate)
Alclometasone dipropionate(Mcdrasone)
Hydmconisone with urea (Alphadem)
Potent Betamethasonevalelate(Bemovate)
fluwinolone acetonide(Symlar)
Fluocinanide (Metosyn)
~ Hydrocortisonebutyrare(loccid)
Veypotent Clobetasolpropionatepennovate)
Topical steroids 183

children.The skin of the elderly is thin, and po- steroids on the face for lengthy periods of
tent steroids will amplify this change-their use time-often inappropriately for mild acne on
over long periods of time should therefore be the chin.The eruption consists of small papules
avoided o r carefully supervised. and pustules on an erythematous background
(Fig. 22.2).The history is virtually identical in all
Side-effects cases. Initially the mild acne appears t o improve,
Side-effects are rarely seen following the use of
mild topical steroids, but they are encountered
more frequently in association with potent
topical steroid use, although much less common-
ly than in the early years of steroid availability.
Side-effects may be divided into local, occur-
ring at the site of application of the steroid,
and systemic, resulting from percutaneous

Local side-effects
Atrophy of the skin
Topical steroids produce dose-related thinning
of the dermis.This effect is particularly notice-
able in areas where the skin is naturally relative-
ly thin, such as the axillae, medial aspect of the
upper arm, groins and the medial aspect of the
thigh. Prominent striae may develop in these
areas (Fig. 22.1). O n the face, cutaneous thin-
ning and telangiectasia produce prominent ery-
thema. Frequent use of potent steroids on the
dorsa of the hands and the forearms results in
easy bruising, particularly in the elderly.

Perioral dermatitis
Perioral dermatitis is a condition usually seen in Fig.22.1 Prominent striae in the axilla following
young women who have used potent topical use of a potent topical steroid.

Fig.22.2 Perioral dermatitis.

184 Chapter 22: Treatment of Skin Disease

probably because the vasoconstrictor action of Pustular psoriasis

the steroid reduces erythema, and inflamma- If large quantities of a potent topical steroid are
tory papules become less noticeable. However, used inappropriately t o treat psoriasis, and the
stopping treatment results in a rebound flare of treatment is then suddenly stopped, the psoria-
the erythema, and the patient therefore consid- sis may exacerbate dramatically, and pustular
ers the treatment is keepingthe condition con- psoriasis may occur.
trolled and continues t o apply the steroid; she
may even increase the frequency of application. Infection
Eventually, as the eruption around her mouth Folliculitis may occur in areas treated with topi-
becomes more noticeable, she asks her doctor cal steroids, particularly when ointments o r
for 'something stronger', and is often given a polythene occlusion are used, and the use of
more potent topical steroid. steroids on moist, warm flexural areas may en-
Treatment consists of explaining the nature courage superinfection with Candida. Inappro-
of the condition, stopping the potent topical priate use of topical steroids on dermatophyte
steroid, warning the patient about the rebound fungal infections alters the appearance of the
flare of erythema, and prescribing a mild topical eruption, producing so-called 'tinea incognito'
steroid ( I % hydrocortisone) for 2-3 weeks t o (see Chapter 4). Scabies inappropriately treated
reduce its severity. In addition, oxytetracycline with topical steroids becomes extremely florid,
should be given in a dose of 500 mg bd; gradually with many burrows and a very numerous mite
reducing over a period of several weeks as the population (see Chapter 5).
condition improves.The reasonfor its efficacy is
unknown. Systemic side-effects
Topical steroids are absorbed through the skin,
Steroidrosacea and excessive use of potent steroids may result
Topical steroids will worsen pre-existing in inhibition of the pituitary-adrenal axis and
rosacea, and can precipitate a rosacea-like iatrogenic Cushing's syndrome. This problem
eruption. is rarely encountered nowadays, because those
prescribing potent steroids have become more
Allergic contact dermatitis familiar with their potential adverse effects, and
Allergic contact dermatitis t o topical steroids restrict the amounts prescribed.
may develop, usually in patients usingthem long- In children, growth retardation is an impor-
term, for example in the treatment of atopic tant consequence of the long-term use of
dermatitis (see Chapter 7). potent topical steroids.

Note: Page numbers in bold refer t o tables. Page numbers in italics refer t o figures.

acantholysis 123 adrenaline (epinephrine) 132, 132 anhidrotic ectoderrnal dysplasia 106
acantholytic cells 123 adverse drug reactions see drug animal mites 47
acanthosis 68 reactions anogenital pruritus 158, 158
acanthosis nigricans 16 1, 167, 16 7 AIDS 168 anthralin (dithranol), psoriasis 73,
hyperpigmentation 1 13 albinism 1 10 74
aciclovir therapy, eczema herpeticum alginate dressing 143 antibiotics
25 allergic contact dermatitis acne vulgaris 53
acne (vulgaris) diagnosis 62-3 drug reactions 175, 175-6
age of onset and course 48 topical steroids causing 184 rosacea 56-7
assessment 54 treatment 63 antidepressants 1 7 1
clinical features 49,49-5 1,50,5 1 allergic vasculitis (leucocytoclastic antiflea agents 46
exacerbation by drugs 179 vasculitis) 144, 144-5 antifungal agents, topical 32-3,36
family 48 alopecia antihistamines
infantileljuvenile 48-9 androgenetic 1 15 senile pruritus 160
late-onset 49 cicatricial (scarring) 1 17- 18, 1 18 urticaria 132, 132
lesions 50 traction 1 17, 117 antinuclear antibodies, SLE l48
management 54 see also hair loss antiphospholipid syndrome 149
myths 54 alopecia areata 1 14, 116, 1 16- 17 antipruritic, topical 39
pathogenesis 5 1,s 1,52 alopecia totalis 1 17 antiretroviral drugs 168
psychological impact 49 alopecia universalis I 1 7, 1 18 antiseptics, topical 53
severe (acne conglobata) 50,s 1 amyloidosis 165 acne 53
severity assessment 54 anaemia, pernicious 1 13 anxiety neurosis 159
site and distribution 49 anaesthetic, local, skin biopsy 14 apocrine sweat glands 3
systemic symptoms (acne fulminans) anaesthetic lesions, leprosy 2 1 apple jelly nodules, lupus vulgaris 20
51 anagen 4,5 arrector pili muscles 3-4
treatment 53,53 anaphylactoid purpura arsenic 87
acne, secondary 54-5 (Henoch-Schonlein purpura) artefactual skin lesions 169
drug-induced 55 144-5 arthropathic psoriasis 7 5 4 7 6
oil-induced 55 anaphylaxis 176 arthropod bites 45-7
acne conglobata 50,s 1 Anderson-Fabry disease arthropods, bullae 123
acne excoriee (desjeunes filles) 56, (angiokeratoma corporis ash leaf macules 104, 1 10
173 diffusum) 107, 10 8 asteatotic eczema (eczema craquelk)
acne fulminans 5 1 androgenetic alopecia (common 66,66
acneiform disorders 55-7 balding) 1 15, 1 16 athlete's foot, (tinea pedis) 28-9,29
drug-induced 55 androgens 3 atopic eczema (dermatitis) 63-4,64
acneiform eruptions, drug-induced sebum production increase 5 complications 63
55,177 angel's kisses 98 treatment 64
acne keloidalis 56 angioedema 130-2 atopy 63
acne rosacea 56-7 hereditary 13 1, 132 atrophie blanche 142, 142
acral melanoma 9 1 treatment 132 atrophy of skin, topical steroids 183
acrochordons (skin tags) 80 angiofibromas 104 atypical mycobacteria 20
acrodermatitis continua 70 facial 105 autoantibodies
acrodermatitis enteropathica 107 angiokeratoma corporis diffusum alopecia areata 1 16
acrokeratosis verruciformis 104 (Anderson-Fabry disease) antiphospholipid syndrome 149
actinic dermatitis, chronic 139 107, l 0 8 pemphigus 123
actinic (solar) keratoses 85,86 angioma (Campbell de Morgan) 82, SLE 148
actinic prurigo 139 82 autoirnmunity, derrnatomyositis 150
acute bacterial paronychia 35 angiomatosis axillary body odour (axillary
Addison's disease 163 bacillary 168 bromhidrosis) 3
hyperpigmentation 1 13 neonatal 101 axillary bromhidrosis 3
adenoma sebaceum 104 angiomatous naevi 99, 10 1, 101 azathioprine, psoriasis 74
adrenal disease 163 angular cheilitis (perleche) 34,34 azelaic acid I I I
186 Index

bacillary angiomatosis 168 subepidermal 125,126,126 cheilitis, angular (perlkhe) -34

bacterial infections 17-22 see also bullae cheiroarthropathy 162
erythrasma 19,19 blood tests 12 chemicals
mycobacterial 19-22 blue naevus 98,99 hypopigmentation due t o I I I
staphylococcal 17- 19 body odour 3 scleroderma due t o 154-5
streptococcal 17 boils (furunculosis) 18 chickenpox 26
see also individual infections Bowenf disease (squamous cell chilblains (perniosis) 145-6, 146
bacterial overgrowth 153 carcinoma in situ) 85-7,86 children, steroid adverse effects
balanitis Breslow thickness 9 1,92 l82-3,184
Candida infection 35 brittle psoriasis 7 1, 71,75 Chinese herbal therapy 64
circinate 164 buccal mucosal candidiasis 34 chloasma (melasma) 1 12, 113
balanitis xerotica obliterans 135-6 buckles, nickel dermatitis 6 1 chloracne 55
balding bullae 122 cholecalciferol (vitamin D) 7
male-pattern 3, 1 15, 1 16 causes 122, 122-3 cholinergic urticaria 13 1
in women 1 16 diabetic 16 1 chondrodermatitis nodularis helicis
banana scalpel 38 insect bites causing 45, 123 83,83
basal cell carcinoma (rodent ulcer) 84, tense 123, 125 'Christmas tree' lines 136
84-5 see also blisters chromate 62
differential diagnosis 143 bullous disorders 122-9 chromosomal abnormalities 108,108
Gorlin's syndrome 105-6 acquired 1 2 2 4 chronic actinic dermatitis 139
treatment 85 causes 122, 122-8 chronic paronychia 35
variants 85 congenital 122 cicatricial alopecia 1 17-1 8, 1 18
basal cell naevus syndrome (Gorlinf drug-induced 123,177,177 cicatricial pemphigoid l l8, 126, 126
syndrome) 105-6 rarer diseases 128-9 differential diagnosis 125-6
basal cell papillomas (seborrhoeic bullous erythema multiforme ciclosporin (cyclosporin) 74
keratoses; seborrhoeic warts) (Stevens-Johnson syndrome) atopic eczema 64
79,79-80 129, 133,133 Cimex leaularius (bed bugs) 46-7
basal layer 2 bullous impetigo 18 circinate balanitis 164
basement membrane 2 bullous pemphigoid 125-6, 126, 126 clothing lice (Pediculus humanus) 43,
Bazin's disease 20 appearance l 2 5 44
Beau's lines 6, 1 19 differential diagnosis 125-6 coal tar 73
beauty spots 96-7 immunofluorescence 126 cold sores (herpes labialis) 25
Becker's naevus 94 butterfly rash 148 collarette, peripheral 1 36, 13 7
bed bugs (Cirnex leaularius) 46-7 Bywaters' lesions 164 collodion baby 103, 103
Behget's disease 145 colophony 6 1
benigntumours, of skin 79-83 cafe-au-lait patches 1 1 2, 113 comedones 50
dermal 81-3 calcinosis, systemi(sclerosis 155 common warts 22-3
angioma (Campbell de Morgan) calcipotriol, in psoriasis 74 communication 10
82,82 calluses 23 over treatment 18 1
dermatofibroma (histiocytoma) Campbell de Morgan spot (angioma) compliance, with therapy 18 1
81,81-2 82,82 compression bandaging 142-3
pyogenicgranuloma 82,82-3 cancer, systemic see tumours condylomata acuminata (genital warts)
epidermal 79-8 1 Candida albicans 28,34 234
epidermal cysts 8 1,8 1 Candida infection (candidiasis) 33-5 congenital erythropoietic porphyria
keratoacanthoma (molluscum angular cheilitis (perleche) 34,34 (Giinfher's disease) 139
sebaceum) 80,80 balanitis/vulvovaginitis 35, 16 1 connective tissue disorders 148-55
seborrhoeic keratoses 79,79-80 buccal mucosal 34 dermatomyositis 150-2
skin tags (acrochordons) 80 chronic paronychia 34-535 lupus erythematosus see lupus
fibroblastic 83 in diabetes 16 1 erythematosus
melanocytic 8 1 intertrigo 35 scleroderma 152-5
benzoyl peroxide 53 oral, inAIDS 168 see also individual disorders
benzyl benzoate emulsion 3 9 4 0 pruritus vulvae 158 contact dermatitis 62
beta-carotene, hyperpigmentation capillary naevi drug-induced 176, 176
113 deep (port wine stains) 98-9,100 occupational 62
biopsy 13- 14 superficial 9 7 4 , 1 0 0 see also allergic contact dermatitis
incisional/excisional see carbuncle 18 corns 23
incisional/excisionaI biopsy carcinogens 83,87 Corynebacteriumrninutissirnum 1 9
punch 14,15 carcinoma erysipeloides 166 Coxsackie virus infection 24
tumours 77 'carpet-tack' sign 149 crab lice (Pthirus pubis) 43-5,44
bird fleas 46 Casal's necklace 164 clinical features 44-5
bird mites 47 catagen 4,s eyelashes 45,45
birthmarks 94 cattle ringworm 3 1,33 treatment 45
blackheads (open comedones) 50 cautery, skin tumours 7 7 , 7 7 4 creams 180
blistering diseases see bullous cavernous (angiomatous) naevi 99, oily l80
disorders 101 Crohn's disease 164, 164
blisters 122 cellulitis 17, 18 Crowe's sign 1 04
arthropod bites causing 45, 123 cement dermatitis 62 crusted (Norwegian) scabies 40-1
drug-induced 123 ceramide trihexoside l07 clinical features 40
Index 187

Down's syndrome 40,4 1 dermatitis artefacta 169-7 1, 170 pigmentary disorders 177, 178
institutional outbreaks 4 1 dermatitis herpetiformis 126-8, 127, pruritus 159
treatment 40- 1 l28 systemic lupus erythematosus (SLE)
cryotherapy immunofluorescence 128 149
hypopigmentation I I I pathological findings 127 urticaria 176
tumours 78,78 dermatofibroma (histiocytoma) 8 1, vasculitis 176
warts 22-3 81-2 drug reactions 174, 174-9
curettage andlor cautery ('C&C'), skin dermatofibrosarcoma protuberans 92 causes 174
tumours 77.77-8 dermatoglyphics 6, 7 exacerbation of pre-existing disease
Gushing's syndrome 54, 163 dermatological assessment I I 178-9
steroid side-effects 184 dermatological history 10, 10 exanthematic eruptions 175, 175-6
cutaneous herpes simplex 25 dermatological non-disease fixed eruptions 176-7, 177
cutaneous metastases 166-8,16 7, (dysmorphophobia) 17 1 lichenoid (lichen planus-like) 177
168 dermatological pathomimicry 1 7 1 light-induced 139
cutaneous tuberculosis 19 dermatomyositis 150-2, 15 1 patterns 174-9, 175
cuticle muscles 1 5 1-2 Duplex sonography, venous leg ulcers
hair 4 skin changes 150, 150 142
nail 5-6 dermatophyte infections 28-33 dysbetalipoproteinaemia 165
cyclosporin (ciclosporin) 74 cattle ringworm 3 1,33 dysmorphophobia (dermatological
cyproterone acetate 53 diagnosis 32 non-disease) 17 1
cyst(s) 'ide' reaction 32 dysplasia 83
acne vulgaris 50 kerion 3 1,32 dysplasticlmalignanttumours, of skin
epidermal 81 mycetoma (Madura foot) 33 dermal 92-3
epidermoid 8 1 treatment 32-3 Kaposi's sarcoma 92-3
keratin (milia) 8 1,8 1 see also entries beginningtinea lymphomas 92-3
myxoid 120,12 1 dermatophytes 28 epidermal 84-7
pilar (trichilemmal) 8 1 dermatosis 62 actinic (solar) keratoses 85,86
cystic fibrosis 3 juvenile plantar 66,66 basal cell see basal cell carcinoma
cytotoxic drugs dermis 6 (rodent ulcer)
adverse effects 74 tumours involving 78 prevention 9 1-2
psoriasis 74 dermoepidermal junction 96 squamous cell see squamous cell
dermographism 1 3 1, 13 1 carcinoma (SCC)
danazol 132 desmosomes 2 melanocytic tumours 89-9 1
dapsone 127 diabetes mellitus 16 1, 16 1-2 lentigo maligna (Hutchinson's
Darier's disease (keratosis follicularis) candidal balanitis/vulvovaginitis 35, malignant freckle) 89,89,90
1 04,104 16 1 melanoma see melanoma,
delayed hypersensitivity 60, 176 pruritus 159 malignant (MM)
delusions of parasitosis (parasitosis) diabetic bullae 16 1 prevention 9 1-2
171-2 diabetic dermopathy 16 1 dysplastic naevus 98
dendritic cells 2 diagnosis of skin disease 9-1 6 dysplastic skin changes 8 3 4
depigmentation l 10, l l I dermatological history 10
dermabrasion, acne vulgaris 53 diagnostic process 9-1 0 eccrine sweat glands 3
dermal papillae 6 value 9,9 absence 106
dermal tumours see also examination; investigation ectodermal dysplasia, anhidrotic
benign 8 1-3 dimethyl sulfoxide (DMSO) 180 l06
see also benign tumours dioxin exposure 55 ectoparasite infections 37-47
malignant 92-3 discoid eczema 65 crusted (Norwegian) scabies 40- 1,
see also dysplasticlmalignant discoid lupus erythematosus 1 18, 41
tumours 149,149-50 papular urticaria 45-7
dermatitis disseminated zoster 26 pediculosis 4 1-5
allergic contact see allergic contact dithranol (anthralin), psoriasis 73,74 psychological effects 169
dermatitis Down's syndrome 108 scabies 3740,39
atopic see atopic eczema crusted (Norwegian) scabies 40,4 1 see also individual infections
(dermatitis) drug-induced conditions eczema 58-66,59
chronic actinic 139 acne 55 asteatotic (eczema craquele) 66,66
exfoliative see erythroderma acneiform lesions 55, 177 atopic see atopic eczema
hair dye 62 blisters 123 (dermatitis)
nickel 60-1,6 1 bullous disorders 123, 177, 177 classification 58,59
occupational contact 62 contact dermatitis 176, 176 clinical features 58,59
occupational irritant 60 eczema 176 discoid 65
in pellagra 164 erythema multiforme 177 drug-induced 176
perioral 57 erythroderma (exfoliative endogenous 5 8 , 6 3 4
topical steroids 183, 1 83-4 dermatitis) 176 palms and soles 65-6
plant 62 hair abnormalities l 7 7 exogenous 58,5943
primary irritant 59-60,60 hair loss I 16 lichenified 58,59
rubber 61 hypopigmentation I I I photosensitive 139
topical medicaments causing 62 lupus erythematosus 178 varicose 65,142
see also eczema photosensitivity 178, 178, 178 see also dermatitis
188 Index

eczema craquele (asteatotic eczema) eumelanins 5 'goose pimples' 4

66,66,160 Eurax-Hydrocortisonecream 39 Gorlin's syndrome (basal cell naevus
eczema herpeticum (Kaposi's examination, dermatological II, syndrome) 105-6
varicelliform eruption) 25-6, 11-12 Gottron's papules 150
26 skin lesion assessment II gout 163
Ehlers-Danlos syndrome 104 exanthematic eruptions 175, 175-6 'granny's tartan' 162
elderly excisional biopsy see granular layer 2-3
benign skin tumours 79,80 incisionallexcisional biopsy granuloma
herpes zoster 26 exfoliativedermatitis see fish tank 20,2 1
senile pruritus 157, l60 erythroderma pyogenic 82,82-3
steroid adverse effects 183 exogenous eczema 59-63 sarcoid 166
emollients eyelash infection,crab lice (Pthirus swimming pool 20
atopic eczema 64 pubis) 45,45 granuloma annulare 162, 163
psoriasis 73 granulomatosis,Wegener's 146
senile pruritus 160 facial erythema 148, 149 griseofulvin 33
en coup de sabre (frontoparietal facial features Giinther's disease (congenital
morphoea) 153 hypothyroidism 162 erythropoietic porphyria) 139
endocrine disease 16 1-2 SLE 148 guttate psoriasis 70,70
ephelides (freckles) 8 1 systemic sclerosis 153, 154 treatment 75
epidemic typhus 43 fibroblasts, dermis 6
epidermal appendages fibromas, periungual 104,105 haemangiectatic hypertrophy 99
apocrine sweat glands 3 fingerprints 6 haematologicaldisorders 143
eccrine sweat glands 3 fish tankgranuloma 20,2 1 pruritus 159
hair see hair fixed drug eruptions 176, 177 haemochromatosis 1 13
nails see nail(s) fleas 46,46 nail changes 166
sebaceous glands 5 flexural psoriasis 71, 71,75 Haernophilus influenzae 17
epidermal cysts 8 1 treatment 75 hair 3-5
epidermal growth factor (EGF) I flucloxacillin,carbuncle 18 colour 5
epidermallorganoidnaevi 94 5-fluorouracil 85 drug-induced abnormalities 177
epidermal transit time 1,67 flushing 167 'exclamation mark' 1 16, 117
epidermal tumours 78 folie a deux 1 70 greying 5, 1 14
benign 79-81 folliculitis 17-1 8 growth cycle 4-5, 1 15
see also benign tumours AIDS 168 microscopy 13
dysplasticlmalignant 84-7 Pityrosporurn 56 pigment 4,5
see also dysplastic/malignant topical steroids causing 184 structure 4,4
tumours foot types 3
epidermis 1-6 athlete's (tinea pedis) 28-9,29 vellus 3, 168
epidermoid cysts 8 1 ischaemic ulcers 143 hair disorders 1 1 4 - 1 8
epiloia l l 0 Madura (mycetoma) 33 colour 1 14
epinephrine (adrenaline) 132, 132 see also soles excessive hair 1 18
equestrian cold panniculitis 145-6 Fordyce spots 5 loss of hair see hair loss
eruptions see drug reactions freckles (ephelides) 8 1 textural abnormalities 1 14-15
erysipelas 17 fungal dystrophy,toenail 30,3 1 hair dye dermatitis 62
erythema fungal infections 28-36 hair follicles 3,5
dermatomyositis 150, 150, 15 1 Candida infection see Candida hair loss 1 15-1 8
heliotrope 150, 150 infection (candidiasis) generalized 1 18
violaceous 150 dermatophyteinfections 28-33 scalp 115-18
erythemaannulare 29 pityriasisversicolor 35-6,36 abnormal scalp skin 1 15, 1 17-1 8
erythemagyratum repens 167 see also individualinfections acquired 1 15, 1 15-1 6
erythemamultiforme 132, 132, fungal mycelium 33 complete 1 16
1 32-3 furunculosis (boils) 18 congenital 1 15, 1 15
bullous (Stevens-Johnson drug-induced 1 16
syndrome) 129,133,133 Gardner's syndrome 106 normal scalp skin 1 15, 1 16-1 7
drug-induced 177 gastrointestinal tract, in systemic scarring with 1 1 5
herpes simplex triggering 25 sclerosis 153 see also alopecia
orf lesions as trigger 24 gels, non-greasy 181 hairy leukoplakia,oral 168
triggers 24, 132 genital herpes 25 halo naevus 98,98
erythema nodosum 145,145,145 genitalia, scabies 37 hamartomatous malformationssee
in inflammatory bowel disease 164 genital warts (condylomata acuminata) naevi (naevus)
in sarcoidosis 166 23-4 hand, ringworm 29-30,30
erythrasma 19,19 giant cell arteritis (temporal arteritis) hand, foot and mouth disease 24,25
erythroderma (exfoliativedermatitis) 147 hands, systemic sclerosis 153, 154
7,133,1334 gingivostomatitis, primary herpetic 24 Hansen's disease (leprosy) see
drug-induced 176 glaucoma, congenital 99 leprosy
erythrodermic psoriasis 7 1-2 glutaraldehyde, warts 22 head lice (Pediculuscapitis) 4 1-3,42
treatment 75 gluten-sensitiveenteropathy 126 clinical features 42-3
erythromycin, acne vulgaris 53 glycosaminoglycans 6 treatment 43
erythroplasiaof Queyrat 85 'goose flesh' 4 heat bumps 45-6
Index 189

heliotrope 150 hypopituitarism 1 18 plug 23,24,24

Henoch-Schonlein purpura hypothyroidism 162 in keratoacanthoma 80,80
(anaphylactoid purpura) hair loss 1 18 keratin cysts (milia) 8 1,8 1
144-5 keratinization I
hepatitis B, urticaria in 13 1 ichthyoses 102-3, 103 disorders 102
herald patches 136 acquired, with lymphoma 167 keratinocyte I
hereditary angioedema 13 1,132 autosomal dominant (ichthyosis keratoacanthoma (molluscum
herpesgestationis 1 37 vulgaris) 102 sebaceum) 80,80
herpes gladiatorum 25 ichthyosiformerythroderma 102-3 keratoderma, palmo-plantar (tylosis)
herpes labialis (cold sores) 25 X-linked 102 103
herpes simplex 24-6 ichthyosiformerythroderma 102-3 keratodermablennorrhagicum 75,76,
AIDS 168 ichthyosisvulgaris (autosomal l64
eczema herpeticum 25-6,26 dominant ichthyosis) 102 keratohyalin granules 3
erythema multiforme 25 'ide' reaction 32 keratolytic agents 73
labial 25 idoxuridine 180 keratoses
primary 24-5 immunofluorescence seborrhoeic (seborrhoeicwarts)
recurrent 25 bullous pemphigoid 126 79,79-80
Herpes virus horninis 24 pemphigus 124, 125 solar (actinic) 85,86
herpes zoster (shingles) 26,267 SLE 148-9 keratosis follicularis (Darier's disease)
disseminated 26 immunofluorescence,dermatitis 1 04,104
motor 27 herpetiformis 128 keratosis pilaris 5 5 5 6
ophthalmic 27,27 impetigo 19 kerion 3 1,32
sacral 27 bullous 18 ketoconazole shampoo 65
treatment 27 head lice 42 Klinefelter's syndrome 108
trigeminal 27 non-bullous 18 Kobner (isomorphic) phenomenon
herpetic gingivostomatitis, primary 24 incisionallexcisional biopsy 1 3- 14, 15 68
herpetic whitlow 25 equipment 14 koilonychia 119
hidradenitis suppurativa 55,55 technique 1 3-1 4, 14
hirsutism 1 18, 1 18 incontinentiapigmenti 108, 1 1 2 'lo be/leindifference' 1 70
histiocytoma(dermatofibroma) 8 1, infants lamellar granules (Odland bodies) 3
8 1-2 acne vulgaris 48-9 Langerhans'cells 2,7
history taking 10, 10 scabies treatment 40 lanolin, reactions to 176
holocrine glands 5 inflammatory bowel disease 164, 164 lanugo hair 3
horripilation 4 inflammatory scalp ringworm 3 1,32 laser therapy 79
housewives' hand dermatitis 59 lngram regime 73 Lassar's paste 73
Hutchinson's malignantfreckle (lentigo ingrowingnails 12 1 latex gloves, dermatitis 6 1
maligna) 89,89,90 inherited disorders of skin 102-8 laxatives, fixed drug eruptions 176
hydrocolloid dressing 143 insect bites 45-7 LS etA see lichen sclerosus et
hydrocortisone 182 insecticide,flea control 46 atrophicus
hydrogel dressing 143 interleukins, atopic eczema 63 legs, venous system 14 1
hydroxycarbamide,psoriasis 74 intermediatefilaments I leg ulcers 14 1-3
hydroxyurea, psoriasis 74 intertrigo 35 haematologicaldisorders 143
hyfrecator 78 investigation,dermatological 12- 15 ischaemic 143
hyperlipidaemia 16 1, 164, 165 'iris' ('target') lesion 1 32, 132 neoplastic 143
hyperpigmentation 1 12-1 3 iron deficiency, chronic 159 vasculitic 143
acanthosis nigricans 167, 167 irritants, dermatitis 59-60,60 venous 141,141-3,143
acquired 1 12-1 3 ischaemic ulcers, leg 143 lentigines 8 1, 105
causes 1 1 2- 13 isomorphic (Kobner) phenomenon lentigo, senile 79-80
congenital 1 12 68 lentigo maligna (Hutchinson's
postinflammatory 1 12- 1 3, 176 isotretinoin ( l 3-cis retinoic acid) 53, malignant freckle) 89,89,
vascular disorders 142 54 90
hyperthyroidism 163 itchingsee pruritus LEOPARD syndrome,
hypertrichosis 1 18, 1 18 itraconazolepulse therapy 33 hyperpigmentation 1 12
hypertrichosislanuginosa 168 ivermectin (Mectizan) 40- 1 lepromatousleprosy 2 1-2,22
hypertrophic scars and keloids 83 lepromin test 2 1
hypochondriacalpsychosis 172 jewellery, nickel dermatitis 6 1 leprosy (Hansen's disease) 20-2,22
hypochromic naevi 1 10 juvenile acne vulgaris 48-9 borderline 2 1,22
hypopigmentation 1 10, 1 10-1 1 juvenile plantar dermatosis 66,66 indeterminate I I I
acquired 1 10-1 1 juvenile spring eruption 1 39 lepromatous 2 1-2,22
causes l l 0 polar forms 2 1
congenital 1 10 Kaposi's sarcoma 92-3 spectrum 22
drugs and chemicals causing I 1 I AlDS 168 tuberculoid 21-2,22, l l l
leprosy 2 1 Kaposi's varicelliform eruption lesions, types, characteristics 12, I 2
pityriasis alba I I I, II 2 (eczema herpeticum) 25-6,26 leucocytoclastic vasculitis (allergic
pityriasis versicolor 35, 1 1 1 Kasabach-Merrittsyndrome 99 vasculitis) 144, 144-5
postinflammatory I I I keloids 83 leukaemia, cutaneous presenting
tuberculoid leprosy I I I keratin 1,s features 168
tuberous sclerosis 1 10 dermatophytegrowth 28 leukoplakia,oral hairy 168
190 Index

lice malignant melanoma see melanoma, mosaic warts 23.23

clothing 43 malignant (MM) motor zoster 27
crab (Pthirus pubis) 43-5 malignant tumours see mucocutaneousfeatures,
head see head lice (Pediculus capitis) dysplasticlmalignanttumours; inflammatory bowel disease
lichenification 58,63 tumours l64
lichenified eczema 58,59 Mantoux reaction 20 mucopolysaccharides
lichen nitidus 135 Marjolin's ulcer 142 (glycosaminoglycans) 6
lichen nuchae 158 mast cell naevi 10 1 mucosal lesions, in inflammatory
lichenoid (lichen planus-like), drug mast cells, dermis 6 bowel disease 164
reactions 177 Mectizan(ivermectin) 40- 1 muscles, dermatomyositis 15 1-2
lichen planus 1 18,134,134-5 medicamentdermatitis 62 muscle weakness, dermatomyositis
oral lesions 135 melanin 2,5, 109 15 1
variants 135 production, response t o UV mycetoma (Madura foot) 33
lichen planus-like(lichenoid) drug radiation l l 0 mycobacterial infections 19-22
reactions 177 melanocytes 2,4 atypical mycobacteria 20
lichen sclerosus et atrophicus(white hair pigment from 4 cutaneoustuberculosis 19
spot disease) 1 1 1, 135-6, 136 naevi formed from see melanocytic leprosy (Hansen's disease) see
lichen simplex chronicus naevi leprosy (Hansen's disease)
(neurodermatitis) 157, 157-8 tumours involving 78 lupus vulgaris 20,20
light-induced drug reactions 1 3 9 4 0 melanocytic naevi 96-7 scrofuloderma 19
light-induced skin disease 138, acquired 96-7,97,98 tuberculides 20
138-40 congenital 95,96 warty tuberculosis 20
disorders exacerbated by light 140, phases 96-7,97 Mycobaaerium leprae 2 1
l40 melanocytic tumours Mycobacteriummarinum 20
limb oedema 169-70 benign 8 1 mycosis fungoides (T-cell lymphoma)
lipoatrophy 16 1 dysplasticlmalignant 89-9 1 93,93
lipodermatosclerosis 14 1, 142 see also melanoma, malignant myxoedema 1 13
lipoma 83 (MM) pretibial 163, 163
lips melanoma, malignant (MM) 89-9 1 myxoid cyst 120, 12 1
bullous erythema multiforme 133 acral 90,91
in inflammatory bowel disease 164 amelanotic 90 naevi (naevus) 94- 1 0 1
squamous cell carcinoma 87,88 checklist 92 angiomatous 99, 10 1
liquid nitrogen cryotherapy 22 clinical features 90 Becker's 94
Lisch nodules l04 differentialdiagnosis 79,90 cavernous (angiomatous) 99, 10 1
livedo reticularis 146 early presentation 9 1 classification 95
liver disease 1 13 nodular 90,9 1 compound (dermal) 96
pruritus 159 patterns 90 deep capillary (port wine stains)
skin and nail changes 166, 166 prevention 9 1-2 98-9,100
local anaesthetic, skin biopsy 1 3, 14 risk factors 89-90 definition 79,94
lotions 18 1 superficial spreading 90,90 dysplastic 98
love bugs see crab lice (Pthirus pubis) verrucous 90 epidermallorganoid 94
lufenuron (Program) 46 melanosome complexes 2 hypochromic 1 10
lupus erythematosus 148-50 melanosomes 2,109 intradermal 96
antiphospholipid syndrome 149 melasma (chloasma) I 1 2, 113 junctional 96
discoid 118, 149, 149-50 metastases, cutaneous 166-8, 167, mast cell 101
drug-induced 149,178 168 melanocytic see melanocytic naevi
subacute cutaneous 150 methoprene 46 sebaceous 94
systemic see systemic lupus methotrexate, psoriasis 74 Spitz 98
erythematosus microabscesses strawberry 99, 10 1
lupus erythematosus-like syndrome dermatitis herpetiformis 127 superficial capillary 97-8, 100
1 78 polymorph 68 Suttonk'halo' 98,98, 1 1 1
lupus pernio, in sarcoidosis 166 microscopically controlled surgery, vascular 97-9, 10 1
lupus vulgaris 20,20, 1 l 8 basal cell carcinoma 85 see also individualnaevi
Lyme disease, vectors 47 Microsporumcanis 3 1 nail(s) 5,s-6
lymphadenopathy 134 milia 81,81 brittleness 1 19
lymphomas 78,92-3,167 miliaria(prickly heat) 137 in chronic liver disease 166, 166
lymphoreticularmalignancy, pruritus moccasin tinea pedis 28-9 clippings 13
159 moles see naevi (naevus) clubbing 119
molluscum contagiosum 24,24 crusted scabies 40
maceration 7 1 molluscum sebaceum dermatomyositis 1 50, 15 1
macrophages, dermis 6 (keratoacanthoma) 80,80 dermatophyteinfections 30,3 1,
macules, hypopigmented 104, 1 10 Mongolian blue spot 96,96 33
Madurafoot (mycetoma) 33 moniliasissee Candida infection discoloration 1 19, 166
malar flush 162 (candidiasis) growth 6
Malassezia 35,36 morphoea 152,152-3 ingrowingnails 12 1
skin scrapings 12 circumscribed 152 in lichen planus 134
malathion 39 frontoparietal 153 loss 1 19
male-patternbalding 3, 11 5, 1 16 linear 152-3 pits 69,70, 1 19
Index 191

roughness 1 19 psoriatic 70 oral lesions 123

spoon-shaped 1 19 ophthalmic zoster 27,2 7 pemphiguserythematosus 124-5
Terry's 166 oral hairy leukoplakia 168 pemphigusfoliaceus 123, 124
transverse grooves 6 orf 24 pemphigusvegetans 124
washboard 1 19 Osler-Weber-Rendu disease pemphigusvulgaris 123, 123, 124
nail abnormalities 1 18, 1 19, 12 1 (hereditary haemorrhagic perchlorethylene 155
acquired 1 19 telangiectasia) 105 perianal lesions, in inflammatory bowel
congenital 1 19 overprescribing 18 1-2 disease 164
disorders associated 1 19 perianalwarts,AIDS 168
ingrowingnails 12 1 Paget's disease 87,87 periarteritis nodosa (polyarteritis
paronychium disorders 12 1 palmo-plantar keratoderma(tylosis) nodosa) 146-7
nail fold, bolstering, in paronychia l03 perioral dermatitis 57
34 palmo-plantar pustulosis 72, 72 topical steroids 183, 1834
nail plate 5 palms peripheralcollarette 136, 137
nail psoriasis 69,69-70 beta-carotene hyperpigrnentation peripheralvascular disease 143
treatment 75 113 periungualfibromas 104, l05
nalidixic acid 123 endogenous eczema 65-6 periungualwarts 12 1
necrobiosis lipoidicadiabeticorum horny layer thickening 103 perleche (angular cheilitis) 34,34
161,162 palmo-plantar pustulosis (pustular permethrin 46
necrolysis,toxic epidermal 128, psoriasis) 72,75 permethrin cream 39
1 28-9 'tripe' 167 perniciousanaemia 1 13
necrolytic migratory erythema 167 xanthomas 165 perniosis (chilblains) 145-6, l46
necrotizingfasciitis 1 7 panniculitis perspiration 7
neonates equestrian cold 145-6 Peutz-Jeghers syndrome 105, 1 12
angiomatosis 10 1 fat 145 phaeomelanins 5, 109
epidermolysis bullosa 123 papillomas, basal cell (seborrhoeic phenylketonuria l l 0
neoplastic ulcers 143 keratoses; seborrhoeicwarts) photo-ageing 138
nervous system, systemic sclerosis 79,79-80 photodynamic therapy (PDT) 79
154 papular urticaria 4545-7 photosensitive eczema 139
nettle rash 130 animal mites 47 photosensitivity, drug-induced 178,
neuralgia, postherpetic 27 bed bugs (Cimex leaularius) 46-7 178, 178
neurodermatitis (lichen simplex fleas 46 phytophotodermatitis 139, 140
chronicus) 157, 157-8 ticks 47 pigmentary disorders 109-1 3
neurofibromatosissee von papules drug-induced 177, 178
Recklinghausen's acne vulgaris 49,50 hyperpigrnentation I12, l 12-1 3
neurofibromatosis Gottron's 150 hypopigmentation 1 10, 1 10-1 1
neuropathic ulcers 16 1, l62 lichen planus 134, 134 see also hyperpigmentation;
neurotic excoriations 172-3 parakeratosis 68,70 hypopigmentation
nickel dermatitis 60-1,6 1 parasitophobia 159 pigmentary incontinence 1 13
nicotinic acid deficiency 164 parasitosis(delusionsof parasitosis) pigmentation
Nikolsky sign 123-4, 128 171-2 hair 114
nipple, Paget's disease 87 paronychia 12 1 normal colourslskin types 109
nits 4 1-3 acute bacterial 34 normal mechanisms 109- 10
'nitwit' 42-3 chronic 34-5 pilar (trichilemmal)cysts 8 1
nodular prurigo 157, 157 paronychiurn,disorders 12 1 pilosebaceous unit 3
nodular vasculitis l46 pastes 181 pimozide 172
nodules patch tests 14-15, /5,63 pityriasis alba I l l, ll 2
acne vulgaris 50 pathomimicry, dermatological 17 1 pityriasis amiantacea 69
apple jelly, lupus vulgaris 20 patient compliance 18 1 pityriasis lichenoides 1 37
Lisch 104 patient preconceptions 10- I I pityriasis rosea 136, 136-7, 137
polyarteritis nodosa 146-7 pediculicides 43 atypical 137
rheumatoid 164 pediculosis 4 1-5 differentialdiagnosis 70
umbilical (Sister joseph's nodule) clothing lice (Pediculushumanus) 43, pityriasis rubra pilaris 137
166 44 pityriasis versicolor 35-6,36, 1 I l
Norwegian (crusted) scabies see crab lice see crab lice (Pthirus pubis) Pityrasporumfolliculitis 56
crusted (Norwegian) scabies head lice see head lice (Pediculus plane warts 23,23
nuclear dust 144 capitis) plantar warts 23,23
Pediculuscapitis see head lice (Pediculus plants
obsessive-compulsive habits 172-3 capitis) dermatitis due to 62
occupational contact dermatitis 62 Pediculushumanus(clothing lice) 43, phytophotodermatitis 139
occupational irritant dermatitis 60, 44 plasters,dermatitis due to 6 1
60 pellagra 139, 164 poison ivy 62
Odland bodies 3 pemphigoid polyarteritis nodosa (periarteritis
oil-inducedacne 55 bullous see bullous pemphigoid nodosa) 146-7
oily creams 180 cicatricialsee cicatricial pemphigoid polycystic ovary syndrome 54
ointments 18 1 pemphigoidgestationis 137 polymorphic eruption of pregnancy
onychogryphosis 1 19, I20 pemphigus 123-5 137
onycholysis 70, 119, 120 investigations 1 24, 125 polymorphic light eruption 139
192 Index

polyserositis 148 arthropathic 75-6,76 renal failure

polyvinyl chloride (PVC) 154-5 brittle 71,71,75 hyperpigmentation 1 13
pompholyx 32,65,66 cardinal features 67 pruritus 159
porphyria 139 causeltriggers 67 renal features, systemic sclerosis
congenital erythropoietic clinical pattern 67-72,68 1534
(Giinther's disease) 139 erythrodermic see erythrodermic repigmentation I I I
exacerbation by drugs 179 psoriasis rete ridges 6
variegate 139 exacerbation by drugs 179 13-cis retinoic acid (isotretinoin) 53,
porphyria cutaneatarda 128 exacerbation in AIDS 168 54
port wine stainslmarks (deep capillary flexural 71, 71.75 retinoids (vitamin A derivatives) 53
naevus) 98-9.100 guttate 70,70,75 psoriasis 74
postherpetic neuralgia 27 nail 69,69-70 rheumatic diseases 1 6 3 4
postinflammatory hyperpigmentation onycholysis 70,70 rheumatic fever 164
Il2-13,176 pathology 67 rheumatoid arthritis 164, 164
postinflammatory hypopigmentation plaque 68,69,74-5 vasculitic ulcers 143
Ill pustular see pustular psoriasis rheumatoid nodules 164
poxvirus infections 24 scalp 68-9,69,75 rhinophyma 56,56
prednisolone, pemphigus 125 treatment 7 2 4 , 7 3 ringworm 28
pregnancy specific clinical patterns 74-5 hand 29,30
conditions associated 137 psychological disorders and skin scalp 30-1
polymorphic eruption 137 conditions 169-73 see also entries beginningtinea
pruritus 137 delusions of parasitosis (parasitosis) rodent ulcer see basal cell carcinoma
rashes 137, 137,138 171-2 rosacea 56,56-7
scabies treatment 40 dermatitis artefacta 169-7 1, 170 steroid 184
prepemphigoid 125 dermatological non-disease rubber dermatitis 6 1
prescription quantities 18 1-2 (dysmorphophobia) 171
pressure urticaria 13 1 dermatological pathomimicry sacral zoster 27
pretibial myxoedema 163, 163 171 salicylic acid
prickle cell layer 2 obsessive-compulsivehabits psoriasis 73
prickly heat (miliaria) 137 172-3 warts 22
protoporphyria, erythropoietic 1 39 psychologicalfactors salmon patches 98
prurigo pruritus associated 159 sarcoidosis 165-6, 166
actinic 139 skin disorders associated 169 Sarcoptes scabiei 37
nodular 1 57, 157 'pterygium' 1 19 sarcoptic mange 47
pruritus 156-60, 159 pubic lice see crab lice (Pthirus pubis) scabicides 3 9 4 0
anogenital 158,158 pulmonary features, systemic sclerosis scabies 37-40,39
causes 156-60 153 aetiology 37
chronic 156 punch biopsy 14, 15 burrows 37,38
clothing lice 43 purpura 168 clinical features 37-8,38
crab lice 44 anaphylactoid (Henoch-Schonlein crusted (Norwegian) see crusted
definition 156 purpura) 144-5 (Norwegian) scabies
generalized 158-60, 167 pustular psoriasis diagnosis 38-9
haematological disorders 1 59 acute, of von Zumbusch 72,72,75 institutional outbreaks 4 1
head lice 42 chronic, palms and soles (palmo- treatment 39-40
localized 157-68 plantar pustulosis) 72, 72,75 infants 40
mechanisms 156 topical steroids causing 184 pregnancy 40
pregnancy 137 pustules scalded skin syndrome, staphylococcal
psoriasis 68 acne vulgaris 49,50 19
scabies 37 'satellite,' in intertrigo 35 scalp
senile 157, 160 pyoderma gangrenosum 165,165, discoid lupus erythematosus 149
in skin malignancies 167 l67 hair loss see hair loss
stimuli/causes 156 inflammatory bowel disease 164 psoriasis 68-9,69,75
systemic disorders associated 159, pyogenicgranuloma 82,82-3 ringworm 30- 1,3 1,32
159 scalpel, banana 38
pruritus ani 158 quality of life 10 scaly disorders 102
pruritus vulvae 158, 158 skin lesions affecting 169 scars
pseudofolliculitis barbae (shaving rash) acne vulgaris 50,52
55-6 radiotherapy 79 hypertrophic 83
pseudopelade 1 18 rashes scleroderma 152, 152-5
pseudoscleroderma 155 butterfly 148 chemically-induced 154-5
pseudo-tumours 78,83 nettle 130 morphoea 152,152-3
chondrodermatitis nodularis helicis pityriasis rosea 136 pseudoscleroderma 155
83,83 pregnancy 137, 137, 138 systemic sclerosis see systemic
hypertrophic scars and keloids 83 scabies 37-8,38 sclerosis
pseudoxanthoma elasticum 107, 107 shaving (pseudofolliculitis barbae) scrapings/clippings 12, 13
psoralen and ultravioletA (PUVA) 74 55-6 pityriasis versicolor 36
psoriasis 67-76 recurrent herpes simplex 25 screening, pruritus 159, 160
acute pustular 72,72,75 Reiter's syndrome 76, 164 scrofuloderma 19
Index 193

scrumpox 25 squamous cell carcinoma (SCC) drug-induced 149

scurvy 164 differentialdiagnosis 80, 143 exacerbationby drugs 179
sebaceous glands 5 invasive 87,88 immunofluorescence 148-9
sebaceous naevus 94 Marjolin's ulcer 142 vasculitic ulcers 143
sebo-psoriasis 7 1 polypoid 88 systemic sclerosis 152, 153-4, 154,
seborrhoeic dermatitis 64-5,65 in situ (Bowen's disease) 85-6,86 I55
AlDS 168 staphylococcalinfection 17-1 9 calcinosis 155
seborrhoeicwarts (seborrhoeic AlDS 168 clinical features 153, 153-4, 154
keratoses; basal cell carbuncle 18
papillomas) 79,7940 folliculitis 17-1 8 tacalcitol, in psoriasis 74
sebum 5 furunculosis (boils) 18 tar, psoriasis 73
Secretan's syndrome 169-70 impetigo 18, 19 tar gels 75
secretory coil, eccrine sweat gland 3 scalded skin syndrome 19 'target' lesion 1 32, 132
selenium sulfide shampoo 36 staphylococcalscalded skin syndrome tar shampoos 75
self-inducedskin lesions 169, 170 19 T-cell lymphoma (mycosis fungoides)
Selsun shampoo 36 Stophylococcusoureus 17, 18 93,93
senile lentigo, differentialdiagnosis steroid rosacea 184 telangiectasia
79-80 steroids basal cell carcinoma 84,84
senile pruritus 157, 160 acne vulgaris 53,54 hereditary haemorrhagic
sensory loss, leprosy 2 1 adverse effects 182,183-4 (Osler-Weber-Rendu disease)
sensory receptor 8 dermatomyositis 152 1 05
shaving rash (pseudofolliculitis barbae) potency 182 telangiectasis, spider 94
55-6 SLE 149 telangiectatic naevi 97-9
shingles see herpeszoster systemic, psoriasis 74 telogen 4,5, 1 15
Sister Joseph's nodule (umbilical topical see topical steroids telogen effluvium 1 15- 16
nodule) 166 Stevens-Johnson syndrome (bullous temperature regulation 7
skin erythema multiforme) 129, temporal arteritis (giant cell arteritis)
atrophy 183 133,133 147
atrophy, topical steroids 183 Still's disease 13 1, 164 tendinous xanthomas 165
biopsy see biopsy stork marks 98 terbinafine 33,36
colour, factors affecting 109 stratum corneum 1,3, 180 terminal differentiation I
functions 6-8,7 functions 3,7 terminal hair 3
reactions to light 138, 13840 hydration 180 terrninologyin dermatology 10, 1 I
structure 1-6,4 'strawberriesand cream' appearance tetracyclines, acne vulgaris 53
systemic disease manifestations l62 thinning of skin, steroid side-effect
1 6 1-8 strawberry naevus 99, 10 1 l83
thinning, steroid side-effect 183 streptococcalinfection,cellulitis 17, thrombophlebitis migrans 167
types 109,138 18 thrush see Condido infection
skin cancers see skin tumours Streptococcus pyogenes 17 (candidiasis)
skin capillaries 14 1 striae, steroid-associated 1 83, 183 thyroid disease 162-3
skin tags (acrochordons) 80 striate palmar xanthomas 165 pruritus 159
skin tumours 77-93 Sturge-Weber syndrome 99 ticks 47
in albinism 1 10 subacute cutaneous LE 150 tinea capitis 30- 1.32
benign see benign tumours sulfamethoxypyridazine 128 tinea corporis 29,30
classification 77,78 sulfapyridine 127, 128 tinea cruris 29,29
dark-skinned people 2 sulfonamide, sensitivity 178 tinea incognito 3 1-2
extensionfrom deeper tissues 93 sunburn 138 topical steroid side-effects 184
genetic diseases associated 84 melanoma risk factor 89-90,9 1 tinea manuum 29-30,30
in immunosuppressedpatients 84 sunlight see ultraviolet radiation tinea pedis (athlete's foot) 28-9,29
malignanddysplastic see Sun Protection Factor (SPF) 138 tinea unguium 30,3 1
dysplasticlmalignanttumours sun screens 92, l l l, 138 toenails
metastatic 93,166-8,168 sun tanning l l 0 fungal dystrophy 30,3 1
treatment principles 77-9 surgery see also nail(s)
types 78 acne vulgaris 53,54 topical antifungalagents 32-3,36
UVB treatment and 74 varicose veins 142 topical antipruritic agents 39
solar (actinic) keratoses 85,86 Sutton's'halo' naevus 98,98, l l l topical antiseptics 53
soles sweat glands 3 topical medicamentsdermatitis 62
beta-carotene hyperpigmentation sweating, absence 106 topical steroids 182-4
113 swimming pool granuloma 20 adverse effects 183-4
endogenous eczema 65-6 symptoms, of skin disease 10 atopic eczema 64
horny layer thickening 103 systemic disorders 16 1-8 choice of preparation 182, 182-3
juvenile plantar dermatosis 66,66 malignancy, skin features 166-8 exogenous eczema 63
palmo-plantar pustulosis (pustular pruritus in 159, 159 potency 182
psoriasis) 72, 72,75 skin manifestations 1 6 1-8 psoriasis 73
spider naevus 94 urticaria in 13 1 topical treatments 180
spider telangiectasis 94 systemic lupus erythematosus (SLE) toxic epidermal necrolysis 128, 1 28-9
Spitz naevus 98 l48,148-9,149 trachyonychia 1 19
sporotrichoid spread 20 clinical features 148, 148, 149 traction alopecia 1 17, 1 17
194 Index

treatment of skin disease 180-4 harmful effects l l 0 herpes simplex 24-6

bases 180-1 psoriasis 74 herpes zoster (shingles) 26-7
communication 18 1 skin reactions 138, 13840 warts due t o 22,22-4
compliancewith 18 1 skin response 1 10 see also individualinfections
quantities prescribed 18 1-2 umbilical nodule (Sister Joseph's vitaminA analogues, psoriasis 74
topical steroids see topical steroids nodule) 166 vitaminA derivatives (retinoids) 53
trichilemmal (pilar) cysts 8 1 underprescribing 18 1-2 vitamin C deficiency 164
trichlorethylene 155 Unguentum Cocois Co. 75 vitamin D (cholecalciferol) 7
Trichophyton tonsurans 30 urticaria 1 30-2, 13 1 vitamin D analogues, psoriasis 74
Trichophyton violoceum 30 cholinergic 13 1 vitamins, deficiency 164
trichotillomania I 17, 172, 172 clinical forms 130-1 vitiligo 1 10-1 1, l ll
trigeminal nerve, vascular naevi drug-induced 176 Volkmann spoon 77
complications 99 papular see papular urticqria von Recklinghausen's
trigeminaltrophic syndrome 1 18 physical 13 1 neurofibromatosis 83, 104-5,
trigeminalzoster 27 in systemic disease 13 1 l06
tripe palms 167 treatment 132, 132 hyperpigmentation 1 12, 113
triradius 6 urticaria pigmentosa 10 1, 1 12, 1 3 1 von Zumbusch, acute pustular
tuberculides 20 psoriasis 72, 72,75
tuberculoid leprosy 2 1-2,22 varicose eczema 65, 14 1, 142 vulvovaginitis,Candida infection 35,
hypopigmentation I I I varicose veins 14 1, 142 16 1
tuberculosis variegate porphyria 139
cutaneous 19 vascular blemishes 97 wart paints 22
warty 20 vascular disorders 14 1-7 warts 22,224
tuberous sclerosis 104, 105 leg ulcers see leg ulcers common 22,22-3
hypopigmentation l l 0 vasculitis 143-7 genital (condylomata acuminata)
tuberous xanthomas 165, 166 see also individualdisorders 23-4
tumours vascular naevi 95,97-9, 10 I 'mosaic' 23,23
in dermatomyositis 150, 15 1 angiomatous 99, 10 1 periungual 12 1
metastases, skin 93, 166-8, 168 complications 99 plane 23,23
primary, skin see skin tumours rare angiomatous 10 1 plantar 23.23
pruritus 159 telangiectatic 97-9 seborrhoeic 79,79-80
systemic, skin manifestations vasculitic ulcers 143 warty tuberculosis 20
166-8 vasculitis 143-7, 144 weals 130
Turner's syndrome 108 classification 144 Wegener's granulomatosis 146
tylosis (palmo-plantar keratoderma) clinical presentations 144-7 wet-wrap technique 64
l03 in dermatomyositis 150 whiteheads (closed comedones) 50
tyrosinase 1 10 digital, in rheumatoid arthritis 164 white spot disease (lichen sclerosus et -
Tzanck preparation 1 3 drug-induced 176 atrophicus) I l l, 135-6, 136
large vessel 144, 146-7 whitlow, herpetic 25
ulcerativecolitis 164, 164 leucocytoclastic (allergic) 144, Wickham's striae 134
ulcers 1 44-5 'winkle-picker' technique 39
ischaemic, foot 143 necrotizinggranulomatous 146 Wood's light 12, 13
leg see leg ulcers nodular 146
Marjolin's 142 small vessel 144, 144-6 xanthelasma 165
neuropathic 16 1, l62 triggers 143 xanthomas 16 1, 165, 165
rodent see basal cell carcinoma vasoconstriction 7 striate palmar 165
(rodent ulcer) veins, varicose 14 1 , 142 tendinous 165
vasculitic 143 vellus hair 3, 168 tuberous 165
ultravioletA light (UVA) 74, 138 venous hypertension xeroderma pigmentosum 107,139
ultraviolet B light (UVB) 74 clinical features 14 1 xerosis 160
ultraviolet light treatment, atopic reduction 142 XYY syndrome 108
eczema 64 venous leg ulcers 14 1, 14 1-3,143
ultraviolet radiation verrucae 22 yellow nail syndrome 119
damage, repair defects 107 see also warts
discoid lupus erythematosus vinyl chloride disease 155 zinc malabsorption 107
149 viral infections 22-7 zoster see herpes zoster (shingles)