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Bhutani's Color Atlas of Dermatology
Bhutani's Color Atlas of Dermatology
Color Atlas of
DERMATOLOGY
Bhutani’s
Color Atlas of
DERMATOLOGY
Sixth Edition
Neena Khanna MD
Professor
Department of Dermatology and Venereology
All India Institute of Medical Sciences
New Delhi, India
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It gives me great pleasure to contribute a foreword for this book, which has been written by an eminent
teacher and research worker. Professor Lalit K Bhutani, who is the Head of the Department of Dermatology
and Venereology at the All India Institute of Medical Sciences, New Delhi, is a renowned dermatologist,
a teacher of repute and an internationally recognized research worker in the field of dermatology. He has
been guest lecturer at many national and international universities and holds memberships of several
international dermatological societies. His research interests have centered around such subjects as
leprosy, pigmentary problems, mycological diseases and sexually transmitted diseases. He has made
special contributions in the field of immunology of leprosy.
This book is the result of much painstaking work and reflects the vast experience of Professor Bhutani.
Through the excellent reproduction of colored pictures of dermatological lesions, the author has provided
a clear understanding of various dermatological disorders. His stress on dermatological problems seen
in the tropics is praiseworthy. The material has been well planned and presented in a very lucid manner.
The book will be useful to clinicians in general as well as specialists in this field. I congratulate Professor
Bhutani for his praiseworthy attempt and wish this work all success.
Dr Bhutani, an eminent physician, and a dermatologist and venereologist of international repute, contributed
immensely to the development of the discipline in India and placed it on the global dermatology map. He
was deeply involved in the management of sexually transmitted diseases including HIV and AIDS besides
development and progression of modern management of leprosy in India and beyond. His other fields of
interest were dermatopathology and photobiology. He was the first to describe the condition lichen planus
pigmentosus.
A graduate of University of Punjab (1958), he moved to join All India Institute of Medical Sciences (AIIMS),
New Delhi. An almost reluctant entrant into the field of dermatology and venereology, he became one of
its most brilliant teachers and took over the reins of the department of AIIMS in 1979. He eventually rose
to become the Dean and Director of this prestigious institute from which he retired in 1996. He continued
to provide invaluable service as an honorary consultant dermatologist at Sitaram Bhartiya Institute of
Science and Research, New Delhi, until his death. He was appointed Honorary Physician to the President
of India in 1983.
In recognition of his brilliant academic achievements, he was awarded Honorary Fellowship of the Royal
College of Physicians (Edinburgh) in 1987 and received the prestigious BC Roy Award for excellence in
teaching. His commitment to teaching was reflected in his authorship of two immensely popular (almost
Biblical) atlases “Colour Atlas of Dermatology” and “Atlas of Sexually Transmitted Diseases”. A keen
researcher, he had to his credit nearly 150 publications in national and international journals of repute
and contributed chapters to several books of Medicine and Dermatology.
He held several prestigious positions in his specialty and was elected President of Indian Association
of Dermatology and Venereology (1979–80) and its General Secretary (1973–74). That he successfully
hosted the VII International Congress of Dermatology and Venereology in New Delhi in 1994, (first ever
for an Asian country) speaks volumes of his organizational skills.
He was invited to participate in and chair a number of national and international conferences and
seminars. He was honored with a number of visiting professorships at various institutes internationally, such
as Mayo Clinic, Rochester, USA, and King Faisal University, Dammam, Saudi Arabia, where he was the
architect of its postgraduate residency program which is still being followed. He was associated in various
capacities with a number of international and national professional bodies such as American Academy of
Dermatology, British Association of Dermatology, American Society of Photobiology, International Society
x Bhutani’s Color Atlas of Dermatology
A classic is often known by the name of its creator rather than by the title given to it. This is so true for the
Color Atlas of Dermatology. It is frequently referred to as Dr Bhutani’s atlas or simply as LKB’s atlas. One
of the most popular books in dermatology, several batches of undergraduate and postgraduate students
swear by it even today. It is also a ‘must have’ for general practitioners (GPs) and practicing dermatologists.
This atlas, like the previous editions, is intended for senior medical students, general practitioners and
also entrants into the field of dermatology. The book is divided into 25 chapters: some diseases have
been classified etiologically while others have been grouped conventionally; however, some groupings
have been made morphologically to emphasize how dermatoses, with different etiogenesis, may simulate
each other. Newer therapies have been incorporated (albeit briefly) to make the book comprehensive.
Throughout the book, Professor Bhutani’s inimitable and crisp style, which became very popular and
identified with the atlas, has been retained.
Neena Khanna
Saurabh Singh
Preface to the First Edition
This atlas is intended primarily for senior medical students, general practitioners and residents in
dermatology and internal medicine. Next to the actual patient, a color photograph is perhaps the most
useful learning material in dermatology. Such atlases in the West have an understandable bias on locally
important conditions. A certain emphasis on tropical dermatoses may be discernable here; other more
cosmopolitan diseases have been given adequate coverage.
On account of the ease with which populations from one part of the world have moved to another,
the physician is constantly faced with the challenge of diagnosing and treating exotic conditions. The
projection in this atlas of skin lesions on a pigmented background will aid the physician in his diagnosis.
A brief description has been given for an easy interpretation; selected photomicrographs are added for
further help. Notes on treatment are brief and meant as a guideline. The book combines visual impression
of morphologic lesions with factual information, and supplements the standard textbooks.
The book is divided into nineteen chapters with various diseases conventionally grouped; some
unconventional grouping has also been made to emphasize close clinical simulation. A chapter on sexually
transmitted diseases has been included since in many countries the specialities of dermatology and
venereology are practised jointly.
All photographs including photomicrographs have been taken by myself. Most are from patients seen in
India; some are from patients seen in Africa or South-East Asia. Acknowledgment is made for permission
to reproduce my two fluorescent photographs on page 98 from Arch. Derm. 110:427, 1974, copyright
1974, American Medical Association.
Three persons played a dominant role in shaping my career in dermatology: Professor KC Kandhari,
my teacher and mentor, initiated me into dermatology; Professor IA Magnus and Professor CD Calnan
encouraged me to continue. This book is in a sense a tribute to them.
My colleague and friend Dr RK Pandhi generously gave his valuable time and suggestions at every
stage; but for his inestimable help, this book would not have been completed. Other friends helped in
different ways: Professor NC Nayak with advice on selection of photomicrographs and histologic description;
Drs Roger Harman, R Bikhchandani, AS Kumar, Jose Thomas, Sharad Goel, Jasleen Kaur, Anuradha
Kathpalia, Messfin Demisse with help in critical evaluation of the manuscript and galley proofs. Dr E Nair
provided specimens of pediculi and mite. Professors RK Panja, BMS Bedi and Dr Dharam Pal gave useful
suggestions. Professors YK Malhotra, R Patnaik and Dr Daljit S Nagreh permitted the use of their patients’
photographs. Mr ML Bhalla and Mr Awasthy helped in the processing of photomicrographs. Mr Krishan
Kumar rendered useful secretarial assistance.
I am most grateful to Mr SN Mehta, the publisher, and Mrs Susan Gale, the editor, for all their help.
They displayed rare patience and understanding in handling the manuscript, the pictures and the entire
work.
xiv Bhutani’s Color Atlas of Dermatology
LK Bhutani
Acknowledgments
The present work would not have been possible without the contributions of several of my colleagues. Drs
Riti, Kamal, Ajay, Gitika, and Tanir who nit-picked the manuscript to its culmination. Ms Tanu and Meenu
who helped in correcting the manuscript in its initial stages.
I am grateful to Dr Somesh Gupta for permitting me to use pictures of his 'operated' patients (2 pictures
on page 470 and 4 pictures on page 472). So also my gratitude to my faculty colleagues (many of whom
are Dr Bhutani's students) for letting us photograph their 'in-patients'. I am also grateful to Dr Harsh Mohan
for permitting me to use picture of histopathology of sarcoidosis (on page 199).
I express my appreciation and gratitude to Shri Jitendar P Vij (Group Chairman) and Mr Ankit Vij (Group
President) of M/s Jaypee Brothers Medical Publishers (P) Ltd., New Delhi, India, which is one of the largest
Medical Publishers in the world, for their assistance and help. Mr Tarun Duneja (Director-Publishing)
has been instrumental in getting the final shape of the book. Also, my acknowledgments to Ms Samina Khan
(Executive Assistant to Director-Publishing), Mr KK Raman (Production Manager), Mr Sunil Kumar Dogra
(Production Executive), Mr Neelambar Pant (Production Coordinator), Mr Manoj Pahuja (Senior Graphic
Designer), Mr Varun Rana (Typesetter), Mr Himanshu Sharma (Proofreader) and other members for their
immense patience to bring out the book.
The task of completing the book would have been impossible, had it not been for the ever-encouraging
presence of Mr Ranbir Singh, whose untiring efforts smoothened all obstacles during the production of
the book. His patience and eye for detail are the reasons for the quality of this book.
I am indeed indebted to Dr (Mrs) Bhutani for again reposing faith in me and believing that I could
carry out the herculean task of bringing out a book which had acquired 'Biblical status'. Her presence and
perseverance were pivotal in helping us meet the targeted dates. She was always there as the guiding
force and her unflinching encouragement brought out the best in the team.
I would be failing in my duty if I did not thank the 'behind the scene' people. Anil and my brats who put
up with sharing my time with the laptop.
Neena Khanna
First of all, I am grateful to Dr Neena Khanna for entrusting me with this herculean task of carrying forward
and being a part of Dr LK Bhutani’s atlas, the work for which I had a desire since my undergraduate
days at AIIMS, New Delhi, India. And I share and echo indebtedness to Dr Mrs Bhutani for giving this
opportunity. The love and support of my parents and sisters have been my guiding force. The ‘quartet’ of
you have always allowed me the liberty to be a ‘parasite’, take the honest utmost difficult path to success
and to bask in the glory thence forth; Papa, Mummy and Didis’, I dedicate this to you! My love to my tiny
little nieces and nephews who had virtually labelled me ‘laptop Mama’, without expecting even an ounce
of attention from my end. I would be failing in my duty if I do not thank Dr Shanta Passi (Dermatology
Specialist, ESIC Hospital, Faridabad) for taking care of OPDs and my patients thus relieving me for book
work whenever needed and allowing photography of few of her patients for the same purpose. I am grateful
to Dr Asim Das, Dean, ESIC College, for allowing me to work on the book and to the office orderly. Mr
xvi Bhutani’s Color Atlas of Dermatology
Shailender for keeping office open, when needed, beyond office hours. I am especially thankful to Drs
Mayank, Piyush and Narendra, for they had been my ‘official chariots’, and all other faculty members
of our college for providing a ‘healthy’ workplace. Finally, a toast to our MBBS teacher Dr Deorari
(Prof. Neonatology, AIIMS, New Delhi) who mentioned once that I must also embark on writing an Atlas
since I had joined a department of Dr Bhutani’s repute!
Saurabh Singh
Contents
1. Pyodermas 1
Impetigo Contagiosa 3
Ecthyma 3
Bullous Impetigo 5
Staphylococcal Scalded Skin Syndrome 5
Cellulitis 7
Folliculitis 7
Boil (Furuncle) 7
Carbuncle 9
Principles of Management of Pyodermas 9
Anthrax 11
Non-Pyococcal Dermatoses Simulating Pyodermas 11
Infections Caused by Resident Flora 11
2. Fungal Infections 15
Pityrosporum Infection 17
Dermatophytoses (Ringworm) 19
Candidiasis (Candidosis) 27
Mycetoma (Madura Foot) 29
Subcutaneous Zygomycosis 33
Sporotrichosis 33
Chromomycosis 35
Lobomycosis (Keloidal Blastomycosis) 37
Cryptococcosis 37
Histoplasmosis 39
3. Viral Infections 41
Warts 43
Molluscum Contagiosum 45
Measles 47
Herpes Simplex 49
Varicella-Zoster Virus Infection 51
4. Leprosy 55
Etiology 57
Epidemiology 57
xviii Bhutani’s Color Atlas of Dermatology
Spectrum 57
Cardinal Features of Leprosy 57
Clinical Manifestations 59
Reactions in Leprosy (Lepra Reactions) 61
Investigations 63
5. Cutaneous Tuberculosis 71
Etiology 73
Epidemiology 73
Clinical Manifestations 73
Investigations 77
Sarcoidosis 197
Skin in Endocrinological Diseases 199
Cutaneous Manifestations of Malnutrition 203
Ecthyma: hemorrhagic crusts which on removal reveal an ulcer. Ecthyma: heals with scarring.
Pyodermas 3
Normal skin not sterile. Inhabited by: an erythematous halo. Heal without sequelae.
Residents: present permanently. Include bacteria Regional lymphadenopathy frequent. Face, limbs
(non-pathogenic staphylococci, micrococci and and less frequently other parts of the body involved.
diphtheroids) and fungi (pityrosporum). Nephritogenic strains of streptococci cause acute
glomerulonephritis, a serious but uncommon
Transients: pathogenic organisms which live
complication.
only transiently on skin.
Skin infections caused by pyococcal (pus producing)
organisms are called pyodermas. Organisms
Investigations
commonly incriminated include Staphylococcus Direct Microscopy
aureus and Streptococcus pyogenes.
Gram stain shows either Gram positive
Pyodermas classsified as: cocci in chains (streptococci) or in clusters
Nonfollicular: when infection is not particularly (staphylococcus). Helps in immediate institution of
folliculocentric. Nonfollicular pyodermas include appropriate empirical therapy.
localized infections which may be caused by
staphylococci (impetigo contagiosa/bullous Culture
impetigo/ecthyma), or by streptococci (impetigo Culture and antibiotic sensitivity done in appropriate
contagiosa/ecthyma). Or spreading infection situations.
which are usually caused by streptococci
(erysipelas/cellulitis). Histopathology
Follicular: folliculocentric pyodermas are invariably Histology reveals a subcorneal split containing
caused by staphylococci and include superficial polymorphs.
and deep folliculitis, furuncles and carbuncles.
Pyodermas also classsified as:
Primary pyodermas: arise in normal (not
diseased) skin; have a defined morphology.
Secondary pyodermas: arise in diseased skin
(scabies, pediculosis, insect bite hypersensitivity
dermatophytic infection, atopic dermatitis,
miliaria); have variable morphology.
IMPETIGO CONTAGIOSA
Etiology
Frequently caused by Strep pyogenes. Less often
Staph aureus (phage group II). Often mixed. Poor
personal hygiene, insect bites, trauma, infestations Histopathology of impetigo contagiosa: subcorneal split con-
(scabies, pediculosis) predispose. taining polymorphonuclear leukocytes.
Epidemiology
Common; spreads amongst contacts. Auto- ECTHYMA
inoculable, infants and children prone.
Etiology
Clinical Manifestations Strep pyogenes or Staph aureus. Or mixed etiology.
Clinically, transient vesicules/pustules evolve into Children with poor personal hygiene, malnutrition,
lesions with dirty yellow ‘stuck on’ crusts with skin infestations affected.
Bullous impetigo: large vesiculo-pustular lesions. Heal in center Bullous impetigo: central healing with peripheral extension
and spread centripetally. resulting in circinate lesions.
Staphylococcal scalded skin syndrome: sheets of ‘scalded’ skin Staphylococcal scalded skin syndrome: sheets of peeling skin.
peeling off. Note denudation is superficial.
Pyodermas 5
Histopathology of Gram stain from lesion of ecthyma: shows Histopathology of Gram stain of exudate from bullous impe-
Gram positive cocci in chains, suggestive of streptococcal infec- tigo: shows Gram positive cocci in clusters suggestive of staphy-
tion. May also show Gram positive cocci in clusters. lococcal infection.
Culture Culture
Culture and antibiotic sensitivity done in appropriate Culture (if done) grows Staph aureus (phage group
situations (non-responsive disease). And recurrent II).
infection.
STAPHYLOCOCCAL SCALDED SKIN
BULLOUS IMPETIGO SYNDROME (SSSS)
Etiology Etiology
Staph aureus (phage group II, type 71). Bullae Serious toxin-mediated (epidermolysin)
caused by staphylococcal epidermolysin. manifestation of staphylococcal infection.
Cellulitis: ill-defined, edematous lesion with vesiculation. Cellulitis: acutely inflammed, erythematous, tender plaque with
ill-defined edge.
Superficial folliculitis: follicular pustules on the face. Deep Sycosis barbae: scaly and crusted plaque surmounted by
folliculitis. pustules.
Pyodermas 7
Furuncle: two adjoining follicles involved. Firm, erythematous Carbuncle: painful, erythematous, dome shaped indurated swell-
nodule, surmounted with pustule. Very painful. ing that pours out pus and necrotic material from several open-
ing. Seen on back, buttocks and neck.
Pyodermas 9
Pseudofolliculitis: multiple follicular and perifollicular inflamma- Post waxing folliculitis: follicular erythematous papules and
tory papules; needle held by the loop formed by ingrown hair. pustules. Occur 2–3 weeks after waxing. A pseudofolliculitis.
Pyodermas 11
Streptococcal infections: in general, treated end of hair released (out of the papule) on gently
with penicillins (benzyl penicillin/amoxicillin) lifting hair shaft. Chronic and recurrent. Pustules or
cephalosporins and macrolides. abscesses may complicate. Beard/pubic area. And
areas waxed.
Staphylococcal infections: treated with
penicillinase resistant penicillins (cloxacillin,
Treatment
methicillin). Or a combination of penicillin with
-lactamase inhibitors (clavulanic acid). Or Preventive measures include hydration of hair
macrolides. Or cephalosporins. before shaving. Using single-edged, foil guarded
safety razors. Or using chemical depilation. In
ANTHRAX active lesions, avoid shaving for 3–4 weeks.
Topical retinoids (remove thin epidermis
Etiology covering of regrowing follicle) and mild topical
corticosteroids (reduce inflammation). Severe
Bacillus anthracis, a Gram-positive rod. Acquired
cases (pustules/abscesses) need topical and oral
from animals. Threatened use, as an agent of
antibiotics (erythromycin or clindamycin).
biological warfare.
Pitted keratolysis: brownish superficial erosions-discrete and Pitted keratolysis: close-up of crateriform pits. Note interdigital
confluent. intertrigo.
Pyodermas 13
Pitted Keratolysis
Etiology
Micrococcus sedentarius. And also
Corynebacterium spp. Plantar hyperhidrosis and
wearing of occlusive footwear predispose.
Epidemiology
Uncommon.
Clinical Manifestations
Young adult males more susceptible.
Wood’s lamp examination of erythrasma: shows coral-red
Asymptomatic, well-defined, circular or irregular,
fluorescence. discrete and confluent superficially pitted lesions
(crateriform pits) on the soles; may be associated
with interdigital intertrigo. Bilaterally symmetrical.
Treatment Treatment
Keep area dry. Apply topical 2–5% erythromycin Preventable condition by keeping area dry. Avoid
lotion/gel. Or 1% imidazole lotion/powder. occlusive footwear. Treat hyperhidrosis (with
Systemic erythromycin therapy perhaps not 20% aluminium chloride hexahydrate). Specific
necessary. treatment includes benzoyl peroxide 5% gel. Or
an imidazole powder/cream.
2 Fungal
Infections
Pityriasis versicolor: hypopigmented, discrete and confluent Pityriasis versicolor: hyperpigmented, scaly macules—discrete
macules with branny scales. and confluent. Note many lesions perifollicular.
Pityriasis versicolor: hyperpigmented macules with scaling Pityrosporum folliculitis: follicular papules and pustules
accentuated by scratching. With permission of Elsevier, Illustrated on trunk. With permission of Elsevier, Illustrated Synopsis of
Synopsis of Dermatology and Sexually Transmitted Infections. Dermatology and Sexually Transmitted Infections.
Fungal Infections 17
PITYROSPORUM INFECTION
Etiology
Malassezia furfur, a saprophytic dimorphic, Potassium hydroxide mount of scale of pityriasis versicolor:
resident fungus. shows typical spaghetti and meat ball appearance. (short septate
hyphae and thick walled spores).
Epidemiology
Very common in warm humid climates. Usually Culture
affects adolescents and adults.
Usually not required.
Clinical Manifestations Treatment
Pityriasis Versicolor
General Measures
Cosmetically objectionable asymptomatic,
hypopigmented (on dark skin) and/or Keep skin dry. Avoid use of synthetic occlusive
hyperpigmented (on white skin), well-defined, garments that prevent evaporation of sweat.
perifollicular, small macules with fine branny scales Specific Therapy
(accentuated by scratching). Confluence may
produce large lesions. On the front and back of the Topical therapy
chest, neck, upper arms. Recurrences frequent. Indicated for limited disease. Several choices
available—ketoconazole, 2% shampoo, used
Pityrosporum (Malassezia) Folliculitis twice a week for 4–6 weeks. Lathered on affected
Itchy, erythematous, small follicular papules/ skin and left for 5–10 minutes for 3 consecutive
pustules. Back, chest, proximal extremities. days. Alternatively, 2.5% selenium sulphide
Tinea corporis: well-defined, annular lesion with erythematous Tinea corporis: circinate lesions; note ‘active’ erythematous ele-
scaly border with papulovesicles and relatively, clear center. vated border.
Tinea imbricata: concentric lesions with brown scales and almost Tinea faciei: circinate lesions. This one has incomplete central
clear intervening skin. clearing.
Fungal Infections 19
Variants
Tinea imbricata
Uncommon variant of tinea corporis confined
to certain geographical areas. Caused by
Trichophyton concentricum. Extremely itchy
concentric rings of brownish scales attached at
one edge. And clear intervening skin.
Tinea faciei
Tinea of face.
Tinea incognito
Tinea infection modified by steroids. Manifests as
ill-defined scaly lesions with minimal inflammation.
Diagnosis often based on a positive KOH mount.
Tinea Cruris
Common. Adults affected more frequently. And
males due to regional anatomical differences
and type of apparrel. Itchy, often bilaterally
symmetrical, large half-moon shaped or multiple
small, well-defined, oval or circinate scaly plaques
with papulovesicles at the active border. Central
clearance often incomplete. Groins, thighs,
Sites of predilection: Tinea. buttocks and scrotum involved.
Tinea incognito: ill-defined scaly lesions with minimal scaling. Patient Tinea cruris: bilateral well-defined half moon-shaped lesions
was using topical steroids. With permission of Elsevier, Illustrated with 'active' borders.
Synopsis of Dermatology and Sexually Transmitted Infections.
Tinea manuum: well-defined scaly lesions on the palm; unilat- Tinea pedis, intertriginous variant: scaly lesions in between the
erality conspicuous. lateral toe spaces.
Fungal Infections 21
Tinea Barbae
Adult males affected. Inflammatory swellings with
follicular pustules—similar to kerion. Occasionally
well-defined alopecic patches with scaling and dull
broken-off hair. Affected hair either break or can be
pulled out painlessly with relative ease.
Tinea Unguium
Caused by dermatophytes c.f., onychomycosis which
is caused by any fungus. Adults affected. Cosmetic Wood’s lamp of tinea capitis: green fluorescence. Useful for
problem and a reservoir of infection (so association screening epidemics, though not always positive.
Tinea pedis, hyperkeratotic variant: diffuse scaling of the Tinea capitis, non-inflammatory variant: alopecic areas with
soles of the feet. black dots. With permission Elsevier.
Tinea capitis, kerion: solitary boggy plaque, studded with Tinea capitis, kerion: alopecic areas with multiple inflammatory
follicular pustules, overlying hair sparsening. swellings.
Fungal Infections 23
Direct Microscopy
Samples include skin scrapings, nail clippings or
plucked hair dissolved in KOH. Area cleaned and
active edge of skin lesion superficially scraped
with scalpel. Or hair pulled. Or nail clippings and
subungual debris collected and treated in a drop
of 10–20% KOH solution, on a glass slide for half
an hour or longer (hair and nails require several
hours). Viewed under microscope with subdued
Culture
Necessary for onychomycosis to differentiate
dermatophytic (tinea unguium) from non
Tinea capitis: diagrammatic representation of endothrix and
dermatophytic infections to optimize treatment.
ectothrix. In endothrix, organisms (spores or arthroconidia) inside Culture media used include Sabouraud's dextrose
the hair shaft while in ectothrix they are outside the shaft. agar and dermatophyte culture medium.
Tinea capitis, favus: depressed crusted lesions with cicatricial Tinea barbae: inflammatory boggy swelling with pustules, in
alopecia. beard area of adult male.
Tinea unguium, distal lateral subungual onychomycosis: man- Tinea unguium, proximal subungual onychomycosis: area of
ifests as distal and lateral yellow white subungual hyperkeratosis leukonychia in proximal part of nail plate.
and onycholysis.
Fungal Infections 25
Treatment
General Measures
Keep area dry and areated.
Tinea capitis: Use antifungal shampoos. Do not
share combs/brushes.
T. pedis: Avoid occlusive footwear.
T. cruris: Avoid synthetic underclothes.
Specific Therapy
Topical therapy
Topical therapy adequate for localized lesions
(except tinea unguium. And tinea capitis and
barbae). Regular application of any of the several
antifungal agents (creams, gels, lotions, powders)
of imidazoles (1% clotrimazole, 2% miconazole,
1% econazole, 2% ketoconazole, 0.5%
fluconazole), 1% tolnaftate, 1% ciclopirox olamine,
1% haloprogin, 1% naftifine hydrochloride, 1%
Culture of dermatophyte: Growth occurs in 7–14 days. Speciation terbinafine, 1% butenafine, 20% undecylinic
based on culture characteristic and morphology of micro and
acid and its salts. Continue treatment for a
macroconidia on microscopy.
couple of weeks, after clinical subsidence. Distal
Courtesy: Dr Immaculata Xess, Department of Microbiology, All
India Institute of Medical Sciences (AIIMS), New Delhi. involvement of single nail plate may be treated
with 1% amorolfine lacquer once a week. Or 8%
Histopathology ciclopirox lacquer daily for 8–9 months.
Rarely required. Fungal elements best visualized Systemic therapy
using special stains like PAS. Invariably needed for tinea unguium, tinea capitis
and tinea barbae. And extensive tinea corporis or
resistant tinea cruris infections.
Terbinafine: fungicidal. Treatment of choice
(except for some forms of tinea capitis, where
griseofulvin preferred). Given as 250 mg daily for
2 weeks (tinea corporis, cruris, pedis, manuum)
to 6 weeks (finger nail infection) to 12 weeks (toe
nail infection). Cost a limiting factor.
Itraconazole: Broad spectrum. Preferred to
terbinafine in onychomycosis which has not been
speciated by culture. Given as 400 mg 7 day
pulses every month for 2 (finger nail infection)-3
(toe nail infection) cycles.
Griseofulvin: Treatment of choice for tinea capitis
(caused by Microsporum spp). Given as 10 mg/
Histopathology of tinea pedis: PAS positive septate hyphae in
stratum corneum. kg, after a fatty meal for 6–12 weeks.
CandidaI paronychia: subacute inflammation of the posterior Candidal intertrigo: macerated and eroded interdigital space.
and lateral nail folds with detachment from nail plate. Deformity
of the nail plate secondary to involvement of nail matrix.
Candidal intertrigo: acutely inflamed erythematous lesions in Napkin candidiasis: acutely inflamed intertriginous areas with
the folds of skin. Note satellite vesiculo-pustules. satellite vesiculo-pustules.
Fungal Infections 27
CANDIDIASIS (CANDIDOSIS) red, swollen, tender and detached from the nail
plate. Occasionally a bead of pus can be expressed.
Etiology Nail plate deformed and discolored secondary to
involvement of the matrix (under the posterior nail
Candida albicans, a yeast-like fungus. Also C. fold). No mass under the nail plate. Many or all
glabrata, C. tropicalis or other species. Candida finger nails may be involved (c.f. tinea unguium).
a normal commensal of gastrointestinal tract and
vagina. Systemic broad spectrum antibiotics, Candidal Intertrigo
corticosteroid therapy, diabetes mellitus, HIV
infection and malignancies and their chemotherapy Common. Obesity and diabetes mellitus
predispose. predispose. Erythematous, raw, moist, macerated
and eroded areas with well-defined fringed edges
Clinical Manifestations and satellite superficial vesiculo-pustular lesions.
Itching or burning present. Interdigital spaces,
Several parts of body affected. groins, axillae or area under pendulous breasts.
Napkin Candidiasis
Infants or young children. Occlusive diapers, poor
local hygiene, use of topical steroids and systemic
broad spectrum antibiotics predispose. Acute
diaper eruption with angry looking intertriginous
denuded areas with or without fringed borders and
satellite vesiculopustules.
Genital Candidiasis
Predisposed by diabetes, intake of broad spectrum
antibiotics, oral contraceptives and pregnancy (in
women). Sometimes sexually transmitted:
Candidal balanoposthitis: macerated glans
and prepuce; lesions with fringed margin.
Candidal vulvovaginitis: thick white curdy
vaginal discharge. And extremely itchy,
erythematous vulvitis.
Oral Candidiasis
Disease of diseased generally. Several variants
described:
Acute pseudomembranous candidiasis: most
common variant. Neonates. And in diseased
mucosa (pemphigus vulgaris, oral lichen planus).
Discrete plaques surmounted by creamy/curdy
Sites of predilection: Candidiasis.
membrane. On buccal mucosa, palate, gums.
Acute atrophic candidiasis: use of broad
spectrum antibiotics and immunosuppression
Paronychia predisposes. Atrophic erythematous areas.
Infection of posterior and lateral nail folds. Seen in Chronic atrophic candidiasis: in denture wearing
adults. Housewives, washermen, cooks, bartenders elderly. Sharply demarcated erythematous
or people engaged in ‘wet’ work prone. Nail folds atrophic area on palate.
Candidal balanoposthitis: macerated glans and prepuce; eroded Vaginal discharge due to candida: Curdy white discharge coat-
lesions have fringed margins. ing the vaginal walls and cervical os.
Oral candidiasis, acute pseudomembranous: discrete and Oral candidiasis, chronic hyperplastic variant: adherent white
confluent 'curdy' lesions in a patient with widespread metastatic plaque on tongue of a smoker. With permission Elsevier. Khanna
malignant lesions. N. Illustrated Synopsis of Dermatology and STDs.
Fungal Infections 29
Etiology
Caused by:
Fungi: called eumycotic mycetoma. Common
organisms include Madurella mycetomatis, M.
grisea.
Actinomycetes: called actinomycotic
mycetoma. Common organism include
Actinomadura madurae, A pelletieri.
Epidemiology
Adults, often males.
Clinical Manifestations
Potassium hydroxide mount of candida: showing budding Ill-defined, firm to woody-hard, subcutaneous
yeasts and pseudohyphae.
swelling that develops sinuses discharging
seropurulent fluid containing white or colored
granules (microcolonies of organisms). Affected
part irregularly enlarged and deformed. Underlying
muscles and bone(s) may be involved. Insidious
Culture progression over years; simultaneous healing with
Usually not necessary. Except for speciation in scars and deformities. Foot (so Madura foot) or
treatment of poorly-responsive patients. other parts of the body. No lymphadenopathy. No
systemic involvement.
Treatment
Investigations
General Measures
Maintain scrupulous dryness of involved Direct Microscopy
part. Rinse off soap thoroughly. Diabetes
Fungi (or bacteria) demonstrable on direct
mellitus, obesity, other endocrinopathies or
microscopy of granules in a 10–20% KOH
immunodeficiency states should be corrected.
preparation or using Gram stain.
Mycetoma foot: woody-hard subcutaneous swelling with mul- Mycetoma: multiple nodules with discharging sinuses on foot–so
tiple nodules and sinuses. called madura foot.
Mycetoma: multiple black granules in pus collected on a gauze Mycetoma thigh: multiple nodules with discharging sinuses.
piece, left covering the sinus for 24–48 hours.
Fungal Infections 31
Culture
To differentiate actinomycotic and eumycotic
mycetoma.
Histopathology
Histological sections using hematoxylin and eosin
stains and preferably PAS or Gram’s stain. Shows
chronic inflammatory reaction with neutrophilic
abscesses and scattered giant cells and grains in Histopathology of mycetoma: hematoxylin and eosin stain
center. (top); Gram's stain (middle); Ziehl-Neelsen stain (bottom).
Subcutaneous zygomycosis: firm subcutaneous swelling of the Subcutaneous zygomycosis: firm subcutaneous swelling of
arm with an erythematous halo. central face, with well-defined margin.
Subcutaneous zygomycosis: facial form in adults. Responded to Sporotrichosis, lymphangitic variant: granulomatous, lesions,
potassium iodide dramatically. arranged linearly due to lymphatic spread. Permission with Elsevier.
Fungal Infections 33
Treatment
Actinomycotic Mycetoma
High doses of dapsone (200 mg/day) or
cotrimoxazole preferably combined with another
antibiotic such as tetracycline for 6 months or
longer. Rifampicin and amikacin also useful in
some patients. Often given as a 2 step regime.
Eumycetoma Mycetoma
Generally not amenable to chemotherapy. Dapsone
or newer antifungals of imidazole (ketoconazole)
or triazole (itraconazole, fluconazole) group
or amphotericin B (highly toxic) may be tried. Histopathology of subcutaneous phycomycosis: fungus pre-
Unresponsive mycetomas require surgical sent as large strap-like elements without septae. Note eosinophils.
intervention, even amputation.
SUBCUTANEOUS ZYGOMYCOSIS
Treatment
Etiology Drug of choice, saturated solution of potassium
iodide. Most lesions respond (almost dramatically)
Caused by Basidiobolus haptosporus (in children)
to 15–45 drops/day for several weeks. Iodism
or Conidiobolus coronatus (in young adults).
temporary and reversible. Treat resistant or
relapsed cases with itraconazole 400 mg/day
Clinical Manifestations
orally for several weeks.
Rare. Asymptomatic, gradually progressive, firm
subcutaneous swelling, freely movable over
SPOROTRICHOSIS
deeper structures but adherent to overlying skin.
Well marginated mass characteristically lifted up
Etiology
by inserting the fingers underneath. Erythematous
periphery. No lymphadenopathy. On buttocks, limbs Sporothrix schenckii. Natural habitat, wood and
in children. And face, paranasal areas in adults. soil.
Investigations Epidemiology
Wide geographic distribution. Common in North
Direct Microscopy Eastern parts of India and foot hills of Himalayas.
Not usually helpful.
Clinical Manifestations
Culture Infection preceded by an injury, often a wood prick.
Organisms readily cultured on SDA. Two variants recognized:
Lymphangitic: initial noduloulcerative lesion;
Histopathology spreads in a linear fashion along the lymphatics.
Chronic granuloma with large number of Intervening lymphatic cord between two lesions,
eosinophils. Fungus presents as large strap like pathognomic.
fungi without cross walls, often surrounded by Fixed: single noduloulcerative plaque. Regional
refractile eosinophilic material (Splendore-Hoeppli lymph nodes may be enlarged. Exposed parts of
phenomenon). body affected.
Sporotrichosis, fixed variant: single noduloulcerative plaque on Chromomycosis: early superficially ulcerative and vegetative
hand (exposed parts). Face another common site. lesions.
Chromomycosis: multiple vegetative lesions on the lower leg. Chromomycosis: multiple vegetating nodules with black dots.
Fungal Infections 35
Investigations Treatment
Direct Microscopy Potassium iodide (KI) is almost specific. Five drops
KOH mount rarely positive. of saturated KI gradually increased to 15 drops
orally, three times a day. Treatment continued
Culture for several weeks after clinical cure. Itraconazole
Ready isolated on SDA. 100–200 mg/day and terbinafine 250–500 mg/
day, useful alternatives. Local application of heat
useful in some patients.
CHROMOMYCOSIS
Etiology
Caused by several saprophytic, pigmented
pathogens of species of Phialophora, Fonsecaea
and Cladophialophora.
Clinical Manifestations
Adult males. Multiple, hyperkeratotic, warty, rough,
dry plaques or cauliflower-like hard tumors with
healthy intervening areas. May assume massive
proportions (mossy foot). Black dots often surmount
plaques. Satellite lesions along lymphatics. Feet and
Culture of Sporothrix schenkii: leathery moist cream colonies legs commonly affected. General health unaffected.
which darken with age.
Courtesy: Dr Immaculata Xess, Department of Microbiology, All
India Institute of Medical Sciences (AIIMS), New Delhi.
Investigations
Direct Microscopy
Histopathology
Skin scraping taken from skin surface. Best to take
Mixed granuloma with neutrophil microabscesses.
a black dot. Shows cluster of pigmented cells.
Sometimes small cigar shaped/oval yeast cells
with surrounding asteroid body demonstrated.
Cryptococcosis: close up of molluscum-like lesion. Note central Cryptococcosis: sharply marginated, ulcerated plaques.
crater.
Fungal Infections 37
Culture CRYPTOCOCCOSIS
Black colonies.
Etiology
Histopathology Caused by Cryptococcus neoformans—a fungus
Histopathology confirmatory. Foreign body of low virulence. Immunocompromised individuals
granuloma with microabscesses. And brown fungal predisposed. Portal of entry, respiratory tract.
cells, in giant cells or in microabscesses.
Epidemiology
Rare. Widespread geographic distribution.
Clinical Manifestations
Central nervous system involvement, a dominant
feature. Skin lesions infrequent and nonspecific–
acneiform papules, molluscum contagiosum–
like lesions, crusted/vegetating plaques. Rarely
primary cutaneous cryptococcosis.
Investigations
Culture
Easy to culture.
Histopathology of chromomycosis: foreign body granuloma
with microabscesses and brown fungal cells.
Treatment
Systemic itraconazole or terbinafine combined
with oral flucytosine often successful. Flucytosine
with amphotericin B, an alternative. Ketoconazole
not effective. Surgical removal of small lesions,
preferably in combination with antifungal therapy.
Treatment given till clinical resolution (several
months).
LOBOMYCOSIS
Culture of cryptococcus neoformans: typical mucoid colonies.
(KELOIDAL BLASTOMYCOSIS) Confirmed by microscopy and biochemical tests.
Rare. Caused by Lacazia loboi. Cutaneous lesions Courtesy: Dr Immaculata Xess, Department of Microbiology, All
include nodules or plaques that progress insidiously to India Institute of Medical Sciences (AIIMS), New Delhi.
keloids/hypertrophic scars. May ulcerate. On exposed
sites (face, legs, arms). No lymphadenopathy. No
Histopathology
systemic involvement.
Skin lesions non-specific, so require histopatho-
Treatment logical confirmation. Pauci inflammatory or
granulomatous lesion. Organisms visible as
Unsatisfactory. Excision of localized lesions an
encapsulated budding yeasts. Special stain
option.
mucicarmine, Indiana ink, Nigro stain.
Histoplasmosis, disseminated in an HIV positive patient: mul- Histoplasmosis, disseminated: multiple papular lesions.
tiple, disseminated crusted nodules and plaques. Diagnosis often
made histopathologically.
Histoplasmosis, primary cutaneous: well-defined erythema- Histoplasmosis, disseminated: oral ulcers in a patient with dis-
tous scaly nodule which on biopsy revealed histoplasmosis. seminated histoplasmosis. Always rule out Addison's disease in
patients with disseminated histoplasmosis.
Fungal Infections 39
HISTOPLASMOSIS
Etiology
Histoplasma capsulatum, an environmental sapro-
phyte. Immunosuppressed individuals predisposed.
Acquired by inhalation of spores.
Epidemiology
Widespread geographic distribution.
Histopathology of cutaneous histoplasmosis: H. capsulatum is
Clinical Manifestations seen packed in macrophages as tiny yeast forms.
Spectrum of asymptomatic infection, benign Courtesy: Prof M Ramam, All India Institute of Medical Sciences
symptomatic infection to progressive symptomatic (AIIMS), New Delhi.
infection. Respiratory infection either acute, or
chronic. Acute disseminated infection in AIDS
Histopathology
patients. Skin lesions non-specific—small papules Histopath confirmation often necessary.
(molluscum-like) to warty/ulcerated lesions. Chronic H.capsulatum appears as intracellular yeast
disseminated infection presents with oral ulcers and cells. Special stains include GMS with which the
Addison's disease. Rare primary cutaneous infection. organism stains black.
Investigations Serology
Serological tests available both for antibody as
Direct Microscopy also antigen detection. Latter especially useful in
Most frequently from sputum and blood. AIDS patients.
Culture Treatment
Confirmed by culture. Always warn the lab, as For localized forms, itraconazole. And for
highly infectious. disseminated forms, amphotericin B.
3 Viral Infections
Verruca vulgaris foot: flat-topped and verrucous lesions. Linear Periungual warts: variant of verruca vulgaris which often distorts
spread due to auto inoculation. the nail.
Plane warts: flat-topped papules on face. Superficial plantar warts (mosaic warts): painless, warts, often
confluent.
Viral Infections 43
Viral infections of the skin may be purely cutaneous Plane and common warts: typical lesions;
such as warts and molluscum contagiosum. Or be penile shaft.
part of an exanthem of a systemic viremia such Bowenoid papulosis: velvety brown papules.
as variola, measles and varicella. Since viruses Buschke Lowenstein tumor: giant condyloma
multiply within living cells, skin infections generally acuminata or verrucous carcinoma.
confined to the epidermis.
Filiform Warts
WARTS Keratotic protruberances. In beard region.
Verruca Vulgaris
Common warts. Children or young adults. Firm,
irregular verrucous papules. Vary in number, size
and shape. Distributed anywhere on the body, but
more particularly on the exposed trauma prone sites.
Variant: periungual warts which often distort nails.
Verruca Plana
More frequent in children. Asymptomatic. Smooth,
skin-colored, flat-topped papules. Trauma (such
as caused by shaving the beard) promotes spread
by autoinoculation (erroneously termed Koebner’s
isomorphic phenomenon). On the face or hands. Histopathology of warts: shows koilocytes which are epidermal
cells with hyperchromatic irregular nuclei and a perinuclear halo.
Plantar Warts
Two variants described: Treatment
Mosaic warts: painless, superficial warts often Spontaneous resolution of warts not uncommon.
confluent. Several treatment modalities available. Destroy
Myrmecia: painful, deeper keratotic papules
mechanically, with cryotherapy or chemical or electric
with a central depression. cautery or surgically. Or treat with immunotherapy.
Anogenital Warts Cryotherapy: with liquid nitrogen using a cotton tip
Sexually transmitted. Several variants described: applicator/spray. Or carbon dioxide slush or stick.
Condyloma acuminata: erythematous fleshy Chemical cautery: scrape with a curette and
lesions with finger like projections. On mucosal cauterize with 50–100% trichloroacetic acid or
surface. phenol. Deep curetting results in avoidable scars.
Deep plantar warts (myrmecia): paring off of the keratotic Condyloma acuminata: verrucous, fleshy plaque on glans. With
plaque will reveal a central irregular core. permission.
Bowenoid papulosis: multiple, small hyperpigmented papules Filiform warts: keratotic protruberance in the neck.
with a velvety surface on upper thigh. Diagnosis confirmed
histopathologically-shows carcinoma in situ.
Viral Infections 45
MOLLUSCUM CONTAGIOSUM
Dermoscopy in molluscum contagiosum: shows central core
Etiology and corona of blood vessels.
Molluscum contagiosum: with secondary infection. Measles: pink macular rash on the trunk.
Viral Infections 47
Giemsa stain of extruded material from molluscum contagio- Histopathology of molluscum contagiosum: shows eosino-
sum lesion: shows intracytoplasmic bodies. philic intracytoplasmic bodies (Henderson-Patterson bodies)
pushing nucleus to periphery (signet ring appearance).
MEASLES
Etiology
A paramyxovirus, measles virus.
Epidemiology
Common exanthem seen in children. Infants
protected because of maternal antibodies.
Clinical Manifestations
Incubation period about 10 days.
Two phases of infection:
Normal saline mount of crush smear of molluscum body: Prodromal/catarrhal phase: accompanied with
shows multiple ballooned up keratinocytes stuffed with round malaise and respiratory catarrh; second day,
intracytoplasmic molluscum bodies.
bluish white spots surrounded by erythematous
halo in the buccal mucosa near the premolars-
Biopsy Koplik’s spots.
Exanthematous phase: disease infectious
Histopathology confirmatory. Shows typical intra-
in this phase. Fourth day, pink macular rash,
cytoplasmic eosinophilic bodies (Henderson-
spreads in 24 hours from face to trunk and
Patterson bodies) pushing nucleus to periphery
limbs. Later papules, discrete or confluent.
(signet ring appearance).
Desquamates in about 10 days.
Primary genital herpes: closely grouped vesicles (some umbili- Primary genital herpes: multiple, erythematous superficial
cated) on the vulva, almost appearing as a white plaque. This erosions, present circumferentially on prepuce and on glans.
patient is pregnant. Difficult to retract the prepuce. Patient had bilateral tender lym-
phadenopathy and constitutional symptoms.
Recurrent herpes simplex: grouped vesicles; the adjoining scar Recurrent genital herpes: grouped tiny vesicles on shaft of penis.
is indicative of a previous attack. No lymphadenopathy or constitutional symptoms.
Viral Infections 49
Complications
Eczema herpeticum (Kaposi’s varicelliform
eruption): generalized HSV infection in patients
Classification of herpes simplex infection with underlying skin disease like pemphigus
and atopic dermatitis. Diagnosis often needs
Epidemiology cytopathologic/culture confirmation.
Erythema multiforme: herpetic infection may
Primary infection, generally subclinical (so
uncommon) but florid in immunodeficient or be followed, weeks later, by targetoid lesions.
atopic subjects. Recurrent herpes simplex
common. Extragenital infection seen in all age Investigations
groups, while genital infection, seen in sexually Diagnosis generally clinical. Sometimes needs
active patients. evaluation, especially in genital lesions.
Genital herpes in HIV positive patient: widespread polycyclic Eczema herpeticum: polycyclic lesions in a patient with under-
lesions on the vulva, mons and lower abdomen. Note grouped lying pemphigus. Other conditions which predispose include
lesions on thighs. atopic dermatitis.
Complication of genital herpes: recurrent erythema multiforme Chicken pox: papules and umbilicated vesicles in different stages
associated with recurrent GH. of evolution.
Viral Infections 51
Chickenpox (Varicella)
Etiology
Varicella-zoster virus (VZV).
Epidemiology
Direct immunofluoresence test on Tzanck smear in herpes: Common. Spread via droplet infection. Commoner
showing apple green intracellular fluorescence. in children, severe in adults.
Herpes zoster: groups of vesicles on an erythematous back- Trigeminal zoster: only mandibular division of the nerve
ground; dermatomes T6 and T7 affected; unilateral distribution. involved.
Trigeminal zoster: only ophthalmic division of trigeminal nerve Herpes zoster in an HIV-positive patient: involvement of trunk
involved. in dermatomal pattern. Deeper ulcers with necrotic slough a clue
to test for underlying HIV infection.
Viral Infections 53
Treatment
General Measures
Antihistamines for relief of itching (varicella) and
analgesics for pain (zoster).
Specific Treatment
For chicken pox in children, no treatment. For
adult and immunocompromised-acyclovir,
famciclovir or valaciclovir. For zoster acyclovir
(2–4 g/day), famciclovir (250 mg, 3 times a day)
or valaciclovir (500 mg, twice a day) for 5–7 days
given, within the first couple of days, may abort
an attack of zoster and prevent post herpetic
Histopathology of varicella: reticular and ballooning degenera- neuralgia. Amitriptyline (25 mg, thrice a day) or
tion of epidermal cells. gabapentine (1,800–3,600 mg/day) effectively
control post herpetic neuralgia.
4 Leprosy
Lepromatous leprosy: mild diffuse cutaneous infiltration. Lepromatous leprosy: infiltration of the skin with complete loss
of eyebrows.
Lepromatous leprosy: infiltrated skin thrown into folds giving Lepromatous leprosy: diffuse ear lobe infiltration with nodulation.
leonine facies.
Leprosy 57
Lepromatous leprosy ocular involvement: uveitis and corneal Histoid leprosy: succulent nodules on an apparently normal skin.
opacities.
Leprosy 59
Borderline tuberculoid leprosy: lesion shows alopecia, infil- Borderline tuberculoid leprosy: uniformly infiltrated plaque.
trated edge and a satellite lesion.
Leprosy 61
Borderline Lepromatous (BL) (always) on the face. Or thigh or any other part of
Much like LL. Most lesions small macules, plaques the body. Doubtful or minimal loss of sensations.
or nodules with ill-defined edges; some lesions are Variable course: transforms into any of the
large and simulate BB or BT. Face and earlobes often polar forms or borderline group. Or more often
infiltrated with or without nodulation and madarosis. spontaneously resolves without any residue; nerve
Sensory loss late and less pronounced. Nerve trunk thickening not definite. Demonstration of bacilli or
thickening relatively late and bilaterally symmetrical. damage to nerves on histology confirmatory.
Systemic involvement less prominent. May upgrade
to BT or BB. Lepromin skin test negative. REACTIONS IN LEPROSY
(LEPRA REACTIONS)
Primary Neuritic Leprosy Acute exacerbations in course of the disease,
Nerve involvement without a skin lesion. Spectral due to immunological changes. Triggered by
classification difficult; most patients possibly specific chemotherapy, emotional/physical stress,
borderline. Peripheral nerve(s) thickened (and pregnancy or intercurrent infections. Two types
sometimes tender); unilateral or bilateral. Any recognized: Type I and Type II.
nerve, though sometimes generally ulnar, radial,
lateral popliteal, trigeminal and facial affected. Type I
Gradual sensory loss or motor deficit: paralysis, Seen in BT, BB, BL (borderline forms). Acute
deformities, painless thermal burns or mechanical exacerbations due to rapid alteration of leprosy-
injuries, trophic changes and non-healing ulcers. specific CMI of the host. Skin lesions acutely
inflamed–red, edematous and tender; may
Indeterminate Leprosy desquamate or ulcerate. Nerves painful and
Non-classifiable early lesion suggestive of leprosy. tender; may develop nerve abscesses or palsies
Ill-defined, often a solitary hypopigmented macule with increase in anesthesia, loss of motor function
Borderline leprosy: ‘inverted saucer’ lesion with elevated slop- Borderline leprosy: ‘inverted saucer’ lesion with elevated slop-
ing border. ing border.
Leprosy 63
Nerve involvement in leprosy: visible thickened greater auricu- Nerve involvement in leprosy: bilateral facial palsy.
lar nerve.
Nerve involvement in leprosy: claw hands due to bilateral ulnar Nerve involvement in leprosy: trophic ulcer. Note hyperkera-
and median nerve palsy. totic edge.
Leprosy 65
Lepromatous Leprosy
Diffuse monomorphic infiltrate of vacuolated, foamy,
Histopathology of tuberculoid leprosy: tuberculoid curvilinear
macrophages laden with AFB often in clumps, called granuloma consisting of epitheliod cells and giant cells with a
globi. Lymphocytes conspicuously few. Overlying rim of lymphocytes at the periphery. Note granuloma encroach-
atrophic epidermis separated by a grenz zone. ing epidermis.
Type I reaction in borderline tuberculoid leprosy: erythema- Type I reaction in borderline tuberculoid leprosy: erythema-
tous, edematous and scaly plaque. tous edematous plaque.
Type I reaction in borderline leprosy: edema and erythema of Type I reaction in borderline lepromatous leprosy: large plaque
pre-existing lesions. with erythema and edema. Patient has infiltration of ear lobes.
Leprosy 67
Borderline Leprosy
Midway between lepromatous and tuberculoid
leprosy; subepidermal zone free; acid-fast bacilli
often demonstrable.
Reactions in Leprosy
Reversal type 1 reaction
Disorganization of granuloma with edema and
infiltration with polymorphs.
Type 2 reaction
Lepromatous granuloma superimposed with acute
inflammation in form of polymorphs and edema.
Treatment
For purpose of treatment all patients classified
into paucibacillary (≤5 lesions, usually
bacteriologically negative). Or multibacillary
(bacteriologically positive) now defined by WHO as
>5 lesions. Monotherapy with sulfones absolutely
Histopathology of borderline tuberculoid leprosy: tubercu- inadequate. Administer combination therapy with
loid granulomas with free subepidermal zone and perifollicular dapsone and rifampicin in paucibacillary types;
location. add clofazimine in multibacillary types.
Type II reaction in lepromatous leprosy: erythema nodosum Erythema nodosum leprosum necroticans in lepromatous
leprosum, erythematous, edematous plaques. leprosy: erythematous tender nodules of erythema nodosum
leprosum which have necrosed.
Multidrug therapy of leprosy: WHO supplied blister packs: A. for Side effect of treatment: clofazimine pigmentation.
paucibacillarly leprosy (adult); B. for multibacillary leprosy (adult).
Leprosy 69
Reactions Immunotherapy
Always continue antileprosy therapy. Rest, MDT kills M. leprae, but does not upgrade
appropriate splints or other physiotherapeutic immune response. Additional immunotherapy
measures for the affected part. induces faster bacterial clearance and decreases
incidence, frequency and severity of reactions.
Type I reaction Agents used include BCG, Mw, killed M. leprae,
Depends on severity: ICRC strain, M. vaccae and M. habana. Role in
Mild
type I reactions: Non-steroidal anti prophylaxis debatable.
inflammatory drugs (NSAIDs).
Severe type I reactions: Oral steroids drugs
Other Measures
of choice, particularly in patients with severe Complications need to be treated.
5 Cutaneous
Tuberculosis
Lupus vulgaris: early lesion-erythematous plaque with barely Lupus vulgaris: erythematous scaly plaque showing progression
discernable scarring. Face a common site. at one edge and scarring at the other. The edge is studded with
papules which on diascopy presents an apple jelly appearance.
Lupus vulgaris: infiltrated annular plaque with central scarring. Lupus vulgaris: infiltrated plaque with central scarring. Lesions
Note development of nodules in the scarred area. sometimes become hypertrophic and verrucous.
Cutaneous Tuberculosis 73
Tuberculosis verrucosa cutis: irregular hyperkeratotic lesion on Tuberculosis verrucosa cutis: thick, hyperkeratotic plaque.
the sole. Scarring seen below the plaque, suggestive of centrifugal spread.
Cutaneous Tuberculosis 75
Slow progression. Spontaneous healing unusual. the several triggers. Includes erythema nodosum
and erythema induratum.
Scrofuloderma
True Tuberculides
Children or young adults. Skin involvement
secondary to an underlying tubercular focus lymph Lichen scrofulosorum
node, bone or joint or testis. Uncommon. Seen mainly in children. Appear
as crops of asymptomatic grouped, small, flat
Morphology topped, lichenoid, follicular, pale or skin colored
Combination of soft fluctuant nodules and chronic papules with fine scales. Symmetrically on trunk,
ulcers and sinuses with undermined edge and extremities. Involute over months, without residue.
bluish hyperpigmentation. Serous discharge
with thick crusting. Heals with irregular scarring. Papular and papulonecrotic tuberculide
Surrounding skin may show features of lupus Appear as crops of bilaterally symmetrical papules
vulgaris. and nodules; may necrose on top. Heal without
residue. Or with scarring.
Tuberculosis verrucosa cutis: irregular hyperkeratotic indurated Scrofuloderma: combination of soft fluctuant nodules and
plaque with minimal scar. chronic ulcers and sinuses with undermined edge and bluish
hyperpigmentation. Lesions occur in relation to lymph nodes,
joints, or bone or testis.
Tuberculous gumma: multiple subcutaneous fluctuant nodules Lichen scrofulosorum: asymptomatic lichen nitidus-like scaly
will eventually rupture to form sinuses like in scrofuloderma. papules, symmetrically present on the trunk. Tuberculin test was
strongly positive. Patient responded to ATT.
Cutaneous Tuberculosis 77
Facultative Tuberculides
Erythema nodosum Tuberculin skin test (Mantoux test): strongly positive bullous
Uncommon. Not all patients have associated reaction in a patient with lichen scrofulosorum; reflects good cell
tuberculosis; other causes such as sarcoidosis, mediated immune response.
streptococcal throat infections, drugs, Behcet’s
syndrome. Children and young adults affected, more
often females. Painful and tender, non-ulcerating
erythematous or purplish nodules. Each lesion
self-limiting (2–6 weeks). Fresh lesions continue to
appear. Resolve with hyperpigmentation. Bilateral
and symmetrical, usually on the shins. Mild to
moderate constitutional symptoms: fever, arthralgia.
INVESTIGATIONS
Always rule out tuberculosis of other organs by
doing relevant investigations.
Tuberculin Test
Indicates immunity. Variable in lupus vulgaris, Lupus vulgaris: diffuse granulomatous infiltrate consisting of
negative in tuberculous gumma and positive in epithelioid cells, lymphocytes, plasma cells and giant cells; top
scrofuloderma, tuberculosis verrucosa cutis. And (10x), bottom (40x).
strongly positive in tuberculides.
Papulonecrotic tuberculide: erythematous nodules surmounted Papulonecrotic tuberculide: erythematous papules and nodules
with necrosis. Note symmetry. Patients tuberculin test was which over period of time developed necrosis.
strongly positive and he responded to antituberculous treatment.
Papulonecrotic tuberculid of glans: Recurrent episodes of Erythema nodosum: painful, tender erythematous nodules on
necrotic ulcer. Note healed punched out and bridging scars shin, which appear in crops. Resolve with hyperpigmentation in
(worm eaten appearance). 2–6 weeks.
Cutaneous Tuberculosis 79
SCABIES
Etiology
Female of mite Sarcoptes scabiei var. hominis.
Epidemiology
An extremely common disease of the poor and
underprivileged. Spreads by close personal
contact; role of fomites uncertain, perhaps
unimportant. Crowded living conditions and poor
personal hygiene predispose. History of similar
complaints in family or contacts often present. Also Sites of predilection: Scabies.
sexually transmitted.
Norwegian scabies: presenting as exfoliative dermatitis. Norwegian scabies: hyperkeratotic lesions on the dorsa of the
hands.
Diseases Caused by Arthropods and Parasites 85
Skin scraping in Norwegian scabies: many acari seen in a scale Histopathology of Norwegian scabies: acari in the stratum cor-
(top); close-up view (bottom). neum, acanthosis and a perivascular infiltrate of lymphocytes,
histiocytes and eosinophils (above); close-up view (below).
Biopsy
Shows acari in stratum corneum. And acanthosis and benzyl benzoate, 10–25% lotion/emulsion.
and a perivascular infiltrate of lymphocytes and Intralesional steroids used in nodular scabies.
eosinophils.
Systemic treatment
Treatment Ivermectin (200 g/kg), the only systemic agent.
Used for Norwegian scabies and scabies epidemics
General Measures
in institutions (orphanages, old age homes).
All family members to be treated simultaneously.
Itching (treated with antihistamines) persists for
PEDICULOSIS
about 1–2 weeks, even after successful treatment;
re-evaluate patient, if persists longer. Etiology
Pediculus humanus capitis (head louse). Or Pediculus
Specific Treatment humanus corporis (body louse). Or Phthirus pubis
Antiscabitics, topical and systemic. (pubic louse). Pediculus humanus capitis and
Pediculus humanus corporis dorsoventrally flattened
Topical treatment
and oblong. Pubic louse (crab-louse) greater in width
Include permethrin, 5% cream, lindane (gamma than length. Lice, obligate parasites. Blood-suckers
benzene hexachloride, GBH), 1% lotion/cream literally. Cannot survive on inanimate objects.
Pediculosis capitis: adult louse (L) appears as greyish white Pediculosis corporis: nits adherent to the seams of clothes.
insects stuck to scalp or seen crawling. Generally more frequently
nits (N) are seen as whitish oval particles attached to hair.
Pediculosis pubis: a louse and nits stuck to the hair. Pediculosis pubis: louse and nits attached to pubic hair.
Diseases Caused by Arthropods and Parasites 87
Chronic cutaneous leishmaniasis: with simultaneous progress Mucocutaneous leishmaniasis: edematous nodules, some ulcer-
and scarring. ated on the lips and nose. Often leads to scarring and mutilation.
Diseases Caused by Arthropods and Parasites 89
Post kala-azar dermal leishmaniasis: extensive facial involve- Post kala-azar dermal leishmaniasis: extensive hypopigmented
ment. Lesions predominantly centrofacial. macules.
Diseases Caused by Arthropods and Parasites 91
Tissue smear of post kala-azar dermal leishmaniasis: stained Histopathology of cutaneous leishmaniasis: dense mixed infil-
with Giemsa stain showing amastigote forms of leishmania (LD trate of eosinophils, lymphocytes, plasma cells and macrophages
bodies) both within and outside macrophages. filled with LD bodies.
Molecular Diagnosis
Kinetoplast minicircle DNA, highly sensitive and
100% specific.
Leishmanin Test
Montenegro test. Positive in most forms, except
in initial phase of infection and in disseminated
cutaneous leishmaniasis.
Serology
Elisa test to detect antibodies against various
antigens of Leishmania spp, most notabily rr 39
and rk 16. Useful in visceral leishmaniasis and
may be in PKDL.
Histopathology
Dense diffuse mixed infiltrate of histiocytes,
lymphocytes, plasma cells (sometimes with
intracytoplasmic eosinophilic Russel bodies),
and eosinophils. Typically intracytoplasmic (in
histiocytes) and extracellular dot-dash LD bodies
diagnostic. Giemsa stained slides more sensitive.
Chronic cutaneous leishmaniasis usually organism
poor, with either diffuse or nodular non-caseating
tuberculoid granuloma.
Treatment
Histopathology of cutaneous leishmaniasis: Giemsa stained
Cutaneous Leishmaniasis slide showing macrophages containing basophilic stained LD
bodies; top (40x), bottom (100x).
Intralesional pentavalent antimony, 15%
paramomycin.
Filarial elephantiasis of the right leg and foot: lymphedema, hyperplasia of connective tissue with verrucosity of skin.
Lymphedema: close up of secondary changes—verrucosity and Wound myiasis: maggots in the wound.
nodulation.
Diseases Caused by Arthropods and Parasites 93
Post Kala-azar Dermal Leishmaniasis usually along with albendazole. DEC also used as
Pentavalent sodium antimony gluconate 10–20 mass drug administration (MDA) usually as fortified
mg/kg/day for 90–120 days, given parenterally. salt. Ivermectin also effective against microfilariae.
Ketoconazole, fluconazole, allopurinol, rifampicin Adult filariae not amenable to treatment.
given alone or in combination with antimony. If Surgical Treatment
available miltefosine, 2.5 mg/kg/day orally for
28 days, is now first line of treatment. Elephantiasis needs surgical intervention—
creation of lymphnode venous-shunt. Or/and
removal of subcutaneous tissue.
FILARIASIS
Insect bite hypersensitivity: excoriated papules on exposed Pedrus dermatitis: due to chemicals from beetles; kissing lesions
sites. In an adult should arouse suspicion of an underlying lym- occur in flexural areas.
phoreticular malignancy or HIV infection.
Diseases Caused by Arthropods and Parasites 95
CYSTICERCOSIS Treatment
Uncommon. Results from ingestion of food or General Measures
drink contaminated with the eggs of Taenia solium, Protection by physical barriers or insect repellents.
subcutaneous, firm, asymptomatic 1–2 cm oblong Antihistamines. Secondary bacterial infections
nodules (larvae of taenia—Cysticercus cellulosae), appropriately treated.
vary in numbers. Brain, muscles and eyes may
also be affected—may manifest as epilepsy or Specific Treatment
intracranial space occupying lesions.
Acute lesions treated with topical corticosteroids.
Treatment
Surgical excision for skin lesions. Rule out
DRACUNCULOSIS
neurocysticercosis. (GUINEA WORM INFESTATION)
Now eradicated. Caused by nematode-Dracunculus
INSECT BITES/REACTIONS medinensis. Acquired by drinking water contaminated
with parasitized cyclops (water fleas). The adult
Etiology female dracunculus rests quietly in the subcutis,
Common, particularly in the tropics. Caused by commonly of legs and feet. Emerges only on contact
hypersensitivity. Or injection squirting of ‘toxic’ with water to discharge larvae. Blister or a superficial
substances. ulcer with the worm protruding. Prodrome of fever,
urticaria, dyspnea. Cellulitis and abscess formation
Epidemiology may complicate.
Children or adults. Seasonal.
CUTANEOUS LARVA MIGRANS
Clinical Manifestations (CREEPING ERUPTION)
Papular Urticaria Etiology
Common. Manifestation of hypersensitivity to Migratory skin lesions produced by movements
insect bites. Children, 2–7 years. May manifest within the skin of larvae of non-human hookworm
in immunocompromised adults (lymphoreticular (Ancylostoma braziliense or A. caninum). Normally
malignancies, immunosuppression and HIV harbored in the intestines of dogs and cats.
infection). Frequent in summers, remits in winter. Acquired on beaches or locations where soil
Pruritic papules and weals or a ‘combination-lesion’ contaminated with fecal matter.
on an erythematous background. Individual lesions
self-limiting. Excoriations and secondary bacterial Clinical Manifestations
infection frequent. Exposed sites.
Uncommon. Itchy, bizarre, tortuous, thread-like,
pink or skin colored migratory ridge with tiny
Paederus Dermatitis
vesiculation. Variable course, largely self-limiting.
Outbreaks common at the end of rainy season. Commonly seen on feet, hands, buttocks.
Acute erythematous vesicular reaction caused by
beetle of genus Paederus (commonly Paederus Treatment
sabaeus) due to a vesicant, paederin, released
Local application of 10% thiabendazole suspension
when the beetle is crushed. Kissing lesions
3–4 times a day for 7–10 days. Ivermectin 200 g/
common. Exposed sites-face, limbs.
kg, single dose, repeated after 7 days.
Dracunculosis: guinea worm protruding out of an ulcer. Creeping eruption: tortuous thread-like ridge.
Onchocerciasis: lichenified and eroded dermatitis. Onchocerciasis: late depigmented lesions on the shins-leopard
skin.
Diseases Caused by Arthropods and Parasites 97
Vectors Investigations
Members of Simuliidae family particularly Simulium Diagnosis based on clinical features. And
damnosum. confirmed by demonstration of microfilariae in
skin snips.
Epidemiology
Common in Africa, Latin America and Yemen.
Treatment
Ivermectin, 100–200 g/kg orally, single dose,
Clinical Manifestations causes pronounced reduction of microfilariae
in the skin for 6–12 months. Repeat treatment.
Pruritus. Localized acute (urticarial papules or
Alternatively use diethylcarbamazine. Suramin
pustules), chronic (excoriated papules or flat topped
kills adult worms.
scars), or lichenified dermatitis accompanied by
atrophy.
7 Dermatitis and
Eczema
Atopic dermatitis, infantile phase: bilaterally symmetrical papulovesicular lesions on an erythematous background.
Atopic dermatitis, infantile phase: disseminated lesions all over Atopic dermatitis, childhood phase: bilaterally symmetrical
the body. lichenified and excoriated lesions in the flexures of the knees.
Dermatitis and Eczema 101
Etiology
Reaction pattern to endogenous or exogenous
stimuli. Genetic factors important in atopic while
exogenous factors paramount in contact (irritant/
allergic) dermatitis, diaper dermatitis. Also Infantile phase
important to some extent in atopic dermatitis. Common. Often first born male infant. Onset at
about 3rd month. Itching severe and paroxysmal.
Histology Bilaterally symmetrical, papulovesicular, exudative
Epidermal intercellular edema (spongiosis) in acute and crusted lesions. Cheeks predominantly
eczema and acanthosis (hyperplasia of stratum involved. Later on extensors of extremities and
malpighi), hyperkeratosis and parakeratosis trunk. Secondary bacterial infections common.
(premature keratinization) in the subacute and Spontaneous remissions and relapses. May
chronic phase. Dermal inflammatory infiltrate, completely remit by about 2–3 years. Or may evolve
polymorphonuclear or lymphocytic, pronounced into childhood phase.
in acute eczema, less so in subacute or chronic
eczema.
Childhood phase
May evolve from the infantile phase or start de
novo. Intensely pruritic papules. Sometimes
ATOPIC DERMATITIS (AD) lichenified and excoriated lesions. Symmetrically
An itchy chronic remitting and relapsing in the flexures of the elbows and knees. Also the
inflammatory skin condition associated with other wrists, ankles and sides of neck.
atopic disorders (asthma, hay fever) in patient or
Adult phase
the family.
Less common. May evolve per se. Or from AD of
Etiology infancy or childhood. Lichenified plaques, often in
the flexures. Or as nummular dermatitis. Extensor
Complex interplay between genetic factors (resulting aspects of the extremities occasionally involved.
in defective skin barrier), defective innate immunity Itching a dominant complaint, thus patients more
prone to develop lichen simplex chronicus.
Atopic dermatitis, childhood phase: bilaterally symmetrical Atopic dermatitis, adult phase: lichenified lesions in the cubital
excoriated papules and lichenified lesions in the flexures. fossae.
Nummular eczema: discoid (coin-like) exudative lesions with Dry discoid eczema: discoid plaques surmounted with scales.
mild crusting.
Dermatitis and Eczema 103
Seborrheic dermatitis, infantile: cradle cap. Seborrheic dermatitis: scaling nasolabrial fold.
Dermatitis and Eczema 105
Variants
SEBORRHEIC DERMATITIS
Dry discoid dermatitis: A non-exudative variant
of nummular eczema. Round or oval, dry, scaly Etiology
plaques on extensors of extremities.
Cause uncertain-possibly related to excessive
growth of the commensal yeast, pityrosporum.
Course
Role of genetic factors debatable. Any relation to
Relapsing and remitting; relapses often abrupt in the quantity or composition of sebum uncertain.
onset and frequent in winter. Individuals have a seborrheic diathesis. Dermatitis
severe in patients with AIDS.
Treatment
General Measures Epidemiology
Not uncommon. Two age peaks, in the first three
Eliminate/treat predisposing factors. Moisturize skin.
months of life infantile seborrheic dermatitis.
And second after fourth decade.
Specific Treatment
Topical steroid (often combined with antibiotic Clinical Manifestations
cream). Antihistamines. Extensive lesions may Morphology
warrant systemic steroids/immunosuppressives.
Characteristic seborrheic look—oily skin with
patulous, prominent follicular orifices. Scalp diffusely
POMPHOLYX involved: ill-defined areas with greasy yellow scales
Etiology on a dull red background (seborrhea capitis).
Intertriginous areas: erythematous scaly lesions or
Multifactorial—atopic or other endogenous
exudative crusted and fissured lesions. Hairy areas—
eczema. Or a dermatophytide (hematogenous
beard, trunk and pubic region show dull red, scaly
dissemination of dermatophyte toxins from a focus
plaques studded with follicular papules. Blepharitis
of ringworm-generally tinea pedis).
and squamous otitis externa common association.
Erythrodermic variant described as rarity.
Clinical Manifestations
More common in summers. Hyperhidrosis a Sites of Predilection
common, association. Young adults of both sexes Characterized chiefly by its distribution. Scalp,
affected. Itchy or painful deep seated vesicular retroauricular folds, eyebrows, nasolabial folds,
(sago-grain like) lesions on the palms, sides of the beard area, interscapular and presternal regions,
fingers and soles. Occasionally bullous or pustular axillae, pubic region, groins, umbilicus and folds
lesions. Protracted relapsing course, each episode under pendulous breasts. Blepharitis: fine scaling
resolving in 2–3 weeks. of eyelid margin.
Seborrheic dermatitis: scaling and papulation on the face; Stasis eczema: diffuse hyperpigmentation with depressed depig-
nasolabial folds, eyebrows, upper lip and chin. mented scar (atrophie blanche).
Stasis ulcer: ulcerative lesions surrounded by hyperpigmentation Irritant contact dermatitis: erythematous papulovesicles on the
and erythema; tortuous dilated veins are seen. thighs due to application of cetrimide 20%.
Dermatitis and Eczema 107
Systemic treatment
For severe/extensive disease. Fluconazole, 150
mg weekly effective. Oral ketoconazole though
effective best avoided because of its hepatotoxicity.
For severe disease, narrow band UVB and PUVA
therapy can be used.
STASIS ECZEMA
Epidemiology
Not uncommon. Middle aged individuals with
compromised venous return. Related to long hours of
continued standing.
Clinical Features
Slate-grey pigmentation. Later edema, acutely
exudative and crusted lesions. Or scaly and
lichenified areas; associated with obvious varicose
veins or other evidence of venous stasis. Occasional
ulceration that may heal with depigmented,
Sites of predilection: Seborrheic dermatitis. atrophic scar (atrophie blanche). Medial aspects of
ankles and lower legs involved. Autosensitization
Course with generalized eczema not infrequent.
Generally chronic with remissions and exacerbations.
Treatment
Patients predisposed to pyococcal infections.
General Measures
Treatment Relieve stasis. Keep feet elevated (at work also).
Use pressure bandages/stockings.
General Measures
Mild scaling of the scalp almost physiological. Specific Therapy
Ordinary shampooing alone adequate in mild Non-sensitizing, protective and bland local
cases. Application of hair oil best avoided. applications such as zinc oxide paste may help;
compresses and non-fluorinated weak or moderate
Specific Therapy strength topical steroids in acute stages. Surgical
or other intervention for correction of varicosities.
Topical treatment
More severe scalp lesions treated with special IRRITANT CONTACT DERMATITIS
medicated shampoos containing ketoconazole,
fluconazole, selenium sulphide or zinc pyrithione, Etiology
tar, salicylic acid. Shampoo best ‘left on’ for a few Non-immunologic dermatitis secondary to
minutes before rinsing off. Repeated twice weekly contact with an irritant substance in ‘adequate’
or more frequently. Acute lesions at other sites concentrations for a ‘sufficient’ length of time.
treated with combination of topical steroids and Individuals with dry skin more prone. Single contact
antibiotics. Subacute stage, salicylic acid steroid (usually accidental) with a strong irritant such as an
combination helpful. Topical ketoconazole, 2% or acid, alkali, strong antiseptic. Or repeated contact
butenafine, 1% cream give additional benefit. (inadvertent, deliberate or occupational) with mild
irritants such as detergents, cutting oils.
Allergic contact dermatitis: nickel in bra clips was causal. Allergic contact dermatitis, footwear: the skin in contact affected.
Allergic contact dermatitis: caused by a topical application fol- Airborne contact dermatitis: eyelids are conspicuously involved.
lowing a minor injury; mild dissemination to the other side was
due to accidental contact.
Dermatitis and Eczema 109
Clinical Manifestations
Common. Acute irritant dermatitis sharply limited to
the area of contact. Similar in morphology to other
acute eczemas—papules, vesicles or pustules, on
an erythematous, edematous background. Heals
with hyper/hypopigmentation. Strong irritants may
cause necrosis or ulcers that heal with scars.
Cumulative insult dermatitis due to repeated contact
with weak irritants. Classic example housewives’
dermatitis. Slow onset. Dryness and fissuring of
the skin, followed by lichenification. Relapses on
brief contact with mild irritants. Hands frequently
affected.
Parthenium hysterophorus: also called ‘congress grass’ is a wild
compositae.
ALLERGIC CONTACT DERMATITIS
A common dermatological problem, more frequent
in the industrialized world.
Clinical Manifestations
Etiology Morphology
Type IV, delayed type hypersensitivity (DTH) Clinical features simulate those of acute, subacute
response. Simple chemicals (haptens) become or chronic dermatitis.
complete antigens on combining with a carrier,
generally epidermal protein. Varying susceptibility Sites of Predilection
of individuals. Racial and genetic factors, perhaps
important. Sensitizing potential of substances Clue to suspecting the causal agent in allergic
variable. Nature of common contactants variable contact dermatitis. Airborne contactants: exposed
in different parts of the world. More frequent ones sites face, particularly the eyelids, neck, hands
include nickel, cobalt, chromium, epoxy resins, and forearms. Textile dermatitis: axillae, flexures of
antioxidants, textile dyes and finishes. Plants: such elbows, thighs or other areas in intimate contact
as Parthenium hysterophorus in India, poison ivy with clothing. Shoe dermatitis: areas in direct
in the USA, Primula obconica in Europe and other contact with the footwear-‘V’ shaped (with certain
members of the compositae family. Medicaments: sandals), dorsa of toes or feet, depending on the
such as nitrofurazone, neomycin, sulphonamides, shape of the shoe. Occupational dermatitis: hands
penicillin, antihistamines, local anesthetics, lanolin, and other exposed sites. Metal dermatitis: sites
ethylene diamine and a host of ‘home’ remedies. of contact with costume jewellery (ear lobules)
Cosmetics: fragrances, paraphenylenediamine watch straps on the wrist. Dermatitis due to local
containing hair dyes, preservatives and eosin. The medications at the site of application for an injury
exposure to contactants would vary depending or a surgical wound or on the hands in doctors,
on sex, age, occupation, hobbies and living nurses and other paramedics. Plant dermatitis:
conditions. Damaged skin, occlusion, hyperhidrosis sites of accidental or deliberate contact—hands,
predispose. Immunocompromised states and forearms. Or diffuse and generalized (airborne
debilitating conditions such as malignancies and contact dermatitis-ABCD). Hair dye dermatitis—at
lymphomas, interfere with the development of the hair line.
contact sensitivity.
Allergic contact dermatitis: to perfumes. Allergic contact dermatitis: to vegetables.
Photocontact allergic dermatitis: upper eyelids and upper lip Photocontact dermatitis: retroauricular fold is conspicuously
are spared. spared; helix of the ear is affected.
Dermatitis and Eczema 111
Investigations
Patch test diagnostic. Helps to identify trigger as
also helps in determining substitutes.
Napkin dermatitis: acutely inflamed diaper area; ‘convex’ sur- Lip-licking dermatitis: well-marginated dermatitis around the
faces affected, depth of fold spared. mouth.
Dermatitis and Eczema 113
Prurigo nodularis: keratotic nodules surmounted by crusts. Dermatitis artefacta: superficial ‘scalded’ lesions and evenly
spaced hypertrophic scars.
Dermatitis and Eczema 115
Cholinergic urticaria: small wheals which disappear in a few Pressure urticaria: wheal under tight undergarment.
minutes.
Abnormal Vascular Responses 121
Group of dermatoses in which blood vessel wall several times each day for days, weeks, or years.
damage is the common denominator. Damage may Variable sizes and shapes of wheals with or
be functional. Or structural. Injury may be physical. without an erythematous halo. Patients may have
Or toxin- or immune-mediated. The spectrum of systemic symptoms (abdominal pain and flushing),
clinical manifestations varies from evanescent if disease severe.
erythema to ischemic necrosis. Angioedema: regarded as a deeper variant
of urticaria; it involves the subcutaneous or
URTICARIA submucosal tissue (rather than only the dermis).
Urticaria and angioedema may coexist or occur
A common vascular reaction characterized
independently. Large, deep seated, painful
by production of wheals—itchy, evanescent,
swellings; each may last >72 hours. Eyelids,
erythematous or pale, edematous swellings of the
lips, tongue, genitals, larynx, common sites for
dermis. And subcutis (angioedema). Allergic or
involvement. Laryngeal angioedema, a rare but
non-allergic in nature.
serious complication.
Etiology
Classification
Etiology of urticaria
Spontaneous urticaria: which includes acute
Spontaneous
• Idiopathic (which lasts < 6 weeks) and chronic (lasts >
• Autoimmune 6 weeks) urticaria. And includes autoimmune
Ingestants urticaria in which circulating auto-antibodies
• Foods: like cheese, eggs, nuts, fish, mushrooms (detected by autologous serum skin test)
• Food additives: yellow dye, tartrazine present.
• Preservatives: salicylates, benzoates Physical urticaria: which are triggered by
Inhalants: pollen, animal dander physical stimuli. And are of different types.
Drugs: penicillins, salicylates, sulphonamides, sera Dermographism: an exaggeration of physio-
Infections: in teeth, tonsils, sinuses, gastrointestinal logical triple response, so manifests as linear
Infestations: gut parasites
wheals on acute brisk stroking.
Pressure urticaria: results from constant pres-
Contactants: foods, additives
sure for long periods, e.g. under belts, straps
or on the feet. May be immediate or delayed.
Pathogenesis Cholinergic urticaria: small (1–2 mm) sized,
Histamine and other mediators released from mast evanescent lesions that last a few minutes
cells cause vasodilatation and escape of fluid in to an hour or so. Precipitated by sweating,
the dermis (and subcutis in angioedema). Either: stress, heat and physical exertion.
Allergic: mostly type I, IgE mediated to variety Cold urticaria: lesions appear on exposure to
of triggers. Less frequently type Ill, IgG mediated cold wind or water; limited lesions or exten-
reaction. sive. May cause histamine shock (and drown-
Non-allergic: varied etiology and pathogenesis. ing while swimming in cold water).
Solar urticaria: young adults commonly
Angioedema: of penile skin leading to temporary paraphimosis. Erythema multiforme: target lesions with cyanotic center and
erythematous halo.
Abnormal Vascular Responses 123
ERYTHEMA MULTIFORME
An acute reaction pattern affecting the skin and
mucous membranes.
Epidemiology
Young adults of both sexes affected.
Dermograder: an instrument to grade urticaria.
Etiology
Triggers
Treatment
Triggers for erythema multiforme
General Measures
Infections: frequent cause
Determining the cause (and then eliminating it) of • Viral: Herpes simplex virus (HSV, 1>2), varicella zoster
chronic urticaria difficult. Clinical history may give virus, infectious mononucleosis, hepatitis B and C virus
a ‘lead’; ideally followed by careful ‘elimination’ • Mycoplasma: M. pneumoniae
and ‘re-exposure’ (challenge) to suspected Drugs: infrequent cause
causal agents. Diagnostic clues to etiology stem Collagen vascular disease
from pattern of occurrence, e.g. season, time of Malignancies
the day, relationship to food, sweating, physical Idiopathic
activity, exposure to pressure, light, heat, cold.
Food items containing dyes, preservatives and Pathogenesis
yeast and all drugs best avoided. Infective foci
ought to be eliminated. In HSV induced EM, fragmented DNA of virus
demonstrated in keratinocytes. This probably
Specific Treatment induces immune mediated damage to blood
vessels.
H1-receptor antihistamines mainstay of therapy.
Antihistamines with low sedating and less Clinical Manifestations
anticholinergic effects which are long acting
preferred. Average daily doses: chlorpheniramine Morphology
maleate, 2–8 mg; promethazine, 25–100 mg; Cutaneous lesion may be macular, papular, vesi-
cyproheptadine, 4–12 mg; fexofenedine, 120–180 cular or bullous. The lesions may be monomorphous.
mg; cetrizine, 10–20 mg and levocetrizine, 5–10 Or polymorphous (multiforme), perhaps through
Erythema multiforme: typical target lesion with central bulla, Erythema multiforme: iris (target) lesions on the trunk.
dusky halo and marginal vesicles (herpetic iris of Bateman).
Erythema multiforme: mild oral lesions with hemorrhagic crusts Toxic epidermal necrolysis: large areas of irregular dusky ery-
on lips. thema developing flaccid bullae.
Abnormal Vascular Responses 125
Clinical Manifestations
Presence of mucocutaneous lesions typical.
Skin lesions: Rapidly progressive large areas of
irregular shaped dusky erythema, sometimes with
atypical targetoid centers (scalded skin). Develop
large, flaccid, clear or hemorrhagic bullae. Rupture
to eventuate into large areas of denudation.
Symmetrical distribution on face, upper trunk
and proximal extremities. Severe constitutional
Histopathology of erythema multiforme: massive edema of symptoms. Serious, life threatening or fatal
upper dermis and perivascular lymphocytic infiltrate. condition. Prognosticated using SCORTEN.
Toxic epidermal necrolysis: dark scalded-looking skin. Necrotic Erythema annulare centrifugum: well-defined erythematous
skin peels off in sheets leaving areas of denuded skin. Hemorrhagic scaly annular lesions.
crusts of lips.
Erythema annulare centrifugum: erythematous plaque with a Erythema chronicum migrans: annular plaque with central tick
trailing edge of scales. bite.
Abnormal Vascular Responses 127
Clinical Manifestations
Reactive erythemas of varied configuration:
annular, arcuate, serpiginous or irregular. Migratory
character. Centrifugal spread with central clearing.
Variable rate of progression (days-weeks). Each
lesion lasts a few days; episodes recur for weeks,
months, or years.
Drug eruption: bilateral erythematous papules, due to Acute generalized exanthematous pustulosis: small pustules
carbamazepine. on an erythematous background. Often starts on face or in flex-
ural areas, rapidly becoming generalized.
Abnormal Vascular Responses 129
Cutaneous vasculitis: superficial ulcers and large hemorrhagic Henoch-Schönlein purpura: bilaterally symmetrical palpable
bullae. purpuric lesions.
Abnormal Vascular Responses 131
threatening (SJS/TEN, EM major, anaphylaxis). May attention and sometimes large doses of systemic
mimic any morphologic pattern—exanthematous, corticosteroids. In FDE, if necessary, challenge
erythodermic, epidermal necrolysis, acute under supervision may be justified.
generalized exanthematous pustulosis, fixed drug
eruption, photosensitive. Different morphologic
CUTANEOUS VASCULITIS
patterns in different patients with the same drug.
Course variable; often prompt remission upon A reaction pattern with a spectrum of manifestations
withdrawal and relapse on readministration. varying from erythema to frank necrosis of the skin.
Common denominator, a histological vasculitis.
Exanthematous Eruptions Acute or recurrent and chronic. Progression and
Commonest drug eruption. Itchy symmetrical prognosis depend on the primary cause and
papulosquamous lesions on the trunk which associated organ involvement.
spread centrifugally. Rash begins within 1–2
weeks of starting the therapy and subsides (with Etiology
desquamation) 1–2 weeks after stopping. Drug Reaction pattern to variety of triggers.
rash with eosinophilia and systemic symptoms Triggers causing vasculitis
syndrome (DRESS), a severe variant.
• Infections
– Bacterial: Streptococcal
Acute Generalized Exanthematous Pustulosis – Viral: hepatitis B, C
Small pustules develop rapidly on an erythematous – Mycobacterial
• Connective tissue disorders: systemic lupus erythema-
background. Often starts on face or in flexural
tosus, rheumatoid arthritis, antiphospholipid antibody
areas, rapidly becoming generalized. May be syndromes
associated with fever and malaise. • Malignancies
• Drugs
Fixed Drug Eruptions (FDE)
Single, a few, rarely multiple lesions. Distinctive Pathogenesis
morphology: circular or oval, erythematous macules Usually immune complex mediated.
or plaques with a bluish, sometimes vesiculated
middle and an erythematous halo. Mucocutaneous Clinical Manifestations
junctions particularly affected. Site(s) once affected Vasculitis can manifest both in skin. And in internal
always gets reactivated on readministration of the organs. Cutaneous manifestations depend on size
drug; fresh lesions may appear at other sites. No of vessel involved (venular involvement as palpable
constitutional symptoms. Self limiting course; heal purpura, arterial as ulcers). And type of infiltrate
with characteristic bluish-black pigmentation. (neutrophils as papules, granulomatous as nodules).
Internal organ involvement depends on pattern of
Treatment involvement. Several patterns recognized.
General Measures
Henoch-Schönlein Purpura
Prompt withdrawal of the drug essential. Patients
with SJS-TEN need excellent nursing and A common small vessel (venular) vasculitis.
supportive care (vide supra). Obscure etiology: streptococcal infection or drugs
incriminated. Children of either sex affected.
Specific Treatment Onset often abrupt. Triad of palpable purpura,
arthritis and gastrointestinal involvement. Crops of
Mild rash: soothing topical applications like urticarial, edematous papular and purpuric lesions.
zinc calamine lotion, or cold cream adequate. On ankles, lower legs. Polyarthritis: knee, elbow,
Moderate or severe: systemic antihistamines ankle or small joints of the hands, painful, tender
and corticosteroids. SJS-TEN need urgent and swollen. Abdominal pain, vomiting, melena
Erythema elevatum diutinum: symmetric erythematous pap- Pigmented purpuric dermatosis: erythematous and brownish
ules on knees. macules on feet and legs.
Pyoderma gangrenosum: large ulcer with bluish edematous Pyoderma gangrenosum: multiple lesions on both lower limbs.
necrotic edge.
Abnormal Vascular Responses 133
Investigations
Establishing Diagnosis
Histopathology
Diagnosis of vasculitis confirmed by biopsy, taken Histopathology of vasculitis: polymorphonuclear leucocytes
and lymphocytes infiltrating and damaging blood vessel wall;
from newest lesion, preferably from a site other than top (10x), bottom (40x).
legs. Demonstrable structural damage to blood
vessel wall with cellular infiltrate which may be
Treatment
polymorphonuclear, lymphocytic or granulomatous.
Fibrinoid degeneration or a total destruction of the General Measures
blood vessel wall pathognomonic. Extravasation Remove/avoid triggers.
of red blood cells present. In PPD, perivascular
lymphocytic infiltrate in upper dermis with deposits Specific Treatment
of hemosiderin. Depends on severity of skin and extent of organ
involvement. Some like PPD need general
Establishing the Etiology
measures like foot end elevation. Systemic steroids
Tests based on clinical evaluation to rule out and other immunosuppressive standard therapy.
infection/connective tissue disorder/neoplasms.
Establishing Extent of Disease PYODERMA GANGRENOSUM
Tests based on clinical evaluation and the pattern of Etiology
involvement important to rule out renal, pulmonary, Idiopathic neutrophilic dermatoses. Association
neurological, abdominal involvement to strategize with ulcerative colitis, Crohn’s disease, hematologic
treatment. or other systemic diseases.
Behçet’s syndrome: aphthous-like ulcer on the inside of the lip. Behçet’s syndrome: large punched-out ulcer on vulva; note scars
of previous ulcers.
Psoriasis: bizarre geographical patterned lesions. Annular lesions Psoriasis: large plaques covering almost the entire trunk; thick
conspicuous. scales.
Papulosquamous Disorders 139
Heterogeneous group of dermatoses characterized and plaques of varying sizes and configurations
by erythematous papules or plaques surmounted (discoid, gyrate, annular). Sometimes lesions
with scales. Color of lesions varies from faint pink uniformly 1–3 cm only (so called small plaque
(pityriasis rosea) to dull red (psoriasis) to purple psoriasis). Lesions covered with varying amount
or violaceous (lichen planus). Scales vary from of loosely attached silvery white scales overlying an
minimal (lichen planus) to abundant (psoriasis), adherent translucent membranous scale. Removal
easily removable (chronic plaque psoriasis) to of the latter reveals punctate bleeding spots (from
adherent (rupioid psoriasis). the elongated capillary loops in dermal papillae)
—the characteristic Auspitz sign. Sometimes
PSORIASIS lesions hyperkeratotic—rupioid (cone shaped),
ostraceous (oyster shaped) or elephantine (grossly
Epidemiology hyperkeratotic). Ring of Woronoff, occasionally
seen as a hypopigmented halo around the plaques.
Extremely common dermatosis with worldwide
Lesions often elicited at sites of trauma (Koebner’s
distribution. Plaque psoriasis has bimodal age
isomorphic phenomenon).
distribution, early (2nd decade) onset, with more
severe course and more frequent arthropathy. And Sites of Predilection
late (5th decade) onset with milder course.
Distributed generally on extensors of the body—
knees, elbows, lower lumbosacral region. As also
Etiology umbilicus and intergluteal cleft. Palms and soles,
Genetic predisposition: autosomal dominant scalp (frequently) and genitals also involved.
inheritance with incomplete penetrance. Multi- Facial involvement a late and infrequent feature.
factorial. Strong association with HLAB13, Less commonly a flexural distribution (inverse
HLA-Bw17 and HLA-Cw6. Environmental factors psoriasis). And rarely a photodistribution.
contribute: trauma, infections, emotional stress,
climatic changes may precipitate relapses. Sunlight
usually protects. Patients with AIDS present with
severe psoriasis.
Pathogenesis
Basic pathogenetic mechanism ill-understood. Earlier
thought to be a disorder of aberrant keratinization with
secondary inflammation. Now recognized as primarily
an inflammatory disorder induced and sustained by
T-cell mediated immune response (complex cascade
of humoral and cellular immunomechanisms) which
leads to accelerated growth of epidermal and vascular
cells. Chief cutaneous manifestations result from
hyperplasia of the epidermis—epidermopoiesis more
rapid. And transit time of epidermal cells diminished
with immature nucleated epidermal cells present in
the stratum corneum (parakeratosis).
Clinical Manifestations
Morphology
Classical psoriasis characterized by asymptomatic
(or itchy), well-defined erythematous, scaly papules Sites of predilection: Psoriasis.
Psoriasis: auspitz sign showing bleeding points. Palmar psoriasis: well-defined scaly, erythematous plaques on
the palms.
Plantar psoriasis: scaly erythematous plaques on the soles. Psoriasis of scalp: rather diffuse, but well marginated involve-
ment, spilling beyond scalp margin.
Papulosquamous Disorders 141
Guttate psoriasis: scaly, erythematous small papules. Psoriatic erythroderma: generalized red and scaly skin surface.
Papulosquamous Disorders 143
Course
Course unpredictable and variable–spontaneous
remissions and relapses a characteristic feature.
Most patients worse in winter, some in summer.
Patients with ill-defined erythematous and warm
lesions indicate an unstable form; could progress
on to erythroderma.
Investigations
None generally required. In doubtful cases biopsy
required.
Histopathology
Biopsy characterized by parakeratosis and
orthokeratosis, loss of granular cell layer, uniform, club
shaped elongation of rete ridges and suprapapillary
thinning of epidermis. Neutrophilic collections in
parakeratotic stratum corneum form subcorneal
pustule (Munro’s micro-abscess) in psoriasis and a
spongiform pustule (spongiform pustule of Kogoj)
in stratum spinosum in pustular psoriasis. Papillary
Histopathology of psoriasis: parakeratotic hyperkeratosis, pro-
blood vessels dilated, elongated and tortuous. nounced and uniform acanthosis and suprapapillary thinning of
Perivascular mononuclear infiltrate in dermis. the epidermis; top (x10), bottom (x40).
Pustular psoriasis, localized: superficial discrete pustules on the Pustular psoriasis, generalized: superficial discrete and conflu-
palms. ent pustules on a generalized ‘fiery’ background.
Pustular psoriasis: nail and paronychial involvement. Impetigo herpetiformis: superficial pustules at the periphery of
an erythematous lesion. Pustular psoriasis of pregnancy.
Papulosquamous Disorders 145
Treatment
Aimed at bringing about remission. And maintai-
ning it, but relapses occur despite therapy. Various
topical, systemic or photo-therapeutic modalities
available. Rationale mostly ill understood. Phototherapy/photochemotherapy units: consists of vertically
mounted UV tubes which emit either UVA or UVB (311 nm).
General Measures
to exceed 100 gm/week of ointment, as increases
Counselling patient regarding chronicity, relapses calcium absorption and cause hypercalcemia or
paramount. Avoidance of smoking, alcohol intake. hypercalcuria. May also cause local irritation.
Specific Treatment Phototherapy and photochemotherapy
Topical therapy, the preferred modality of Phototherapy involves exposure on alternate
treatment. Used exclusively for localized disease. days to incremental doses of ultraviolet B (narrow
And as adjuvant to systemic therapy which is band, 311 nm, given using UVB chambers) after
necessary mostly for extensive disease (>10% application of emollient (to reduce scattering
body surface area), pustular, erythrodermic and of light). Clearance generally in 6–8 weeks.
arthritic psoriasis. Also for debilitating localized Photochemotherapy involves intake on alternate
disease (palmoplantar psoriasis). days of oral 8-methoxypsoralen, 0.6 mg/kg, 2–3
hours before exposure to gradually increasing
Topical therapy doses of sunlight (PUVA sol) or UVA delivered
Several agents available. Crude coal tar, 3–10% using UVA chambers (PUVA). Initial dose of light
or solution of liquor carbonis detergens applied and increments depend on skin type. Response
once or twice a day. Exposure to sunlight or UVB in psoriasis better with photochemotherapy
of additional value. Combining with salicylic acid than phototherapy (probably related to greater
(2–5%) helps in removing scales especially in thick penetration of UVA).
lesions. As also on palms and soles. Anthralin
used as conventional therapy (0.05–0.1% Systemic therapy
applied overnight) or as short contact therapy Methotrexate, perhaps the most frequently used
(2% applied for ½–2 hours); removed with any medication. Used for extensive plaque psoriasis.
oil. Calcipotriol, a synthetic analogue of vitamin And pustular, erythrodermic and arthritic psoriasis.
D, used as 0.005% ointment. Total application not Also for debilitating palmoplantar psoriasis. Dose
Psoriasis nails: conspicuous pitting of the nail plates with mild- Psoriasis nails: uniformly dystrophic nails with proximal nail fold
subungual hyperkeratosis. involvement.
Psoriatic arthropathy: distal interphalangeal joints involved. Rupioid psoriasis: heaped up scales on large papules.
Note nail changes, a common association.
Papulosquamous Disorders 147
Reiter’s disease: multiple discrete to coalescing heaped-up kera- Lichen planus: pink and purplish papules. Note Koebner’s
totic papules. phenomenon.
Papulosquamous Disorders 149
Lichen planopilaris, scalp: slate-grey follicular papules with cica- Lichen planus of palms: yellow brown keratotic papules and
tricial alopecia. plaques, many showing pitting.
Papulosquamous Disorders 151
Course
LP runs a self-limiting course lasting several
months. Relapses uncommon. Hypertrophic
lesions last for several years. Occasional malignant
change in buccal or hypertrophic lesions.
Investigations
None required. Biopsy done occasionally in
doubtful cases.
Biopsy
Compact hyperkeratosis, irregular hypergranulosis
and acanthosis (pronounced in hypertrophic variety) Histopathology of lichen planus: obliterated dermoepidermal
with saw-tooth appearance of rete ridges (c.f. club interface with vacuolar degeneration of basal cells and a dense
shaped in psoriasis). Liquefactive degeneration of band of Iymphohistiocytic infiltrate in upper dermis.
basal cell layer (with formation of Max Joseph’s
space) and band of lymphocytes and histiocytes
Treatment
apposing the epidermis pathognomonic. Cytoid
or colloid bodies at the dermoepidermal interface. General Measures
Melanin incontinence in upper dermis. Perifollicular Remove suspected precipitating agent such as
infiltrate in lichen planopilaris. Subepidermal split drugs, hair dyes. Protect from sunlight in actinic
in bullous LP. form. Antihistamines as antipruritics.
Lichen planus actinicus: small and large violaceous papules Mucosal lichen planus: of the palate and lips.
assuming annular configuration on the side of the neck.
Mucosal lichen planus: whitish plaque on the tongue. Genital lichen planus: violaceous papules and plaques over the
glans penis and coronal sulcus.
Papulosquamous Disorders 153
Investigations
None usually required, though biopsy ofen
performed.
Biopsy
Histopathology variable. Active lesions show
features of LP. Late lesions, focal incontinence of Lichen nitidus: focal collection of lymphocytes and histiocytes
pigment with mild to moderate lymphohistiocytic with atrophy of overlying epidermis; note elongated rete ridges
infiltrate. And melanophages. at the edges giving ‘ball in claw’ appearance.
Lichen planus, nails: note dystrophy of several nails with pteryg- Lichen planus pigmentosus: diffuse bluish-grey pigmentation
ium unguis. Nail plates thin. in a photosensitive distribution.
Lichen nitidus: small grouped shiny papules on shaft of penis. Keratosis lichenoides chronica: violaceous hyperkeratotic pap-
ules in linear configuration.
Papulosquamous Disorders 155
Treatment
Self resolving condition. Topical steroids. And
photoprotection for actinic variant.
PITYRIASIS ROSEA
Epidemiology
Common.
Etiology
Unknown, possibly a viral etiology (HHV-7).
Sites of predilection: Pityriasis rosea.
Clinical Manifestations
Morphology PITYRIASIS RUBRA PILARIS
Clinically distinctive papulosquamous rash with Epidemiology
primary lesion, the herald patch, a hypopigmented
or erythematous, oval plaque with a peripheral Rare.
collarette of fine scales. Similar but smaller Etiology
lesions appear over the next few days. Mildly
pruritic or asymptomatic. Minimal constitutional Etiology obscure. Genetic influence perhaps
symptoms. Lymphadenopathy frequent. Heal operative in some forms.
spontaneously in 6–10 weeks with or without some Clinical Manifestations
hypopigmentation. Second attack is unusual.
Several variants—juvenile (classical/atypical/
circumscribed) and adult (classical/atypical) onset.
Sites of Predilection
And HIV-associated. Onset abrupt or gradual.
Chiefly on trunk along lines of cleavage and As an erythroderma or a seborrheic picture
proximal portions of the upper and lower affecting scalp and face. Gradual development of
extremities. Occasionally distal parts of extremities asymptomatic erythematous, acuminate follicular
and face involved (inversus variety). papules on the dorsae of proximal phalanges,
knees, elbows and later elsewhere. May become
Treatment confluent to form salmon colored plaques with fine
Reassurance—about self-limiting nature. branny or frankly psoriasiform scales. Erythroderma
Bland applications such as calamine lotion shows characteristic skip areas—islands of
or emollients. Occasionally topical steroids. normal skin. Palmoplantar keratoderma is diffuse
Systemic antihistamines to relieve itching. and pale yellow in color, the so-called keratotic
Pityriasis rosea: multiple, red scaly plaques on the trunk. Note Pityriasis rosea: multiple erythematous plaques with collarette
herald patch in the suprapubic area. of scales on the trunk. Note herald patch in the right iliac fossa.
Pityriasis rubra pilaris: keratotic follicular papules. Pityriasis rubra pilaris: keratoderma with yellow to orange hue,
hyperkeratotic lesions on the knuckles with follicular papules on
the dorsa of proximal phalanges.
Papulosquamous Disorders 157
Biopsy
Histopathology shows alternating orthokeratosis
and parakeratosis in both vertical and horizontal
directions (checker board pattern). Keratotic plugs
in the hair follicles with focal parakeratosis in the
follicular shoulder with perifollicular lymphocytic
infiltrate in the dermis.
Treatment
General Measures
Emollients.
Specific Treatment
Combination of topical and systemic therapy.
Topical treatment
Sites of predilection: Pityriasis rubra pilaris.
Use moderately potent corticosteroids or tretinoin
or a combination of the two.
sandal (keratodermic/PRP sandals). Mucous
membrane involvement rare. Nail involvement Systemic treatment
not characteristic. Protracted course; occasional Oral retinoids (acitretin, 0.5–1 mg/kg/day) or
spontaneous resolution. Relapses unusual. methotrexate. Response less satisfactory than in
psoriasis.
Investigations
None usually required. Biopsy sometimes done
especially in erythroderma to confirm the diagnosis.
10 Autoimmune
Vesiculobullous
Dermatoses
Pemphigus vulgaris: early lesion small vesicle on normal skin. Pemphigus vulgaris: large flaccid turbid-fluid filled bulla.
Pemphigus vulgaris: superficial erosions and flaccid Pemphigus vulgaris: extensive lesions.
vesiculopustules.
Autoimmune Vesiculobullous Dermatoses 161
Bulla-spread sign: positive in pemphigus vulgaris. Note sharp Bulla-spread sign: sometimes positive in bullous pemphigoid,
edge of extended bulla and easily ruptured bulla. but note rounded edge.
Autoimmune Vesiculobullous Dermatoses 163
rare. Manifests as painful, superficial erosions; superficial vesiculation. Gradual spread, from
often (in 50%) precede appearance of cutaneous face, scalp, upper trunk (seborrhoeic distribution)
lesions. Cutaneous lesions: superficial and flaccid to becoming generalized. May simulate exfoliative
vesicles and bullae; rupture easily, leaving large dermatitis. Mucosal lesions infrequent.
denuded areas; extend peripherally. Occasional
bleeding. Clinically normal epidermis peeled off
by gentle tangential pressure (Nikolsky’s sign).
Healing slow, with hyperpigmentation. Pressure
areas and sites of friction more prone. Flexural
lesions prone to becoming hypertrophic and
vegetative. Paronychial lesions typical. Little
toxemia. Remissions mostly induced by systemic
corticosteroids or other immunosuppressives.
Relapses common. Mortality, though still moderate,
has come down considerably.
Pemphigus Vegetans
An uncommon variant of pemphigus vulgaris.
Hypertrophic vegetative lesions with superficial
fissuring with or without obvious vesiculation. In
axillae, groins, perineum, perianal areas and lips.
Mucosal lesions infrequent. Prognostically better
than pemphigus vulgaris.
Pemphigus Foliaceus
Less common, less serious than pemphigus
vulgaris. Elderly individuals or children. Scale-
crusts, sometimes with, but more often without, Sites of predilection: Pemphigus.
Pemphigus vulgaris: buccal mucosal lesions showing ragged Pemphigus vulgaris: genital lesions manifesting as erosion with
margin. irregular margins.
Autoimmune Vesiculobullous Dermatoses 165
Pemphigus Erythematosus
(Senear-Usher Syndrome)
Uncommon variant of pemphigus foliaceus;
Erythema and scale-crusts in malar area (butterfly
distribution). And scaly lesions also on the scalp
and elsewhere.
Pemphigus foliaceus: scale-crusts all over the face (similar Pemphigus foliaceus: scale-crusts over trunk. Removal of scale-
lesions also present on the body). crust reveals minimally moist skin or an erosion.
Autoimmune Vesiculobullous Dermatoses 167
IgA pemphigus: intraepidermal neutrophic variant. Flaccid blisters IgA pemphigus: IEN variant flaccid blisters in an annular
in annular configuration. Note central erythema and peripheral configuration.
vesiculation and erosions.
Autoimmune Vesiculobullous Dermatoses 169
Systemic therapy
Aim is to bring about remission rapidly and maintain
it. Systemic corticosteroids, mainstay of treatment.
Large doses (60–80 mg/day of prednisolone
equivalent) given daily orally. Or as oral
(betamethasone) or parenteral (dexamethasone),
100 mg pulse, given monthly or fortnightly.
Pulse therapy perhaps brings about remission
more rapidly. Use of adjuvants (azathioprine,
50–100 mg/ day, cyclophosphamide, 50–100 mg/
day and methotrexate, 7.5–15 mg/week) steroid
sparing; also help maintain remission. Daily oral
steroids, though required to maintain remissions
in some patients. Biologicals, in form of rituximab,
Direct immunofluorescence of pemphigus: fish-net like deposit
of IgG in epidermis. the new drug.
Pemphigoid: early bullae on urticarial base. Bullous pemphigoid: large tense bullae on urticarial base.
Autoimmune Vesiculobullous Dermatoses 171
Sites of Predilection
Lower limbs, abdomen, trunk.
Immunofluorescence
Direct immunofluorescence
Linear deposit of IgG (less frequently IgE) and
complement C3 at BMZ.
Course
Little toxemia. Spontaneous or (more often)
therapeutic remission. Relapses less frequent than
in pemphigus. Prognosis generally good, unless
associated with malignancy.
Direct immunofluorescence of bullous pemphigoid: linear
Associations deposit of IgG and C3 along the basement membrane.
Occasional association with systemic malignancies,
particularly lymphomas (? just age related).
Indirect immunofluorescence
Investigations About 70% (higher, if salt split skin used as substrate)
of patients have circulating IgG (more frequently)
Histopathology and IgE (less frequent, but associated with more
Subepidermal bulla with eosinophils. severe disease) autoantibodies that bind to BMZ
Pemphigoid: large, tense, clear and hemorrhagic blisters on Pemphigoid: bullae heal with hyperpigmentation and milia
the forearms. Bullae do not rupture, but roof settles as fluid is formation.
reabsorbed.
Herpes gestationis: erythematous plaques with clear vesicles at Herpes gestationis: clear vesicles on trunk. Many blisters arranged
the edges. Some lesions targetoid. in arcuate/annular pattern. Patient prematurely delivered a small
for date baby.
Autoimmune Vesiculobullous Dermatoses 173
Benign mucosal pemphigoid: palatal lesions. Begin as transient Benign mucosal pemphigoid: skin lesions, often tend to occurs
vesicles which rupture to form chronic erosions. at the same site on scalp leading to cicatricial alopecia.
Autoimmune Vesiculobullous Dermatoses 175
Sites of Predilection
Face, trunk, perigenital area and extremities.
Indirect immunofluorescence
Low levels of circulating usually IgG (less frequently
IgA).
Treatment
Systemic corticosteroids with an adjuvant usually
azathioprine. Aggressive treatment initiated early
to prevent scarring. Occasionally topical or intra-
Sites of predilection: Chronic bullous dermatosis of childhood
lesional corticosteroid therapy. (CBDC).
Chronic bullous dermatosis of childhood: typical string of pearl Chronic bullous dermatosis of childhood: tense bullae in per-
appearance of tense bullae. igenital region.
Chronic bullous dermatosis of childhood: perigenital lesions Linear IgA bullous dermatosis: itchy grouped small papulovesi-
very characteristics. cles on urticarial background. Most vesicles have been excoriated.
Autoimmune Vesiculobullous Dermatoses 177
Epidemiology
Uncommon. Young adult males.
Clinical Manifestations
Morphology
Intensely pruritic, bilaterally symmetrical, grouped,
small vesicles or papules on an urticarial
background. May form annular plaques with
vesicular edge. Heal with hyperpigmentation.
Grouped excoriations sometimes the only
manifestation. Mucosal lesions not seen.
Sites of Predilection
Direct immunofluorescence of CBDC/LABD: linear deposit of Shoulders, elbows, back, buttocks, trunk and
IgA along the basement membrane in perilesional skin. extensor aspects of extremities.
Linear IgA bullous dermatosis: drug induced LABD mimicking Dermatitis herpetiformis: grouped excoriations and intervening
toxic epidermal necrolysis. vesicles over both elbows.
Dermatitis herpetiformis: grouped vesiculation on an erythe- Dermatitis herpetiformis: grouped excoriation often the only
matous background. Many vesicles have been excoriated. manifestations.
Autoimmune Vesiculobullous Dermatoses 179
Course
Relapsing and remitting course.
Histopathology of dermatitis herpetiformis: subepidermal
split with papillary apical abscesses containing large number of
Association neutrophils. Top (10x). Bottom (40x).
Associated GSE invariable; mostly asymptomatic.
Eruptive xanthomas: multiple reddish yellow papules on but- Eruptive xanthomas: multiple yellow-red papules on trunk.
tocks. Also present on elbows. Associated with type I, IV, V hyper-
lipoproteinemia. Eruptive xanthomas usually subside without
residual skin change.
Metabolic and Nutritional Dermatoses 183
Disorders that manifest with cutaneous lesions as Xanthoma striate palmaris: plane xanthomas on
direct or indirect, effect of metabolic abnormalities. palmar creases, almost pathognomonic of type
Or nutritional imbalances. Environmental factors, III hyperlipoproteinemia. Associated tuberous
such as diet and light, may play an important role. xanthomas.
Tendinous xanthoma: deep-seated, pale yellow, flat nodules Tendinous xanthomas: yellowish linear, flat nodules, overlying
overlying the tendons. Associated with type II hyperlipoproteine- tendons of the foot.
mia (familial hypercholesterolemia). Presence of interdigital web
space plane xanthomas pathognomonic of homozygous state.
Metabolic and Nutritional Dermatoses 185
Other Investigations
Rule out coronary and peripheral arterial disease
in patients with tendinous, tuberous/palmar and
web space xanthomas.
Treatment
General Measures
Depends upon metabolic abnormality. Reduce
total calorie intake. Low cholesterol and/or
low triglyceride and/or low carbohydrate diet.
Substitute polyunsaturated for saturated fatty
acids. Treat primary disease: diabetes mellitus,
hypothyroidism and others.
Specific Measures
Serum in type I hyperlipoproteinemia: creamy top layer.
HMG-CoA reductase inhibitors (statins) and
Histopathology clofibrate 1.5–2 g/day helpful in some cases.
Xanthelasma responds well to topical application of
Biopsy diagnostic. Foamy, lipid-laden histiocytes
trichloroacetic acid (10–25%), electrodessication,
(xanthoma cells). Large giant cells (Touton giant
laser therapy (Er:YAG laser) and excision.
cells) with multiple peripheral and central nuclei
However, recurrences common.
present. Admixture of inflammatory cells seen.
AMYLOIDOSIS
Dermatoses characterized by deposition of
proteinaceous material, amyloid, in dermis.
Distinctive tinctorial properties (stains with Congo
red and metachromatically with crystal violet). Could
be purely cutaneous. Or cutaneous manifestations
of a systemic form.
Clinical Features
Macular and papular (lichen) forms; occasionally
mixed.
Macular amyloidosis
Uncommon. Young or middle aged adults.
Histopathology of xanthoma: foamy, lipid-laden histiocytes
(xanthoma cells). Large giant cells (Touton giant cells) with mul- Asymptomatic or mildly itchy greyish-brown
tiple peripheral and central nuclei present. Admixture of inflam- pigmentation in rippled pattern. Upper back and
matory cells seen; top (x10), bottom (x40). interscapular areas, forearms and arms affected.
Macular amyloidosis: slate-grey pigmentation on the upper Lichen amyloidosis: dark, hyperkeratotic papules on the shins.
back; ripple pattern is characteristic.
Primary systemic amyloidosis: purpuric lesions on the eyelids Primary systemic amyloidosis: pinch purpura, which develops
(racoon eyes). Note infiltrated waxy plaques. after slight trauma.
Primary systemic amyloidosis: macroglossia with indentation Primary systemic amyloidosis: macroglossia with infiltrated
of teeth. papules and nodules, some hemorrhagic.
Metabolic and Nutritional Dermatoses 187
Investigations
Investigate to confirm diagnosis. And to rule out
underlying plasma cell dyscrasia. And to find extent
of involvement.
Histopathology
Biopsy a suggestive skin lesion. If absent, consider
abdominal fat aspiration, rectal or gingival biopsy.
In skin biopsy, amyloid deposits seen in dermis and
subcutis. Also often found around sweat glands
and within blood vessel walls.
Rule out underlying plasma cell dyscrasia
By urine and serum protein electrophoresis and
Histopathology of lichen amyloidosis: homogeneous, amor-
phous eosinophilic material in papillary dermis; H & E (top) and immunofixation electrophoresis. And bone marrow
Congo-red (bottom). aspirate and biopsy.
Primary systemic amyloidosis: purpuric lesions on the palms. Follicular mucinosis: ill-defined erythematous, alopecic plaque.
Acanthosis nigricans, obesity related: brown-black pigmenta- Acanthosis nigricans, malignant: velvety thickening at unusual
tion and papillomatous velvety thickening of axillary skin in an sites. Manifesting here as tripe palms. This patient also had hyper-
obese individual. Note skin tag. pigmented velvety plaques in the perioral region.
Metabolic and Nutritional Dermatoses 189
Treatment
No satisfactory treatment available;
chemotherapeutic regimens containing melphalan,
vincristine, doxorubicin and prednisolone may
prolong survival, in some cases. And autologous
peripheral blood stem cell transplantation.
Congenital erythropoietic porphyria: reddish brown teeth Congenital erythropoietic porphyria: mutilated sclerodermoid
(erythrodontia); milia around alae nasi. hands.
Metabolic and Nutritional Dermatoses 191
Investigations
Fluorescence of Urine, Stool and Red Blood Cells
Urine and stool fluorescence under Wood’s light, a
crude method of evaluating presence of porphyrins
in the specimen. Similarly look for fluorescence of
RBCs.
Lipoid proteinosis: characteristic beaded papules on eyelid mar- Lipoid proteinosis: waxy papules on dorsae of fingers. Also
gins (monilial blepharosis). occur on elbows, forehead.
Metabolic and Nutritional Dermatoses 195
Miscellaneous Tests
Liver function tests
Deranged in PCT
Treatment
General Measures
Photoprotection of paramount importance. Use
broad-spectrum sunscreens containing titanium
dioxide and zinc oxide. And lifestyle modification.
Specific Treatment
CEP
Unsatisfactory. Hypertransfusions (suppress
hematopoiesis) may help. Occasionally
splenectomy. Bone marrow transplant results in
marked improvement.
EPP
Protect from light. ß-carotene (free radical
quencher) 60–180 mg/day, often helpful.
PCT
Avoid alcohol or other triggers. Small doses
(125 mg, twice a week) of chloroquine (increases
excretion of porphyrins). Or phlebotomy (500 mL,
once a fortnight, reduces iron stores) 4–6 times. Or
a combination of the two brings about a remission
Fluorescence of skull and skeleton: in CEP. which may last a year or more.
Lipoid proteinosis: verrucous plaques on elbows. Sarcoidosis, plaque variant: infiltrated papules coalescing to
form infiltrated plaque on back.
Sarcoidosis, papular variant: asymptomatic, juicy translucent Sarcoidosis, angiolupoid variant: soft, hemispherical well-
papules on neck. demarcated plaques with telangiectasia.
Granuloma annulare: erythematous, well marginated plaques Granuloma annulare: multiple lesions in a diabetic.
with beaded border and atrophic center.
Metabolic and Nutritional Dermatoses 199
Treatment
Intralesional steroids for limited skin disease.
Oral steroids for extensive cutaneous disease.
And for systemic disease. Chloroquine,
hydroxychloroquine, isotretinoin, methotrexate,
allopurinol, minocycline, and thalidomide useful
alternatives. But recurrences common.
Diabetes
Diabetes sometimes detected first by
dermatologists. Recurrent bacterial infections
Histopathology of sarcoidosis: granulomas, typically mono- (carbuncle pathognomonic), fungal infections
morphic and naked (devoid of cuff of lymphocytes). (tinea, candidal) typical.
Acanthosis Nigricans
Manifestation of insulin resistance. Brown to gray-
black velvety skin thickening along with skin tags.
Symmetrically in flexures including posterolateral
surface of neck, axillae and groins.
Diabetic Bullae
Chest X-ray, sarcoidosis: hilar lymphadenopathy. Cause, uncertain. Non-immunologic, non-
Courtesy: Dr Chandan Das, Department of Radiology, All India acantholytic. Asymptomatic, non-inflammatory,
Institute of Medical Sciences (AIIMS), New Delhi. spontaneously occurring, intra or subepidermal
bullae. Legs, arms, hands and feet affected.
Evaluation of Chest Last several weeks. Heal spontaneously without
scarring.
Radiological evaluation: Chest radiograph
abnormal in 90% of patients with sarcoidosis. Granuloma Annulare
Bilateral hilar adenopathy noted in 75%, while An uncommon, benign, morphologically distinctive
pulmonary parenchymal infiltrates seen in half. dermatosis. However, not necessarily associated
CT has a higher pick up. with diabetes.
Necrobiosis lipoidica: yellowish, atrophic plaque on the shin Hypothyroidism: dry, coarse skin; puffy eyelids and a lethargic
with telangiectatic blood vessels. look.
Addison’s disease: generalized pigmentation, pronounced in the Cushing’s syndrome: moon facies, hypertrichosis and redistribu-
palmar creases. tion of fat.
Metabolic and Nutritional Dermatoses 201
Treatment
Unsatisfactory. Try moderately potent corticos-
teroids on the active border. Or intralesional
steroids.
Hypothyroidism (Myxedema)
Histopathology of granuloma annulare: foci of degenerated
collagen with palisading granulomatous infiltrate at the periphery. Iodine deficiency or idiopathic. Characteristic facies:
pale, puffy face with coarse features, lethargic look.
Patient feels cold and sweats less. Coarse sparse
Treatment hair on the scalp; loss of lateral third of eyebrows.
May resolve spontaneously. Intralesional or
topical steroids and topical tacrolimus in localized Treatment
disease. PUVA therapy and systemic retinoids for
Thyroxine replacement therapy.
disseminated disease.
Addison’s Disease
Necrobiosis Lipoidica
Diminished production of adrenocortical hormones
Frequently associated with diabetes mellitus but
and an increased secretion of melanocyte
uncommon. Not related to its severity, duration or
stimulating hormone (MSH) by the pituitary.
treatment.
Clinical manifestations Clinical Manifestations
Middle-aged women. Asymptomatic, irregular Uncommon. Young adults. Diffuse brown-black
or oval, large, yellowish, shiny, atrophic plaques pigmentation, most pronounced in the flexures,
Cushing’s syndrome: extensive purple colored striae in a case Acromegaly: large face with thick lips, big nose and large jaw.
of recalcitrant pemphigus vulgaris on high dose daily steroids.
Kwashiorkor: crackled scaly pigmented skin on the trunk and Phrynoderma: keratotic follicular papules.
extremities; the latter edematous.
Metabolic and Nutritional Dermatoses 203
Pellagra: well demarcated, hyperpigmented scaly lesion on the Pellagra: magenta-colored tongue and angular stomatitis.
dorsae of hands and feet.
Metabolic and Nutritional Dermatoses 205
Hypervitaminosis A Pellagra
Result of intake of large doses of vitamin A for Uncommon. Adults generally affected. Caused by
prolonged periods. deficiency of nicotinic acid—nutritional (consuming
maize or corn), alcoholism, malabsorptive or due to
Clinical Manifestations isonicotinic acid hydrazide (INH) therapy, Hartnup
disease or rarely a functional carcinoid tumor.
Skin lesions
Dry, itchy, scaly skin. Alopecia. Clinical Manifestations
Systemic manifestations Common manifestations dermatitis, diarrhea,
Include headaches, insomnia, lassitude, anorexia, dementia—the infamous 3 D’s. Dermatitis: a
bone and joint pains. photodermatosis; erythema, followed by brown-
black, large coarse scales with a peripheral red
Carotenoderma halo. Sharply delineated to the light exposed
Due to excessive intake of carrots or other yellow/ areas—face, ‘V’ of the neck (Casal’s necklace),
orange fruits and vegetables. dorsa of hands and feet. Tongue: atrophic and
magenta colored. Psychotic symptoms: mild or
Clinical Manifestations severe depression or irritability.
Vitiligo with koebnerization: linear lesions and linear extension Vitiligo: follicular repigmentation following PUVA therapy.
of old vitiligo lesions.
Disorders of Skin Pigmentation 209
Dermatoses affecting the skin pigment result Work-up for underlying autoimmune disorders
from presence of increased or decreased done based on relevant clinical and family history.
amount of normal pigment(s). Or the presence of
abnormal pigment(s). Socially and psychologically
Sites of predilection
devastating. Physiologically not so critical. Overlying hair retain pigment. Or turn white
(leukotrichia). No autonomic or sensory disturbances.
Acute sunburn on light-exposed areas. Or chronic
DISORDERS OF DECREASED solar damage in long-standing cases. Malignancies
PIGMENTATION seldom seen. Confined to mucocutaneous junctions
or acral parts, tacrot aciati or randomly all over the
Vitiligo body (vulgaris), or limited to one segment of body,
Acquired depigmentation of the skin. usually unilaterally (segmental). May be generalized
or universal. Skin overlying bony prominences
Epidemiology particularly affected, sometimes symmetrically.
Extremely common; Worldwide prevalence
approximately 1%. Both sexes equally affected. Course
Considerable psychosocial importance. No age is Unpredictable and capricious. Lesions stationary,
exempted; the disease frequently starts in children self-healing or progressive. Prognosis better in
and young adults. young patients, early lesions and hairy areas.
Repigmentation—spontaneous or therapy induced
Etiology —often caused by repopulation of depigmented skin
Biochemical, neural, autoimmune and genetic are with melanocytes from hair follicles (perifollicular) or
the classic four hypotheses but newer ones include the adjoining normal pigmented skin.
the convergence theory by Le Poole et al, imbalance
of epidermal cytokines, melanocyte dysfunction and Histopathology
melatonin theory. As yet etiology is uncertain. Auto- Decrease or absence of melanocytes.
immune hypothesis most widely accepted. Genetic Early cases: May show interface dermatitis with
predisposition: autosomal dominant trait with variable mild Iymphohistiocytic infiltrate.
penetrance; history of vitiligo amongst first degree Advanced/fully developed cases: Lymphocyte
relatives in about a fifth; relative risk of developing induced apoptosis of melanocytes. Absence
vitiligo is 7 amongst children whose parents are of melanocytes and melanin subsequently.
affected association with other autoimmune disorders Immunohistochemical staining demonstrates a
and presence of humoral and cell-mediated immune preponderance of CD4 positive cells.
aberrations cited as evidence of autoimmunity.
Associations—Type I diabetes mellitus, autoimmune Treatment
thyroiditis, uveitis, alopecia areata and halo nevi. Treatment for vitiligo is difficult and usually pro-
longed. Based on body surface area of involvement,
Clinical Manifestations topical therapy for vitiligo is usually recommended
Asymptomatic macules of varied configuration- when depigmentation is less than 10–20% of
irregular, oval, circular, linear or punctate. Discrete the skin surface and systemic therapy when
or confluent. May start as hypopigmented exceeding these limits; but when topical therapy
macules on an otherwise normal skin and become of small areas fails or the disease is progressive,
depigmented. Depigmentation, hypopigmentation systemic treatment may be indicated. With
and normal or hyperpigmentation in the same lesion available medical therapies, high repigmentation
trichrome vitiligo. Koebnerization or isomorphic percentages mostly on facial and neck lesions
phenomenon is seen in active disease; lesions are achieved, although they are less effective on
develop at sites of trauma or may exacerbate with trunk and limbs and poor on the acral parts of the
sunlight. extremities. Persistence of achieved regimentation
Vitiligo with photo-koebnerization: predominance of vitiligo Halo nevus: depigmentation around a pigmented mole.
lesions on sunexposed sites.
Contact leukoderma: due to adhesive in the ‘bindi’ worn on Chemical hypomelanosis: mottled depigmentation caused by
forehead by Indian women. monobenzyl ether of hydroquinone.
Disorders of Skin Pigmentation 211
is variable and an undefined percentage of may be single or multiple. Associated with organ
patients may have variable recurrence. The specific autoimmune disorders like vitiligo, nevus
medical modalities include topical agents such as gradually involutes leaving behind a depigmented
corticosteroids, calcineurin inhibitors, phototherapy area for long time; frequently repigments to normal
[topical psoralen plus ultraviolet A (topical PUVA), skin color. No treatment/excision required.
topical psoralen plus sunlight (topical PUVA sol),
targeted phototherapy (308 nm excimer laser or Chemical Leukoderma
light only to involved area)] and systemic agents Acquired hypomelanosis induced by repeated
including immunosuppressives and phototherapy/ exposure to chemical substance either by
photochemotherapy [oral psoralen plus UVA (oral direct contact or from systemic exposure
PUVA), oral psoralen plus sunlight (oral PUVA (inhalation, ingestion). Except during early stage,
sol) and narrow-band ultraviolet B (311+/– 2 nm, depigmentation is irreversible.
NB-UVB)]. NB-UVB has emerged as probably the
most effective therapy for generalized disease with Epidemiology
superior repigmentation, color match and tolerance
as compared to psoralen photochemotherapy. Off The incidence of chemical leukoderma has been
late, combination of topical calcipotriol and photo/ increasing considerably in recent years due and
photochemotherapy have been used with some this is often misdiagnosed clinically as idiopathic
benefit but the efficacy of topical calcipotriol is as vitiligo. Contact/occupational vitiligo is distinct
yet questionable. When vitiligo becomes refractory, from chemical leukoderma in that the initial
surgical methods may improve depigmentation cutaneous depigmentation extends from the site of
as effectively as with medical therapy. Surgical chemical contact and subsequently develops into
methods include micro punch grafting, split progressive, generalized vitiligo.
thickness grafting, non-cultured epidermal cell
suspension (NCES) and suction blister grafting. Etiology
NCES is the most well established, and along with Cytotoxic effect to melanocytes more important
SBG, is one of the most common vitiligo surgeries as opposed to an immunological effect. The
performed. Lately, new technique of follicular compounds known to produce leukoderma are
outer root sheath cell suspension has also been chemically rather similar to tyrosine and DOPA,
used with success. In resistant generalized or the endogenous substrates of the melanocyte-
universal vitiligo, residual normally pigmented specific enzyme tyrosinase. The majority of these
skin may be depigmented with 20% monobenzyl chemicals are aromatic or aliphatic derivatives of
ether of hydroquinone. Q switched alexandrite and phenols and catechols. Other contributory toxins
ruby laser have also been used for depigmenting are sulfhydryls, mercurials, arsenics, cinnamic
residual pigmented skin. aldehyde, p-phenylenediamine, azelaic acid,
corticosteroids, tretinoin, otic preparations such
Halo Nevus (Sutton’s Nevus) as eserine and thiotepa as well as systemic
Area of hypopigmentation/depigmentation around medications such as chloroquine and fluphenazine
a cutaneous ‘tumor’. Most commonly halo (prolixin). Subsequently para phenylene diamine
phenomenon appears around acquired melanocytic (hair dyes), epoxy resins and azo dyes (‘alta’,
nevus but can occur with congenital melanocytic lipstick, eyeliner) have also been incriminated.
nevi, melanomas and non-melanocytic tumors Depigmentation on contact with articles
as well. Usually a marker of resolution of nevus. containing MBH or other depigmenting chemicals,
Appears as a round to oval area of hypo/ rubber footwear, adhesive coated ‘bindi’
depigmentation. Leukotrichia may be present in (adornment worn on forehead by Indian women),
hairy areas. Most common site is back. Lesions rubber gloves, possibly some condoms.
Contact leukoderma: caused by contact with rubber footwear. Pityriasis alba: ill-defined hypopigmented macules on the cheek.
Treatment Histopathology
Self-limiting. Hypopigmentation may persist for Biopsy may be performed when close differential
long. Bland emollients. Mild topical steroids may diagnoses present like drug induced hyper-
help in long standing cases. melanosis, exogenous ochronosis, acanthosis.
Biopsy shows increased epidermal melanocytes
Progressive Macular Hypomelanosis (more dendritic than normal skin) and dermal
Uncommon condition, predominantly affecting melanophages.
trunk. Discrete to confluent, non-scaly, discoid
hypopigmented macules. Topical steroids/ anti- Course
fungals do not work. Spontaneous resolution may Chronic. According to a study only 8% patients
occur, may subside with sunexposure/phototherapy. showed spontaneous remission.
Melasma: predominant malar involvement. Berloque dermatitis: caused by application of scented cream.
Periorbital melanosis: often familial. Lichen planus pigmentosus, face: typical slate-grey pigmentation.
Disorders of Skin Pigmentation 215
Treatment of melasma
Line Main modality Alternative
First choice 2–4% hydroquinone or triple combination (hydroquinone 4%, Azelic acid 20%
tretinoin 0.05% and fluocinolone 0.01%)
Adjuvant Ascorbic acid (stable form, 5%) Kojic acid 2%
Second choice Glycolic acid peels (starting from 30%) Tretinoin 1% peel
Third choice Fractional CO2, low fluence Q switched Nd:YAG Intense pulse light
Better studies awaited but Tranexamic acid (oral 250–1 gm daily, topical 5% gel, injectable
hold promise intradermal solution), arbutin, soy extracts, licorice
Broad spectrum sunscreens to be used several sunglasses. Sunscreens and mild demelanizing
times during day time. topical preparations like kojic acid/arbutin/vitamin
C may provide some relief but evidence poor/
Treatment lacking. Shadow effect may be mitigated by
Broad spectrum sunscreens needed irrespective dermal fillers or autologous fat transfer.
of treatment modality. Numerous botanical
extracts used but evidence poor for most. Well Lichen Planus Pigmentosus
studied modalities mentioned in table. Diagnosis Macular variant of lichen planus. Characterized by
mainly clinical. slate-grey pigmentation. Common in India; also
described from Latin America, Japan and Europe.
Berloque Dermatitis Differentiation from ashy dermatosis (erythema
Uncommon. Females affected. Distinctive streaky dyschromicum perstans) is as yet uncertain. Still
brown-black pigmentation generally on the sides of debatable whether they are distinct clinical entities
neck, upper chest or face. or part of same spectrum. Differentiation between
Result of photosensitivity caused by bergapten the two entities (as per the authors’ opinion) shown
(5-methoxypsoralen) present in perfumes containing in table. Young adults affected. Asymptomatic or
bergamot oil. mildly itchy, small or, by confluence, large, irregular
or oval macules of slate-grey to blue-black color.
Treatment
Protect from sunlight. Avoid contact of perfumes
with light exposed parts of the body.
Periorbital Melanosis
Common in certain races-often familial. Dark brown
pigmentation around the eyes, accentuated with
physical or mental fatigue. Other factors include
atopy, vascularity (dermal venules give dark color)
and shadow effect (due to loss of periorbital fat
and subsequent sunken appearance). Stationary
or progressive.
Treatment
No satisfactory treatment but innumerable have
Histopathology of lichen planus pigmentosus: vacuolar
been tried. General measures include proper degeneration of epidermal cells, incontinence of melanin and
sleep/diet/stress management, photoprotective focal upper dermal mononuclear infiltrate.
Lichen planus pigmentosus: diffuse bluish-grey pigmentation Ashy dermatosis (erythema dyschromicum perstans): solitary
in a photosensitive distribution. slate grey patch with raised erythematous border.
Idiopathic eruptive macular pigmentation: scattered hyperpig- Facial acanthosis nigricans: dark brown-black pigmentation
mented macules over the trunk; inset shows lesions with velvety involving the temple and lateral cheek showing subtle velvety
surface resembling acanthosis nigricans. texture.
Courtesy: Dr Geeti Khullar, Department of Dermatology, PGI,
Chandigarh.
Disorders of Skin Pigmentation 217
Starts from preauricular region with gradual spread in children and adolescents (peri-pubertal). Appear
to face, upper extremities, trunk and abdomen. in crops over face, trunk and proximal extremities.
Associated with other, particularly follicular, Etiology is unknown. May be form of eruptive
lichen planus. Mucosal involvement infrequent. acanthosis nigricans. Spontaneous remission over
Progressive course. months to years.
Dowling Degos disease: punctate perioral pits (especially along Reticulate acropigmentation of kitamura: punctate dark brown
borders of lips and angles of mouth). macules over dorsae of both hands.
Disorders of Skin Pigmentation 219
RAPK: Reticulate acropigmentation of Kitamura; NFJS: Naegeli Franceschetti Jadassohn syndrome; DPR: Dermatopathia
pigmentosa reticularis; DSH: Dyschromatosis symmetrica hereditaria; DUH: Dyschromatosis universalis hereditaria;
DKC: Dyskeratosis congenita; CARP: Confluent and reticulate papillomatosis; RPD: Reticulate pigmentary disorders;
DDD: Dowling Degos disease.
13 The ‘Connective
Tissue Diseases’
Chronic discoid lupus erythematosus: depigmented scaly Hypertrophic discoid lupus erythematosus: erythematous ver-
plaques over right malar eminence, lower eyelid nasal bridge rucous plaques involving both cheeks almost symmetrically.
and lower lip.
Chronic discoid lupus erythematosus: scaly red plaque involv- Palmoplantar discoid lupus erythematosus: depigmented
ing the ear concha, a typical site. scaly plaques with blanchable erythema involving the palms and
soles in a patient with disseminated DLE.
The ‘Connective Tissue Diseases’ 223
LUPUS ERYTHEMATOSUS
Cutaneous lupus erythematosus (LE) is of three
Discoid Lupus Erythematosus
types: acute cutaneous LE (ACLE); subacute
cutaneous LE (SCLE) and chronic cutaneous LE Chronic. Cutaneous and/or mucocutaneous
(CCLE). Details of the Gilliam classification are involvement. No systemic component. Common in
given in the table. These subtypes are probably a young or middle aged adults, more often females.
continuous spectrum. Autoimmune etiology. IgG and complement bound
Discoid lupus erythematosus-lichen planus overlap: vio- Discoid lupus erythematosus with squamous cell carcinoma:
laceous plaques over the nose and upper lip suggestive of LP. long standing discoid lupus erythematosus plaques with over-
Dipigmented plaque with peripheral hyperpigmentation over the lying ulcerated and cauliflower like squamous cell carcinoma
lower lip. plaques.
The ‘Connective Tissue Diseases’ 225
Histopathology
Hyperkeratosis with follicular plugging. Irregular
atrophy of the stratum malpighii. Liquefactive
degeneration of basal cell layer. Periappendageal
and perivascular lymphocytic infiltration. Mucin
deposition in dermis.
Direct immunofluorescence (DIF): IgG and C3
deposit in basement zone, only lesional skin.
Course
Untreated lesions tend to persist. Scarring in about
2/3rd and pigmentary abnormalities in 1/3rd. Risk
of developing SLE 6.5% for localized DLE, 22%
for disseminated DLE, 20% for chilblain like LE,
and about 50% of lupus profundus have SLE.
Long standing DLE lesions may rarely develop
malignancies like SCC and occasionally BCC.
Treatment
Protect from sunlight. Sunscreens—organic
like benzophenones or inorganic such as
titanium dioxide and calamine lotion. Protective
clothing. Topical (often potent) or intralesional
corticosteroids for small localized lesions. For more
Histopathology of chronic discoid lupus erythematosus: atro- extensive lesions, systemic chloroquine (250 mg
phy of the epidermis, keratotic plugs, vacuolar degeneration of twice a day for about a month, later once daily) or
basal cell layer, dense inflammatory infiltrate in upper dermis and hydroxychloroquine (200 mg twice a day). Watch
around the appendages; top (10x). Bottom (10x) shows marked for ocular toxicity when on chloroquine therapy.
hyperkeratosis in addition to above findings.
Or corticosteroids (prednisolone 20–40 mg/day or
equivalent).
to the basement zone of the lesiotial skin (c.f. Systemic Lupus Erythematosus
SLE). Light exposed areas preferentially affected. A multisystem disease. Cutaneous involvement, an
Commonly affects the face (cheeks, bridge of important, often diagnostic, but not an invariable
the nose, forehead), ears, scalp. Less frequently, component. Kidneys, joints, heart, lungs, nervous
extremities and trunk, lips and buccal mucosa. system and other organs/systems affected.
Designated disseminated OLE when lesions below
neck. Bilateral, often symmetrical; occasionally Etiology
unilateral. Well-defined, erythematous and scaly Autoimmune etiology. Organ-specific and non-
plaques with follicular plugs. Scales large and specific antibodies in circulation. Immunoglobulins
adherent. Removal of the scale reveals adherent and complement bound to the basement zone in
follicular plugs at its undersurface and this is known the clinically affected and unaffected skin (c.f. DLE).
as ‘carpet tack’ or ‘tin tack’ sign. Peripheral spread Genetic predisposition perhaps operative. Certain
with brownish discoloration; central healing with drugs, such as procainamide, sulfonamides,
depigmentation and atrophy or scarring. Scalp: hydantoin and griseofulvin known to precipitate
cicatricial alopecia. SLE. Young adults; females far more frequently,
Lupus profundus et hypertrophicus (DLE with LE panniculi- Complement deficiency systemic lupus erythematosus: exten-
tis): erythematous scaly plaques with atrophy scarring and fat sive lesions on the cheeks and nose, upper lip and eyelids with
loss over left mandibular area. facial scarring and bilateral ectropion.
Systemic lupus erythematosus (acute lupus erythematosus): Toxic epidermal necrolysis like lupus erythematosus: hemo-
characteristic malar rash, sparing the nasolabial folds, perioral rrhagic crusting over lips, palatal ulceration with malar rash.
area and eyelids.
The ‘Connective Tissue Diseases’ 227
Subacute cutaneous lupus erythematosus: erythematosqua- Rowell’s syndrome: a typical targetoid lesions subsiding with
mous lesions on face, ‘V’ of neck, shoulders and extensors of arms. exfoliation over right palm in a patient of systemic LE.
The ‘Connective Tissue Diseases’ 229
Atrophoderma of pasini pierini: burnt out morphea plaque Morphea profundus: multiple depressed plaques with subcuta-
over flank causing soft tissue atrophy. neous atrophy with overlying normal or slightly pigmented skin
seen over the forehead.
The ‘Connective Tissue Diseases’ 231
Systemic sclerosis: typically taut expression-less facies with Systemic sclerosis: mat-like telangiectasia over the face.
pinched nose.
The ‘Connective Tissue Diseases’ 233
Treatment
Localized Morphea
Topical tacrolimus is first line agent. Mid potent topical
steroids may be used. Further, topical calcipotriol-
betamethasone dipropionate combination, topical
imiquimod may be given. UVA/UVA sol/NBUVB/
MTX in resistant cases.
Generalized Morphea
Phototherapy is first line (UVA/NBUVB). Further
methotrexate, systemic steroids and mycopheno-
late mofetil may be given.
Systemic sclerosis: acro-osteolysis-resorption of terminal ends
Systemic Sclerosis of distal phalanges.
A common multisystem disease. Autoimmune
etiology.
Clinical Manifestations
Young adult females affected more frequently.
Raynaud’s phenomenon, often the first complaint.
Skin changes—edematous, thickened and
indurated; later, atrophic, smooth, shiny and
bound-down. And difficult to pinch. Hands, feet,
face affected first. Later (though not always) trunk
and proximal parts of extremities. Characteristic
fades—expressionless (‘mask facies’); ironed out
skin folds. Pinched nose. Small mouth with radial
furrows on closing. Telangiectasia-‘mat-like’—on
face and palms. Hands and feet—edematous;
pulp of fingers resorbed; sclerodactyly. Painful
gangrenous ulceration and flexion deformity of X-ray chest of advanced systemic sclerosis patient: showing
fingers. Pigmentary changes—diffuse generalized extensive interstitial lung diseases and fibrosis, more prominent
hyperpigmentation or depigmentation or both— salt in bilateral lower lung zones.
Lichen sclerosus: wrinkled hypopigmented plaque with pitted Vulval lichen sclerosus: depigmented plaque involving the
follicular scars over abdomen. vulva causing destruction of clitoris, urethral stenosis and punc-
tate erosions over labia minoria.
The ‘Connective Tissue Diseases’ 235
and pepper pattern typical. Calcinosis of skin some authors consider it to be a form of
around the joints and stellate scars on finger tips. morphea. Because numerous cases of morphea
Systemic involvement—variable, depending on associated with LS exist in literature as well as
type whether limited variant or diffuse. Arthralgia/ clinical practice. Uncommon. Etiology unknown.
arthritis, dysphagia, alternating constipation and Biphasic age distribution. Children or young adults.
diarrhea. Exertional dyspnea due to pulmonary Small, discrete or confluent, ivory white, atrophic
interstitial fibrosis. Cardiac, renal and skeletal maculopapules or plaques with central depression.
muscle involvement occasional. Dilated pilosebaceous orifices with keratotic plugs.
Trunk, genitals and perianal region affected. Rarely
Course seen in oral mucosa.
Variable, often progressive. Death from intercurrent Vulvar lesions—atrophy and shrinkage of
infections. Or respiratory, cardiac or renal failure. the vulva and narrowing of the vaginal introitus.
Poorer prognosis in males. Malignant potential, often debated.
Diagnosis Course
American Rheumatology Association criteria are Variable and unpredictable—spontaneous
sensitive as well as specific. Single major criterion is resolution, stationary or progressive. Malignant
scleroderma proximal to the digits. At least two of the potential controversial.
minor criteria should be met—sclerodactyly, digital
pitted scarring and bilateral basal pulmonary fibrosis. Histopathology of Lichen Sclerosus
Atrophic epidermis with hyperkeratosis and
Treatment keratotic plugs. Basal cell degeneration. Edema
None satisfactory. Protect from cold and trauma. and hyalinization of the upper dermis. Lymphocytic
Abstain from smoking. Raynaud phenomenon/ infiltrate below the hyalinized area.
digital ischemia—calcium channel blockers
(nifedipine) are first line. Others therapies proven
useful include intravenous iloprost, oral bosentan
(endothelin receptor antagonist), sildenafil/
tadalafil (phosphodiesterase inhibitors), alpha
adrenergic blockers. Immunomodulator therapy
for internal involvement—cyclophosphamide,
methotrexate, extracorporeal photopharesis.
Less proven therapies include cyclosporine,
mycophenolate mofetil, rapamycin.
Symptomatic management for system wise
complaints.
LICHEN SCLEROSUS
Term lichen sclerosus (LS) is preferred in place Histopathology of lichen sclerosus et atrophicus: epidermal
of lichen sclerosus et atrophicus since all lesions atrophy, keratotic plugs, edema and hyalinized upper dermis
may not be atrophic. Although it is debatable, with inflammatory infiltrate in the mid dermis.
Balanitis xerotica obliterans: hypopigmented glans with a Dermatomyositis: erythema and edema of both upper and
fleshy plaque suggestive of early squamous cell carcinoma and lower eyelids.
preputial phimosis.
Calcinosis cutis in juvenile dermatomyositis: chalky white pap- Relapsing polychondritis: swelling involving the cartilaginous
ules and ulcers studded over a calcinosis cutis swelling near the part of ear, sparing the lobe.
elbow.
The ‘Connective Tissue Diseases’ 239
Treatment
RELAPSING POLYCHONDRITIS
Moderately large doses of systemic corticosteroids
(1–2 mg/kg). Immunosuppressives are useful Recurrent attacks of chondritis. Most common being
as steroid sparing agents and adjuvants. auricular (>80%) followed by nasal, respiratory and
Methotrexate (primarily for skin manifestations), costochondral. Swelling of ear involves only the
azathioprine, mycophenolate mofetil, cyclosporine, cartilaginous portion, sparing the lobe. Repeated
cyclophosphamide, etc. Newer agents reported attacks cause cartilage loss and sagging.
to be of use—etanercept, infliximab, rituximab. Associated with—arthralgias, ocular inflammation,
Calcinosis usually does not respond to any mode cardiac manifestations, etc. Treatment depends on
of therapy although several agents have been system involvement. Usually NSAIDs, colchicine
anecdotally found to be useful—alendronate, or dapsone sufficient for mild or moderate attacks.
14 Disorders Due
to Physical
Agents
Callosities: on the palms of a manual worker due to intermittent Callosities: due to caliper splints.
moderate trauma.
Flutist’s chin: flute in contact with the chin and lower lip. Flutist’s chin: hyperkeratotic plaque on the lower lips and chin
the site of contact with the flute.
Disorders Due to Physical Agents 243
Erythema ab igne: reticulate pigmentation and slight hyperkeratosis. Chilblains: erythematous to dusky edematous plaques over fingers
of both hands with focal scaling.
Disorders Due to Physical Agents 245
Frostbite
DISORDERS DUE TO EXPOSURE TO LIGHT
Rare. Result of exposure to extreme dry cold and
freezing of the tissues. Seen in mountaineers and Sunburn
troops stationed at high altitudes or other cold
Common. Physiological. Acute inflammatory,
conditions. Superficial or deep. Extent of injury
response seen 8–10 hours after (over) exposure
apparent only on thawing. Superficial frostbite:
of the skin to short ultraviolet light (UVB) from the
erythema, edema and vesiculation; deeper tissues
sun or artificial sources. Albinos, skin type I and
feel soft. Deep: dry, shrivelled-up, gangrenous
II (fair skinned, blue eyed, red haired) individuals,
peripheral part(s) of the body—the hands, feet, tip
and patients with vitiligo more susceptible. More
of the nose and pinna of the ears. Complete tissue
common at high altitudes or sandy beaches because
death; dead portion may drop off. Deceptive lack of
of higher UVB content, (due to reflection); hence
pain during exposure; thawing painful.
amongst mountaineers and trekkers. Face or other
exposed areas. Erythema, edema, and vesiculation.
Treatment
Itching, burning or pain. Heal with desquamation
Rapid rewarming till erythema returns. and hyperpigmentation. Self-limiting course.
Scrupulously avoid further exposure to cold or
any trauma and pressure. Analgesics to relieve Treatment
pain associated with thawing. Surgery of the
Prevent. Avoid overexposure to sunlight. Or use
gangrenous part to be postponed for several
sunscreens or sunblocks. Topical (or systemic)
weeks until demarcation occurs.
corticosteroids for moderate to—severe cases.
Antihistamines help only as antipruritics.
Raynaud’s Phenomenon
Can be idiopathic, called raynaud’s disease. Most Solar Elastosis
common cause in dermatology connective tissue
Common among white populations exposed for
diseases (scleroderma, SLE, RA, Sjögren’s). Other
prolonged periods to sunlight; hence commoner
causes trauma, atherosclerosis, neurological or
Frostbite: gangrenous tips of toes; erythema and blistering Raynaud’s phenomenon: pallor of 2–3 fingers and rest all show
located proximally. bluish discoloration, including the palms.
Severe sunburn, back: desquamating, sharply defined lesions; Solar elastosis: skin colored papules and comedones with exag-
area covered by vest spared. gerated skin markings.
Disorders Due to Physical Agents 247
amongst farmers and sailors. Uncommon among Polymorphous Light Eruption (PLE)
colored races except in albinos, mountain dwellers
and patients with vitiligo. Middle aged or older Epidemiology
individuals. Face (malar areas), neck (nape) and Most common photosensitivity disorder with an
dorsa of the hands generally involved. Several closely estimated prevalence varying from 1 to 21%.
related syndromes. Characterized by degeneration
of elastic tissue (and atrophy of epidermis). Pale Etiology
translucent papules with wrinkling or furrowing of the
Radiation spectrum—UVA >UVB >UVA + UVB.
skin. Comedonic lesions on the circumocular areas.
Possible delayed type hypersensitivity to
Combination of solar elastosis and patulous open
endogenous cutaneous photo-induced antigen.
comedones known as Favre Racouchot syndrome.
Genetic predisposition.
Clinical Manifestations
‘Polymorphous’ designates interindividual
variation. Usually there is development of eruption
after minutes to hours after sun-exposure. Most
commonly appears as grouped erythematous to
skin colored papules sometimes coalescing to
form plaques. Other variants include micropapular,
hypopigmented, eczematous, lichenoid, papulo-
vesicular, vesiculo-bullous, insect bite like, EM like,
PMLE sine eruptions, etc.
Juvenile spring eruption—Involvement of ear
helices of young boys in the form of papules and
vesicles.
Histopathology of solar elastosis: basophilic, fine, short elastic Photosensitive spongiotic and lichenoid eruption
fibers and amorphous material, upper dermis. (PSLE) discrete to confluent papules over sun-
exposed sites sparing face and histology showing
spongiotic and lichenoid reaction patterns.
Treatment Investigations
Prevention—avoid excessive exposure to sunlight. Photoprovocation testing with UVA may be done in
Use sunscreens—organic. Or inorganic. Topical case of diagnostic confusion.
retinoic acid (0.05–0.1%) may help.
Chronic Actinic Dermatitis
IDIOPATHIC PHOTODERMATOSES (Actinic Reticuloid; Photosensitivity
Dermatitis; Photosensitive Eczema;
Classification Persistent Light Reactivity)
Polymorphous light eruption
Chronic actinic dermatitis Epidemiology
Solar urticaria Relatively uncommon disease. Males are much
Actinic prurigo more commonly affected. More common in 6th to
Hydroa vacciniforme 7th decade.
Polymorphous light eruption: lichenoid, micro-papular form, Hypopigmented variant of PMLE: hypopigmented patches over
extensors of forearms. the shoulder.
Chronic actinic dermatitis: erythematous infiltrated plaques Chronic actinic dermatitis: erythematous scaly plaques diffusely
over face, sparing the periorbital area, part of forehead covered involving face with sparing of the forehead wrinkles, periorbital
by turban and relatively sparing the nasolabial folds. and perioral area, and the nasolabial folds.
Disorders Due to Physical Agents 249
Photo onycholysis: distal onycholysis and adjoining brownish Actinic cheilitis: of the lower lip.
discoloration of a patient on PUVA therapy.
Disorders Due to Physical Agents 251
Treatment of photodermatoses
Phase Polymorphous Chronic actinic Solar urticaria Actinic prurigo Hydroa
light eruption dermatitis vacciniformis
Acute flare Topical/systemic Topical/Oral steroids Photoprotection, H1 Thalidomide None/symptomatic
steroids antihistamines
Chronic/refractory Antimalarials, Topical calcineurins, CsA, plasmapheresis/ Antimalarials, -
cases Immuno- Cs, MTX, AZA, MMF, ECP antihistamines,
suppressants low dose PUVA steroids (t/s)
Prevention Sunprotection > Avoid allergens, UV Sunprotection, UVA/ Sunprotection > Sunprotection >
Phototherapy protective clothing PUVA desensitization Phototherapy Phototherapy
Cs: Cyclosporine; AZA: Azathioprine; MTX: Methotrexate; MMF: Mycophenolate mofetil; ECP: Extracorporeal photopheresis;
PUVA: Psoralen and ultraviolet A; Steroids (t/s): topical/systemic
Radiodermatitis: atrophy, depigmentation and ulcerations on Frictional sweat dermatitis: diffuse but well-defined area of thin
the fingers of a radiotherapist. polythene like scaling with focal peeling and fissuring over the
back in area apposed to the vest (please note that shoulders and
nape of neck and adjoining upper back are free).
Disorders Due to Physical Agents 253
Acne vulgaris, healed: atrophic and pitted scars; occasional Acne vulgaris: multiple papules and pustules.
papules.
Diseases of the Skin Appendages 257
unit. Primary lesion, a comedone, a non- Local perifollicular CD4 T-cells and IL-1 levels
pilosebaceous passage. Obstruction on the surface Increase lymphocytes and foreign body giant
or within the skin; the comedone respectively, cells → papules, nodules and cysts.
called an open (black head) or closed comedone Other factors
(white head). Most common acneiform dermatosis,
Diet: Still unclear. Few studies established
acne vulgaris. Several others—drug induced, due
causation with high glycemic diets and dairy
to topical applications or unrelated factors.
products.
Acne vulgaris, healed: disfiguring hypertrophic scars. Acne vulgaris: polymorphic eruption on the back.
Drug-induced acne: monomorphic papules. Acne conglobata: interconnecting sinus tracts with nodulocystic
acne.
Diseases of the Skin Appendages 259
Senile comedones: note associated wrinkling of skin due to solar Acne venenata, chest: comedones and inflammatory papules on
elastosis. contact with cutting oils.
Diseases of the Skin Appendages 261
In each course, 120–150 mg/kg can be given. Lesions chiefly open comedones, with or without
Potentially teratogenic, hyperlipidemic and mildly inflammatory papules or papulopustules. Site
hepatotoxic. Cheilitis, dryness of skin and eyes, depends on area of contact.
dry mouth and nose universal. Contraception
mandatory in females of childbearing age. Single Treatment
course generally adequatea—second course only Mild lesions resolve spontaneously. Or with
in case of severe relapse. topical retinoic acid. Severe disease may need
systemic isotretinoin.
ACNE VARIANTS
Acne Fulminans
Acne Conglobata
Adolescent males, most commonly trunk, abrupt/
Suppurating acne. Chronic. Relatively uncommon.
rapid onset over mild to moderate acne, associated
Young males affected. More frequent on back, chest
with fever, polyarthropathy, leucocytosis, malaise,
and shoulders; occasionally on buttocks and thighs.
bony pain, etc. Lesions become nodular, painful,
Double or triple comedones, inflammatory papules,
ulcerate and heal with scarring. Triggers include-
nodules and cysts; burst on the surface. Or burrow
testosterone, anabolic steroids, isotretinoin,
within the skin, forming interconnecting sinus tracts.
seasonal infections like EBV, propioniobacterium
Heal with disfiguring scars. Acne conglobata, part
acnes. Pus and blood culture usually sterile.
of follicular retention triad; hidradenitis suppurativa
and dissecting folliculitis of the scalp.
Treatment
Treatment Oral prednisolone. 0.5–1 m/kg/day slowly tapered
Appropriate systemic antibiotics with or without and stopped over 2–3 months. Crust removal
steroids. Isotretinoin, in doses of 1–2 mg/kg/day using emollients and antibiotic creams.
for 4–5 months, drug of choice.
Infantile and Neonatal Acne
Acne Venenata Neonatal acne—presents at 2–3 years of age as
Occupational hazard in people exposed to erythematous papules over nasal bridge. Infantile
halogenated hydrocarbons, cutting oils, crude acne—presents at 3–6 months of age usually as
petroleum. Use of cosmetics, vegetable oils or comedones.
topical corticosteroids responsible in some.
Erythemo-telangiectatic rosacea: diffuse erythema and telangi- Topical steroid induced rosacea: telangiectasias over cheek and
ectasias over cheeks and nose. nasal ala after chronic use of topical steroid for melasma.
Papulo-pustular rosacea: diffuse erythema over nose with mild Rosacea fulminans: confluent erythematous plaques, nodules
rhinophymatous change, studded with papules and pustules. and pustules over the nose and adjoining cheeks.
Courtesy: Prof AJ Kanwar, Dermatologist, Noida.
Diseases of the Skin Appendages 263
Hidradenitis suppurativa, early lesion: multiple ‘blind’ boils. Hidradenitis suppurativa: multiple abscesses, bridging sinuses
and scarring.
Diseases of the Skin Appendages 265
Systemic Therapy
Tetracyclines,
erythromycin.
Etiology
Histopathology of rhinophyma: dilated sebaceous glands. Initiallythought primary apocrine inflammation
but now postulated apocrine glands become
secondarily involved.
Rhinophyma
Follicular oulusion.
Uncommon. Elderly males generally affected. Familial tendency may be there.
Caused by hyperplasia of sebaceous glands and Bacterial infection as a cause is +/–.
connective tissue. Skin colored or reddish, often Smoking may worsen.
lobulated swelling of the tip and distal part of the
nose. Patulous pilosebaceous orifices. Foul smelling Clinical Manifestations
cheesy material expressed on pressure. Often end
Intertriginous area: Axillae, inguinal, perineal,
result of long standing/untreated rosacea. Huge
thighs.
nasal swelling may cause obstruction to breathing.
Hurley staging
But complaints is usually of cosmetic and social
Stage I: Isolated abscesses
nature.
Stage II: Sinuses with bridging scars.
Treatment Stage III: Chronic discharge, bridging scars,
Mild initial swelling/nodulation may respond sinuses and fibrosis, soft tissue hypertrophy.
Initial lesion a tender, painful, erythematous,
to oral isotretinoin but fully formed lobulated
swelling requires surgical interventions like CO2 intracutaneous nodule a ‘blind’ or ‘closed’
laser. Electrosurgery and blade surgery followed boil. Bursts on the surface with seropurulent
by flap repair. discharge. Or burrows within the skin to infect
adjoining glands and form interconnecting sinus
Perioral Dermatitis tracts. Recurrences common. Simultaneous
appearance of fresh lesions and scars continues
Correct term should be periorificial dermatitis, for months or years.
perioral, perinasal, periorbital. Most common Complication: Anal/urethral/rectal strictures.
trigger topical fluorinated steroid use/abuse.
Idiopathic in children (esp. granulomatous variant). Investigations
Other lesions papules, vesicles/pustules. Pus cultures from abscesses/deep part of
sinuses.
Treatment MRI to see complete event of scarring, sinuses
Female pattern hair loss, hamilton type: bitemporal recession Female pattern hair loss, advanced: diffuse hair loss and
and early baldness. baldness.
Diseases of the Skin Appendages 267
Alopecia universalis: loss of hair from scalp, eyebrows and rest Trichotillomania: solitary alopecic patch on frontal scalp with
of body. broken hair of variable lengths.
Diseases of the Skin Appendages 269
Etiology Investigations
Autoimmune disease—Autoreacting CD8 T-cells.
Usually clinical is sufficient.
Hair pull test usually negative.
Genetic factors—Family history (+) in many
KOH mount—to rule out tinea capitis.
cases.
Biopsy lymphocytic infiltrate in perifollicular and
Associations intrafollicular area esp. around the hair bulb
Certain associations—vitiligo, atopy, uveitis, (‘Swarm of Bees’ appearance).
diabetes mellitus, Down’s syndrome.
Treatment
Clinical Manifestations
Hair loss < 50% of scalp/localized
Morphology Topical
or ½ steroid
Patterns Minoxidil
5%
Patchy Topical dithranol.
Ophiasis (band like)
Reticular Hair Loss > 50% of Scalp/Extensive
Ophiasis immunesus Contact
immunotherapy (DPCP).
Diffuse Minoxidil
5% + topical steroid/anthralin.
Extent PUVA
Patchy – Areata Systemic
steroid—oral mini pulse.
Total scalp – Totalis Experimental therapy—JAK inhibitors (ruxolitinib,
Total body – Universalis tofacitinib).
New subtype – Acute diffuse and total alope-
cia. TrichotiIlomania (Hair Pulling Tic)
Generally sudden onset. One (or a few), well-defined, An impulse control disorder.
oval or circular area of total loss of hair. Exclamation
mark (!) hair at the periphery. Underlying scalp (or Epidemiology
skin) normal in color and texture; occasionally shiny
More common in children.
and mildly wrinkled. Recovery, often with regrowth
Overall,more common in females.
of white hair, later regain pigment. Associated nail
In adults, it may be associated with mood
changes—pitting, dystrophy.
disorders.
Sites of Predilection Etiology
Scalp, beard, upper lip, eyebrows, eyelashes or Children: Lack of attention, sibling rivalary,
other hair—bearing regions affected. learning disability, unhappiness.
Course Adults: Anxiety, mood disorders, mental
Unpredictable. Spontaneous recovery common retardation.
in solitary lesions. May show gradual or rapid
progression. Remitting and relapsing course. Clinical Manifestations
More favorable course in mild cases < 25% of Most commonly scalp is affected, other
area involved. May progress to involve the total uncommon like the eyebrows/beard.
Trichotillomania, tonsure type: diffuse alopecia over scalp with Anagen effluvium: due to cyclophosphamide therapy.
sparing of a rim of long hair along the hairline.
Traction alopecia: fronto-temporal baldness in a sikh boy due Pseudopelade of Brocq: discrete to confluent skin colored
to hair tying for turban. patches of hair loss, sparing intervening hair. Some oval patches
appear in line suggestive of ’foot prints in the snow’.
Diseases of the Skin Appendages 271
Epidemiology Treatment
More common in females. Minoxidil 2% may reduce the severity and duration
2ndmost common cause of hair fall after AGA. of anagen effluvium.
Can coexist with AGA.
Traction Alopecia
Etiology Caused by chronic, almost continuous ‘pull’ due to
Multifactorial and still poorly understood. hair dressing styles of special groups of people,
Endocrine such as the blacks or wearing of special head
Systemic illness gear (turban-wearing Sikhs) or beardstring (worn
Stressful events by Sikhs to keep the beard in place). Initially non-
Nutritional cicatricial, later scarred alopecia.
Intoxication
Drugs Treatment
Inflammatory scalp dermatoses.
Relieve traction. Reversible in early stages.
Clinical Manifestations
CICATRICIAL ALOPECIAS
Patterns
Permanent hair loss, replacement of follicles by
Acute/classic (duration < 6 months).
firosis. Starts in a patchy manner. Classification
Chronic (> 6 months)—idiopathic.
shown in Table.
Chronic diffuse telogen hair loss (> 6 months)—
2° to above factors.
Complain of bunches of hair coming out on combing.
Pseudopelade of Brocq
Baldness in advanced cases only. Uncommon. More in adults/young adults than
children. Nosological status uncertain. Variably
Pseudopelade: crab-like cicatrical alopecia. Folliculitis decalvans: shiny atrophic scaly area of scarring hair
loss, follicular pustules and crusting in the periphery.
Dissecting cellulitis of scalp: boggy scalp skin with scarring Acne keloidalis of nuchae, early: grouped follicular erythema-
alopecic patches, subtle interconnecting abscesses. Also note tous papules and pustules. Mild sparsening of hair.
depressed follicular scars.
Diseases of the Skin Appendages 273
Histopathology
Histopathology of pseuodopelade of Brocq: Unremarkable
Non-specific mild lymphocytic infilitrate around epidermis with paucity of pilosebaceous units and no dermal
infundibulum. Advanced cases show follicular inflammation. A linear scar replacing the follicle completely (run-
fibrosis and absence of sebaceous glands. ning perpendicular to the epidermis) and surrounded by atrophic
arrector pili.
Clubbing of nails: angle between the nail plate and nail fold Nail pitting: fine nail pits in patient with alopecia areata.
reduced.
Beau’s lines: horizontal furrows uniformly on all nails indicating Onychogryphosis: thickening and curving of the nail plate.
cessation of nail plate formation. Patient had pityriasis rubra pilaris.
Diseases of the Skin Appendages 275
Treatment Histopathology
Frustrating. Topical steroids with or without Perifollicular mixed infiltrate around isthmus and
intralesional steroids. If progressive, oral sebaceous gland region of hair.
prednisolone, oral minipulse (betamethasone),
HCQs. Treatment
Initially, class I and II topical steroids with or without
Dissecting Cellulitis of Scalp (also known as intralesional steroids. Oral or topical antibiotics
Perifolliculitis Capitis Abscedens et Suffodiens) (tetracyclines) may help. In advanced cases
Very rare. Mainly in dark skinned/Afro-American surgical excision upto muscle fascia and healing
individuals. Adults. with secondary intention or staged excision and
primary repair.
Etiology
Folliculitis Decalvans
Unknown. Abnormal follicular keratinization leading
to obstruction, secondary bacterial infection, and Rare, progressive purulent folliculitis. May involve
follicular destruction probable. any hair bearing site but mostly over vertex of
scalp. Adults only mostly. Etiology unknown. Many
Clinical Manifestations cases show isolates of Staphylococcus aureus.
May be localized immune deficiency against the
Distinctive clinical appearance. Painful fluctuant/ bacterium.
boggy nodules, abscesses and interconnecting Start as isolated pustules but progress to form
sinus tracts with scarring alopecia. May give miliary follicular abscesses followed by scarring in
cerebriform appearance to scalp. Associations: center and centrifugal spread. Tufting of intervening
Follicular occlusion triad—(with acne conglobata, hair may be seen.
hidradenitis suppurativa) and tetrad (as above
along with pilonidal sinus). Histopathology
Treatment Dense intra- and perifollicular neutrophilic infiltrate,
centered over upper and middle parts of hair.
Resistant disease. Oral isotretinoin (starting with
1 mg/kg/day then slow tapering over 1–2 years) Treatment
treatment of choice. Oral antibiotics—tetracyclines
(mino and doxy), cloxacillin, erythromycin. Oral antibiotic with/without topical antibiotic.
Spiration of abscesses and intralesional steroid. Oral erythromycin, minocycline/doxycycline,
clindamycin, rifampicin, fusidic acid. Long-term
Acne Keloidalis of Nuchae control needs intermittent therapy. Dapsone also
useful.
Not so uncommon inflammatory condition. Young
post pubertal males, more in dark skinned people.
DISORDERS OF NAILS
Etiology Caused by local factors—trauma, infections, other
Uncertain but possibly multifactorial-racial local diseases or part of a generalized (systemic or
differences in pilosebaceous units, friction, cutaneous) disease. One or a few nails involved in
seborrhea, infection, autoimmunity. local diseases, many or all in generalized disease.
Direct nail plate involvement. Or secondary to
Clinical Manifestations involvement of nail matrix.
Smooth, firm follicular papules and pustules that
Clubbing
may coalesce into plaques and destroy hair. Most
common on occipital scalp near posterior hairline, Obliteration or reversal of the angle between the
may also affect vertex. nail plate and posterior nail fold. Hereditary or
Pterygium unguis: posterior nail fold adherent to the nail bed; Muehrcke’s pair white bands: paired white bands with interven-
note the split in the nail plate. ing pink bands.
Apparent leukonychia: diffuse whitening of the nails in an Pigmented longitudinal bands, nails: normal.
anemic (Hb = 7 gm/dL) patient.
Onycholysis, due to tinea unguium. Nail changes due to cosmetics: paronychia and nail plate dam-
age caused by nail polish and removers.
Diseases of the Skin Appendages 277
Onychogryphosis Onycholysis
Thickening, increase in length and curvature of Separation of nail plate from nail bed. Idiopathic,
nail plate. Great toe nails most frequently affected. due to psoriasis or onychomycosis. Photo-
Trauma, the most common factor. Psoriasis, pityriasis onycholysis (PUVA or PUVA sol), tetracyclines.
rubra pilaris, fungal infections responsible in some.
Trachyonychia
Treatment Rough surface of up to all 20 nails (20 nail
Regular, careful clipping off of nails. Avulsion in dystrophy).
some. As known as ‘Sand blasted nails’.
Housewife onycholysis: distal and lateral onycholysis of finger Ingrowing toe nail: lateral nail fold of great toe is swollen, with
nails of dominant hand in a housewife. purulant discharge and excess granulation overlapping the nail
plate.
Ingrowing toe nail: bilateral lateral nail folds of great toe are Periungual wart: huge wart involving the lateral fold, tip and
swollen, with excess granulation. nail bed of the great toe nail. Note distal and lateral onycholysis
of the involved nail.
Myxoid cyst, middle finger: solitary cyst over the proximal nail Nail bed glomus tumor: localized bluish discoloration visible in
fold and adjoining longitudinal gutter in the nail plate. the lateral part of nail bed and adjoining lateral nail fold.
Courtesy: Dr Shanta Passi, Dermatologist, ESIC Hospital, Faridabad.
Diseases of the Skin Appendages 279
Lamellar ichthyosis: large, coarse scales. Note ectropion. Epidermolytic ichthyosis: thick corrugated scales over both
knees. Note blisters over the left inner thigh.
Genodermatoses 283
Minor variants
Self-healing collodion baby Autosomal recessive TGM1, ALOX12B, ALOXE3
Acral self-healing collodion baby TGM1
Bathing suit ichthyosis TGM1
Keratinopathic ichthyosis
Major variants
Superficial epidermolytic ichthyosis Autosomal dominant KRT2
Epidermolytic ichthyosis Autosomal dominant KRT1/KRT10
Minor variants
Ichthyosis Curth-Macklin Autosomal dominant KRT1
Autosomal recessive epidermolytic ichthyosis Autosomal recessive KRT10
Annular epidermolytic ichthyosis Autosomal dominant KRT1/KRT10
Epidermolytic nevi Somatic mutations KRT1/KRT10
Other forms
Loricrin keratoderma Autosomal dominant LOR
Erythrokeratodermia variabilis Autosomal dominant GJB3/GJB4
Peeling skin disease Autosomal recessive Unknown
Epidermolytic hyperkeratosis: generalized erythema and scal- Epidermolytic hyperkeratosis: localized erythema and verru-
ing, corrugated scaling over joints. Note bilateral wrist widening cous scales.
suggestive of rickets.
Ichthyosis hysterix generalized type: thick verrucous scales Ichthyosis hysterix nevoid type: Hyperkeratotic plaques
over dorsum of foot. arranged linearly on right thigh and leg.
Genodermatoses 285
conspicuously spared. Pronounced in cold dry hyperkeratotic and verrucous corrugated scales
weather. May completely remit in hot and humid particularly pronounced in the flexures. Offensive
season. Palmar creases accentuated with minimal body odour. Heat intolerance. Variable palmo-
or moderate hyperkeratosis. Heels dry and plantar keratoderma. Localized epidermolytic
fissured. Significant association with atopy and hyperkeratosis resembles epidermal naevi.
keratosis pilaris.
Ichthyosis Hysterix
X-linked Ichthyosis (Syn–Ichthyosis Nigra) Spiny hyperkeratotic scales in localized, nevoid or
Relatively uncommon. X-linked recessive generalized distribution. Localized more common.
inheritance. Steroid sulfatase gene mutation. Autosomal dominant inheritance. Associated striate
Only males affected. Manifests in infancy or early or diffuse paloplantar keratoderma. Erythema and
childhood. Large, brown to black (hence nigra), blistering minimal.
flat, adherent scales. Trunk, extremities, neck,
sides of face, buttocks affected. Flexures not Herlequin Ichthyosis
necessarily spared. Palmo-plantar keratoderma Severe erythrodermic variant. Autosomal
minimal. Heat and humidity help in relieving the recessive, sporadic. ABCA12 mutation. ‘coat of
condition. Extracutaneous associations include armour’ like appearance and distorted facies
crypto-orchidism, non-blinding corneal opacities, and extremities. Deep fissures, thick adherent
male pattern baldness etc. skin. High mortality rate in first few weeks of life,
improves with ICU care. Main concerns being fluid,
Lamellar Ichthyosis electrolyte, feeding and temperature management.
Uncommon. Variable clinical severity but
usually more extensive than IV and XLRI. Now Netherton Syndrome
classified under ARCI (autosomal recessive Ichthyosiform syndrome. Autosomal recessive.
congenital ichthyosis). Autosomal recessive SPINK5 mutation. Triad of erythroderma (ichthyosis
inheritance; consanguinous parents. Mutation linearis circumflexa), atopic dermatitis and hair
in transglutaminase 1 gene. Present at birth. A shaft defect (trichorrhexis invaginata).
parchment like membrane enveloping the whole
body—Collodion baby. Membrane sheds off in a
few days; generalized erythema (less than non-
bullous ichthyosiform erythroderma, NBIE). Later,
generalized coarse, large scales fixed in the
center. Facial skin rigid and taut with ectropion and
eclabion (eversion of lips). Offensive body odor.
Blockage of sweat glands and heat intolerance.
Marked keratoderma and fissuring of palms and
soles. Deep fissures on flexures and joint surfaces.
Flexion contractures, sclerodactyly may occur.
Unna Thost PPK: Diffuse yellowish thickening of palms with ery- Olmsted syndrome: Marked verrucuous thickening of bilateral
thematous border. palms and soles causing functional disability.
Pachyonychia congenita: Focal PPK of both palms and diffuse Striate palmoplantar keratoderma: Linearly arranged yellowish
PPK of both soles. Marked tubular thickening of nails. plaques involving the palms and fingers.
Genodermatoses 287
or 16 gene. Thick and tented nails (fingers and toes) Treatment of Palmo-plantar Keratodermas
with onycholysis. Paronychia frequent. Follicular
Keratolytics mainstay of therapy. Salicylic acid
keratosis (elbows and knees) and palmoplantar
(5–20%) ointment, retinoic acid (0.05–0.1%),
hyperkeratosis. Oral cavity-leukokeratosis. Hoarse-
propylene glycol (40–70%) in water. Systemic
ness, if larynx involved. Type II (Jackson-Lawler
retinoids in patients with severe disease. Or
syndrome): Mutation in keratin 17 gene. Features
mutilating variants—may prevent or delay mutilation.
of type I with natal teeth and steatocystoma
Oral retinoids may also improve periodontitis in
multiplex. Type III (Schafer-Branauer syndrome):
papillon-lefevre syndrome.
Features of type I with leukokeratosis of cornea.
Type IV (pachyonychia congenita tarda): Late-
Porokeratotic Disorders
onset in second or third decade.
Porokeratosis of Mibelli
Striate PPK
Uncommon. Autosomal dominant inheritance,
Linear thickening over palms, palmar aspect of genetic basis unknown. Annular plaques with
fingers and soles. More focal/diffuse over soles. To central, sometimes atrophic, depression.
look for woolly hair for syndromes. Characteristic edge: greyish, keratotic; often with
a central furrow. Centrifugal spread. Limbs, trunk,
Punctate Keratoderma of Palmar Creases face may be affected.
Keratotic papules involving the palmar creases, fall
of leaving punctate pits at the places. Linear Porokeratosis
Linear plaques along the lines of blaschko.
Papillon-Lefevre Syndrome Congenital or childhood onset, genetic basis
Autosomal recessive. Mutation in cathepsin unknown but aberration on chromosomes 12, 15
C gene. Triad of symmetrical transgradient or 18 postulated as for disseminated superficial
palmoplantar keratoderma, juvenile aggressive actinic porokeratosis. Morphology and histology
periodontitis and recurrent pyogenic infections of similar to classic porokeratosis.
skin and internal organs, especially liver. Mental
retardation, intracranial calcifications can occur. Histopathology of Porokeratotic Disorders
Parakeratotic plug (cornoid lamella) in the
hyperkeratotic epidermis. Granular cell layer
absent beneath the cornoid lamella.
Papillon Lefevre syndrome: panoramic view, showing ‘floating Histopathology of porokeratosis of Mibelli: cornoid lamella—
in air’ appearance of teeth. a parakeratotic plug.
Darier’s disease: scaly, discrete and confluent greasy papules in Darier’s disease: characteristic greasy papules in a seborrhoeic
seborrhoeic sites. distribution.
Darier’s disease: characteristic pits on palms. Darier’s disease: acral lesions with angular nicks in the nails.
Genodermatoses 291
Treatment
Topical steroids and tretinoin help. Topical
5-flurouracil, imiquimod, oral acitretin (if extensive)
also useful.
Histopathology
Moderate to marked hyperkeratosis, papillomatosis.
Suprabasal split with villi, acantholysis and
dyskeratotic cells in upper malpighian layers (corp
ronds and grains).
Treatment
Treatment difficult and unsatisfactory. Mild cases,
topical retinoic acid. Severe cases need systemic
Histopathology of darier’s disease: intra-epidermal split. Note retinoids (acitretin, 0.5–1 mg/kg/day). Relapses
dyskeratotic cells (corps ronds) in upper epidermal layers; top
occur on discontinuation of therapy.
(10x), bottom (40x).
Hailey hailey disease: macerated plaque in axilla with linear
fissures.
Acrokeratosis Verruciformis of Hopf: skin coloured plane Epidermolysis bullosa simplex: intact blisters; healing with
topped papules over dorsum of left hand. hyperpigmented macules.
Epidermolysis bullosa simplex (Weber cockayne variant): Epidermolysis bullosa, junctional, non-lethal: atrophy, hemor-
Tense and flaccid bullae restricted to soles. rhagic bullae, loss of nails.
Genodermatoses 293
Histopathology
Hyperkeratosis, acanthosis and papillomatosis in
‘church spire’ pattern.
Treatment
Histopathology of benign familial chronic pemphigus: irregu-
Topical retinoids. Or ablation with radiofrequency,
lar intraepidermal clefts with acantholytic cells. Note dyskeratotic
lasers or cryotherapy. cells in upper layers.
Recessive dystrophic EB: ‘Mitten like’ appearance of toes. Epidermolysis bullosa, recessive dystrophic: mitten-like hands,
poor dentition and cicatricial alopecia.
Genodermatoses 295
Anhidrotic ectodermal dysplasia: typical facies with saddle- Neurofibromatosis: irregular, uniformly light brown, cafe-au-fait
shaped nose, thick everted-lips and peg-shaped teeth. macules.
Genodermatoses 297
Segmental NF
Mosaic localized NF1 better term. Usually
somatic mosaicism. Gonadal mosaicism possible.
Involvement of a part of body with CALMs and
neurofibromas.
Histopathology of neurofibroma: numerous spindal cell fasci-
Treatment
cles seen with nuclear buckling and wavy nerve fibers.
Multiplicity of lesions makes treatment difficult.
Excise large or unsightly tumors or ones
suspicious of a malignant change. Offer genetic
counseling. Surgical management of systemic
cafe-au-lait macules & schwannomatosis). One conditions likeoptic nerve tumors, kyphoscoliosis.
type chiefly skin related (NF1/von Recklinghausen’s Experimental treatments for plexiform NFs include
disease) and the other related to nervous system sirolimus, imatinib, pegylated interferon alpha etc.
(NF2).
Tuberous Sclerosis (Epiloia)
Etiology of NF1
Uncommon. Autosomal dominant inheritance.
Mutation of NF1 gene on chromosome 17, reduced Mutation in either TSC1 or TSC2 genes. Varied
production of neurofibromin protein. cutaneous and neurological manifestations. Skin
lesions: depigmented macules: oval, well-defined,
Clinical Manifestations depigmentation on the back or trunk—ash–leaf
Cutaneous neuromas, cafe-au-lait macules and macules may be the first manifestation.
freckling. Cutaneous neuromas (molluscum Angiofibromas (adenoma sebaceum, a
fibrosum): soft, skin or brown colored, dome- misnomer): small, firm, skin colored or pink
shaped, sessile or pedunculated cutaneous or papules or nodules present in the central part of
subcutaneous tumors. Vary in number and size. the face particularly in the naso-labial folds. Also
Plexiform neurofibromas—large, with a ‘worms-in- pale-yellow, or skin colored, slightly elevated,
a-bag’ feel. Asymptomatic. Cafe-au-lait macules: plaques—shagreen patches. Periungual fibromata
sharply defined, uniformly light brown macules —Koenen’s tumors—firm, skin colored ‘clove-like’
of varying sizes; margin irregular. Often the first projections from the nail folds.
manifestation of the disease; increase in number Neurological manifestations: variable—mental
and size during the first decade. Axillary freckling deficiency, epileptiform seizures; cortical
(Crowe sign) and palmar freckling (Premlata calcifications. Rarely renal, cardiac or pulmonary
Yesudian or ‘PY’ sign) are diagnostic. Neural hamartomas.
fibromas occur along the cranial or peripheral Course: unpredictable but not unfavorable except
nerves or in spinal cord. Acoustic and optic nerve in full blown cases.
Tuberous sclerosis: angiofibromas coalescing over the centro-
facial area to form of plaque.
Tuberous sclerosis: angiofibromas on the nose and cheeks. Tuberous sclerosis: collagenoma on the forehead(‘fibrous fore-
head plaque’).
Tuberous sclerosis, shagreen patch: skin colored grouped Tuberous sclerosis, periungual fibromas: fleshy papules and
papules and plaques over trunk. nodules arising from the nail folds producing guttering of the
nail plate.
Genodermatoses 301
Trichoepithelioma
Uncommon. Hamartoma of pilosebaceous
apparatus. Multiple familial trichoepitheliomas has
autosomal dominant inheritance. Gene for multiple
trichoepitheliomas mapped to chromosome 9.
Treatment
Electrofulguration or laser ablation. Symptomatic
treatment of seizures. Histopathology of trichoepithelioma: focal collection of basa-
loid cells; hairshaft like structures in the center.
Trichoepitheliomas: multiple skin colored to few yellowish pap- Steatocystoma multiplex: deep seated, pale yellow rounded
ules and nodules over centrofacial area. and oval nodules.
A B
C D
Brooke spiegler syndrome: composite image (A) scalp cylindromas; (B) forehead trichoepithelomas; (C) eccrine spiradenoma near
outer canthus; (D) parotid tumor.
Genodermatoses 303
Ehlers-Danlos syndrome: hyperextensible skin. Incontinentia pigmenti: vesicular and verrucous lesions, lower
limbs and trunk.
Genodermatoses 305
Histopathology
Histopathology of pseudoxanthoma elasticum: fragmented
elastic fibers (black fibers), mid dermis (Verhoeff-van Gieson
Intraepidermal spongiotic vesicle containing
stain, 10x). eosinophils in the vesicular stage. Verrucous phase:
hyperkeratosis and irregular acanthosis. Incontinence
of pigment in the pigmentary stage conspicuous.
Treatment
Chiefly directed at preventing and combating
vascular complications. Skin lesions only
cosmetically objectionable.
Ehlers-Danlos Syndrome
(Cutis Hyperelastica)
Rare. Genetic defects variable and not known in
many variants; mutations in collagens 1, 3 and
5, fibronectin etc. Several variants: autosomal
dominant. Or recessive. Or X-linked recessive
inheritance. Skin: soft, smooth, hyperextensible
(not lax atrophic scars, molluscoid pseudotumors, Histopathology of incontinentia pigmenti bullous stage:
Intra-epidermal spongiotic vesicles containing eosinophils.
easy bruisability etc.). Others: hypermobile joints,
fragile blood vessels, risk of bowel rupture, bony
deformities, kyphoscoliosis etc.
Course: severity and prognosis variable. Treatment
No treatment available, topical steroids and
PIGMENTARY DISORDERS tacrolimus may help reducing pruritus in vesicular/
verrucous stage.
Incontinentia Pigmenti
(Bloch-Sulzberger Syndrome) Linear and Whorled Nevoid Hypermelanosis
Rare. X-linked dominant inheritance. Lethal Disorder of chromosomal mosaicism.
in males, so patients always females barring Hyperpigmentation in linear and whorled
anecdotal cases of gonadal mosaicism. NEMO configuration over the trunk and limbs, sparing
(nuclear factor kappa B essential modulator) gene face, palms and soles. Extracutaneous features
mutation on X chromosome. Three independent include developmental and growth retardation,
or overlapping phases: vesiculobullous, verrucous body asymmetry. No treatment available.
Incontinentia pigmenti stage 3: Streaky whorled hyperpigmen- Linear and whorled hypermelanosis: Fine hyperpigmented
tation over trunk in a child. lines in blaschoid pattern over trunk.
Peutz Jegher syndrome: brown-black lentigines involving perio- Oculo-cutaneous albinism: tyrosinase negative type. Pink irides,
ral area, lip mucosa and fingers. white hair and actinic keratosis—a total lack of pigmentation.
Genodermatoses 307
Xeroderma pigmentosum: mottled hypo- and hyperpigmen- Xeroderma pigmentosum variant(pigmented xerodermoid):
tation on sun exposed areas, dry skin and multiple basal and mottled hypo- and hyperpigmentation over face and sun exposed
squamous cell carcinomas. areas of upper limbs.
Genodermatoses 309
POIKILODERMATOUS DISORDERS
Dyskeratosis Congenita
X-linked recessive. Mutant gene, DKC1, encodes
Griscelli syndrome: light microscopy of hair shows large clumps
protein dyskerin. Triad: reticulate or mottled of pigment distributed irregularly along the shaft.
pigmentation, nail dystrophy and premalignant
leukoplakia. Other features: palmo-plantar
hyperkeratosis and adermatoglyphia (absent
dermal ridges on fingers and toes). Progressive Histopathology
pancytopenia in most patients—principal cause of Enlarged hyperpigmented basal melanocytes with
mortality. sparse pigmentation of adjacent keratinocytes.
Bloom syndrome: erythematous/telangiectatic macules on face, Dyskeratosis congenita: reticulate pigmentation (poikiloderma),
prominent nose and malar hypoplasia. multiple nails dystrophic.
Dyskeratosis congenita: oral leucokeratosis. Griscelli syndrome: silvery grey hair, hypopigmentation and
normal eyes.
Genodermatoses 311
Treatment
Supportive: control infections and seizures.
Definitive: bone marrow transplantation. Palliative:
systemic steroids and immunosuppressives.
OTHER GENODERMATOSES
Monilethrix, hair mount: Hair shafts beaded at regular intervals
Monilethrix seen on naked eye examination itself.
Rare, autosomal dominant hair shaft disorder.
Mutation in genes encoding for human hair keratins
hHb1 and hHb6.
Hair shaft is beaded at regular intervals (nodes
and internodes), breaks easily at the internodes.
Variable clinical severity. Hair loss and breaking off
associated with follicular keratosis, mostly over the
nape of neck and occiput. Eyebrows, eyelashes,
axillary and pubic hair may also be affected.
Treatment
Spontaneous improvement known in some cases.
Oral retinoids gave relief, probably in follicular
Monilethrix, hair mount: Hair shaft showing nodes (swollen
keratosis. Topical minoxidil may give some relief. areas) and internodes (constricted areas) at regular intervals.
Elejalde syndrome: silver-grey hair, photophobia and photosen- Monilethrix: keratotic papules over nape of neck. Note: hair loss
sitivity giving bronze colored skin. involving the occipital scalp.
Acrodermatitis enteropathica: scaly psoriasiform plaques Acrodermatitis enteropathica: erosive eczematous plaques
involving perinasal, perioral and neck regions. involving genitalia and groins.
Genodermatoses 313
Hyper IgE syndrome: coarse facial features—wide nasal root, Fabry’s disease: red papules on abdomen, most concentrated
broad nasal tip, prominent forehead. Scaly papules (few excori- around umblicus. Inset shows similar lesions over lip mucosa and
ated) over face suggestive of atopic dermatitis. conjunctiva.
Genodermatoses 315
Infantile hemangioma, mixed type: superficial ulcerated Capillary malformation, port wine stain type: unilateral vascu-
hemangioma overlying a faint green swelling suggestive of deep lar malformation confined to trigeminal distribution. Patient also
component. had seizures (Sturge-Weber syndrome).
Nevoid Conditions 319
VASCULAR NEVI
Two types of lesions recognized: vascular
malformations and hemangiomas.
Blue rubber bleb nevus syndrome: deep blue nodule on trunk Maffucci syndrome: multiple blue nodules over the toes and
(tender on palpation). soles. Enchondroma involving the right second toe.
Nevoid Conditions 321
Lymphangioma circumscriptum, microcystic type: grouped Lymphangioma circumscriptum, microcystic type: grouped
vesicles, clear (‘frog spawn’ appearance) as well as hemorrhage. vesicules causing macroglossia.
Nevoid Conditions 323
Klippel-Trenaunay Syndrome
Classically, capillary malformation of the skin
with soft tissue and bony hypertrophy of the
affected limb, and varicose veins with or without
deep venous anomalies. Orthopedic consultation
needed for limb length discrepancy. Recurrent
thrombophlebitis and cellulitis may complicate the
picture. Risk of deep venous thrombosis also there.
Blue Rubber Bleb Syndrome
Mostly sporadic. Few reports of familial cases.
Cutaneous and gastrointestinal venous
malformations. Most characteristic are blue
compressible subcutaneous nodules on skin. Histopathology of angiokeratoma: dilated blood vessels in
Commonly on trunk and extremities. Gastrointestinal papillary and mid dermis.
malformations cause for morbidity. Whole GI tract
may be involved. Rectal occult bleeding or frank Angiokeratoma of Fordyce
blood loss from upper or lower GI tract. Symptoms Uncommon. Superficial ectatic blood vessels in
in childhood or adulthood. Malformation may superficial dermis. Elderly or adults. Deep red or
involve CNS, orbit or genitourinary tract. purple, 1–5 mm, vascular papules on the scrotum, and
infrequently on the glans penis. Usually asymptomatic.
Maffucci Syndrome Occasionally bleed. Associated varicocele.
Sporadic genetic disorder. Combination
of enchondromas and vascular anomalies Treatment
(superficial and deep venous malformations, Left alone or ablate with radiofrequency,
hemangioendotheliomas, etc). Blue to purple cryotherapy or laser.
soft nodules, may be tender. Usually on distal
extremities, may involve internal viscera. Lymphangioma Circumscriptum
Enchondromas involve phalanges and long bones. Uncommon. Present at birth or appears during
early childhood. Axillae, neck, upper trunk,
proximal parts of extremities. Or mucosa affected.
Multiple, closely-set, mulberry—like, clear or
hemorrhagic vesicular lesions. Underlying deep
seated subcutaneous lymphangioma. Associated
hemangiomatous component may be present.
Treatment
Superficial lesions: destroyed with electric
diathermy. Or radiofrequency ablation. Deep
component poorly delineated and thus not easy
Maffucci syndrome: X-ray both hands showing multiple areas of to treat, even surgically.
bony destruction and lucencies in phalangial bones, suggestive
of multiple enchondromas.
Phakomatosis pigmentovascularis type 2: combination of port Verrucous epidermal nevus: a linear verrucous lesion.
wine stain (right half of face) and bilateral nevus of Ota (cheeks,
nasal alae, periorbital and sclera).
Verrucous epidermal nevus: nevus unius lateris (unilateral, Verrucous epidermal nevus: systematized lesions.
widespread VEN).
Nevoid Conditions 325
Epidermal nevus syndrome: unilateral VEN involving the right Becker’s nevus: dark brown macules with ‘splashed’ appearance
forearm and right lower limb. Severe, deforming hypophos- and overlying hypertrichosis. (Typical site- upper chest and proxi-
phatemic rickets. mal arm).
Nevoid Conditions 327
Histopathology Histopathology
Two variants: epidermolytic and non-epidermolytic. Vertically alternating orth—and parakeratotic
Both show hyperkeratosis and acanthosis. Only hyperkeratosis. Varying picture—dermatitic,
minimal infiltrate indermis. Epidermolytic variant psoriasiform or lichenoid. Infiltrate conspicuous.
shows vacuolization of keratinocytes, begins
in granular layer and may extend to basal layer.
Within vacuolated keratinocytes, keratohyalin and
trichohyalin granules seen.
Syringocystadenoma papilliferum: solitary red glistening nod- Nevus comedonicus: linearly arranged large open comedones
ule over nevus sebaceous (scalp). over cheek arranged in linear fashion.
Nevoid Conditions 329
Histopathology
No nevus cells. Variable epidermal changes like
hyperkeratosis, mild acanthosis may be seen.
Increased basal pigmentation and few dermal
melanophages. Dermis may show thickened or Histopathology of nevus sebaceous: hypertrophic sebaceous
glands in upper dermis-increased size, close set lobules.
increased smooth muscle fibers, suggestive of,
smooth muscle hamartoma. Syringocystadenoma Papilliferum
Treatment Rare. Usually over an underlying sebaceous
nevus. Origin from apocrine gland, uncommonly
Several pigmentary and hair reduction laser tried
from eccrine gland. Most common on scalp.
with variable and modest response. Q switched
Solitary or linearly arranged pink-brown nodules,
ruby laser and Er:YAG lasers shown best response.
friable/glistening surface. Enlarge around puberty.
Nevus Sebaceous Rapid increase suggests basal cell carcinoma.
Sometimes called organoid nevus due to
Histopathology
hamartomatous involvement of epidermis,
pilosebaceous unit as well as other appendages. Multiple dilated ducts connecting epidermis to
Uncommon usually solitary. Well demarcated, dermal cysts. Latter lined by twin layers of cuboidal
yellow-brown hyperpigmented oval, circular or or columnar cells, containing papillary projections
irregular, plane topped to mildly verrucous, waxy and villi. The stroma shows moderately dense
plaque(s), most common on scalp but also occurs infiltration by plasma cells.
on face, chest. Scalp lesions have associated
alopecia. Enlarge around puberty. Prone to develop
benign tumors over time and rarely malignant.
Commonest benign tumors: syringocystadenoma
papilliferum and trichoblastoma. Others include
leiomyoma, syringoma, spiradenoma, hidradenoma
and keratoacanthoma. Malignant tumors may
develop: basal cell carcinoma, apocrine carcinoma,
squamous cell carcinoma and malignant eccrine
poromas. Occasional malignant transformation to
basal cell carcinoma.
Histopathology
Epidermis showing changes of hyperkeratosis,
acanthosis, papillomatosis. Sebaceous glands Histopathology of syringocystadenoma papilliferum: high
present higher up in dermis. Mature sebaceous power showing numerous villi in a cavity. Both villi and the cavity
lined by twin layer of cuboidal cells and stroma showing plasma
follicles—increased in number, increased size of cell infiltrate. Many cells lining cavity show apical pinching off,
lobules which are closely set, increased ducts. suggestive of apocrine differentiation.
Nevus comedonicus: unilateral lesions of grouped comedones Shagreen patch: grouped, skin colored papules and nodules
along lines of Blaschko. over trunk.
Buschke-Ollendorff syndrome: disseminated skin colored pap- Nevus lipomatosus cutaneous superficialis: grouped skin
ules and plaques on trunk of an infant. colored papules and nodules over the right buttock, increased
hair growth.
Nevoid Conditions 331
Giant melanocytic nevus on the face: dark brown to black with Nevoid cutis verticis gyrata: cerebriform folds of skin overlying
terminal hair. a melanocytic nevus.
Nevoid Conditions 333
Nevus spilus: irregular edged light brown pigmented patch around Nevus of Ota: slate-grey pigmentation of face and sclera.
left eye, studded with dark brown macules and few papules.
Nevoid Conditions 335
or papillomatous surface with terminal hair on top. pigmentation of the skin; (often superimposed)
Intradermal variant—skin colored nodule. conjunctiva (brown discoloration), sclera (slate
Malignant potential of melanocytic nevi variable. grey discoloration), and palate. Speckled or diffuse.
Generally unilateral and confined to trigeminal
Nevus Spilus area.
Rare. Since infancy or early childhood. Initial lesion
may be tan/light brown macule which increases Nevus Depigmentosus
in size and later many brown/black macules (Nevus Achromicus)
progressively develop over it. Conversely dark Not uncommon. Well-defined, depigmented or
macules may appear earlier. Rarely, these darker hypopigmented macule of irregular configuration.
macules may have hypertrichosis suggestive of On the trunk or elsewhere. Present at birth. Stable;
compound melanocytic nevi. no change in size.
Lichen striatus: tiny hypopigmented papules grouped in a fine Linear spiradenoma: linearly arranged pinkish nodules over
linear Blaschko’s distribution over upper chest. forearm.
Nevoid Conditions 337
distribution. Possible role of dysfunction of papules arranged in a linear manner. Often occur
sympathetic nerves. Clinically, lesions are either along lines of Blaschko. Hypopigmented papules
unilateral zosteriform or blaschkoid or dermatomal occur commonly in darker skin types. Usually
or multifocal yet maintaining the distribution asymptomatic, but may be itchy. Spontaneously
pattern. Variation in pigmentation more common subside in months to years. Subside with temporary
than in non-segmental forms—islands of pigment hypopigmentation.
inside the lesion, leucotrichia extending outside
the margins. Common sites include head, trunk Histopathology
and limbs. Common associations: family history, Variable. In active lesions, it may resemble lichen
atopic dermatitis, halo nevus. planus in form of band like dermal lymphoid
infiltrate. Associated focal basal layer degeneration,
Course acanthosis may be there. Infiltrate may go deeper
Static throughout after initial rapid spread. to concentrate around appendages.
Spontaneous repigmentation known but partial
or minimal. Rarely, evolves into generalized non- Treatment
segmental variants. Observation. Topical steroids for symptomatic
relief.
Treatment
Good prognosis in early stages. Topical steroids, Linear Spiradenoma
calcineurin inhibitors,PUVA/PUVA sol give relief Extremely uncommon variant of a the uncommon
to variable extent. Stable lesions best candidates tumor spiradenoma, which arises from sweat glands.
for vitiligo surgeries like epidermal suspensions, Only few reports exist in literature, few in relation with
suction blister grafting, etc. malignant degeneration (spiradenocarcinoma).
Lesions occur as skin colored or pinkish/greenish,
Lichen Striatus dermal or subcutaneous nodules arranged
Idiopathic disorder of unknown etiology. Usually linearly over a limb, more commonly on the lower
occurs during childhood, age of onset varying limbs. As with the commoner non-linear variants,
from infancy to 15 years. Possibly an eczematous pain is associated. And histopathology is also
disorder. Starts as erythematous or lichenoid, tiny similar. Complete excision curative. Ablation with
radiofrequency or laser may benefit.
18 Cutaneous
Tumors
Seborrheic keratoses: dark brown, ‘stuck on’ papules. Dermatosis papulosa nigra: dark brown, dome-shaped papules.
Acrochordon (Skin Tags): tiny pedunculated papules on side of Seborrheic keratoses, eruptive: on the trunk—Leser-Trélat sign.
neck.
Cutaneous Tumors 341
EPIDERMAL TUMORS
Treatment
Seborrheic Keratoses (SK) Shave off lesions—flush with the skin surface. Or
Common, almost normal in elderly individuals. radiofrequency ablation. Topical retinoids.
Both sexes affected. Well demarcated, light brown
to brown black, flat or finely verrucous papules. Keratoacanthoma
Typically ‘stuck on’ appearance with keratotic
plugs. Variable sized. Genetic predisposition Exact nosological status uncertain yet. Some think
present. Face, dorsa of the hands, trunk commonly it to be benign, some think it to be harbinger of
involved. Eruptive appearance of seborrheic SCC. Term abortive malignancy proposed.
keratoses Leser-Trelat sign should raise suspicion
of internal malignancy. Etiology
More common in whites, rare in pigmented
Acrochordon (Skin Tags) population. UV, HPV, immunosuppression, genetic.
Clinical and histological overlap with SK. Soft,
usually 1 to 2 mm pedunculated papules in flexural Clinical Presentation
sites—neck, axillae, inframammary. Predisposing Solitary or multiple; latter often familial. Usually
conditions—familial, type 2 diabetes, obesity, etc. solitary lesion with three phases—rapid growth
over few weeks, stable for few weeks, spontaneous
Dermatosis Papulosa Nigra resolution over few months. Lesions is dome
A variant of seborrheic keratosis. More common shaped/nodular with central firm keratin core.
in darker skin types. Younger adults. Bilateral, Usual sunexposed sites—face, forearms, hand
sessile, tiny 0.5 to 2 mm sized dark brown to brown dorsae, etc. Heal with hypopigmented scar.
black papules on the cheeks and temples.
Histopathology
Histopathology Central keratotic core covered with an atrophic
Sharply defined basaloid epidermal tumor, may be epidermis. Acanthotic epidermis, on either side.
exophytic or endophytic. Hyperkeratosis, follicular Well-differentiated cells with horn cysts. No atypia.
plugs and horn cysts are characteristic features. Heavy lymphohistiocytic infiltrate in the upper
Variable, often pronounced, acanthosis. dermis.
Keratoacanthoma: dome-shaped nodule with keratin filled Cutaneous horn : on plaque of genital lichen sclerosus with asso-
crater, forehead. ciated squamous cell carcinoma.
Syringomas
Uncommon. Young females more frequently
affected. Asymptomatic. Multiple, skin colored or
pale, flat angulated papules. Eyelids—upper, lower
or both-affected, often symmetrically.
Infrequently present on the trunk. May also
involve vulva, penis and flexors. Eruptive variant is
generalized.
Histopathology
Variably shaped and sized eccrine ducts lined
Histopathology of keratoacanthoma: keratin plug in the crater; by two layers of cuboidal epithelium. May have
acanthotic epidermis around; note horn cysts.
comma like tails resembling tadpoles. Solid nests
with basaloid appearance, embedded in sclerotic
stroma.
Treatment
Watchful waiting, complete surgical excision,
electrosurgery, localized radiotherapy,
intralesional chemotherapy.
Cutaneous Horn
Firm, hyperkeratotic, horn-shaped papule, height
at least 2 times the diameter. Clinical entity,
arising over a background pathology, rarely
idiopathic. Common causes include actinic
keratosis, seborrheic keratosis, verruca vulgaris
and squamous cell carcinoma. Uncommonly over
keratoacanthoma, basal cell carcinoma, epidermal
nevus and angiokeratoma. Rarely on penis.
Histopathology
Keratinous material which may be lamellated
or compact. Keratinization may be epidermal or Histopathology of syringoma: basaloid aggregates with tubu-
tricholemmal (without granular layer). Base may lar differentiation, solid nests and horn cysts in a sclerotic stroma.
reveal primary pathology. Epidermal hyperplasia
may occur, usually without atypia.
Treatment Treatment
Rule out underlying malignancy. Radiosurgical Results not statisfying. Electrosurgery,
ablation or surgical excision. dermabrasion, topical tretinoin, trichloroacetic acid.
Syringomas: skin colored, flat, angulated papules on the lower Trichoepithelioma: multiple skin colored to whitish, dome
eyelids. shaped papules over the nose tip and nasolabial fold.
Eccrine poroma: shiny red nodule (bifurcated by a thin sleeve of Eccrine hidrocystoma: multiple bluish papulo-vesicles over the
epidermis) over the palm. Also note the peripheral collarette of dorsum of nose, side of nose, cheek and nasolabial fold.
thick skin, closely resembling pyogenic granuloma.
Cutaneous Tumors 345
Trichoepithelioma Histopathology
Common hair follicle tumor with primitive follicular Thin twin cell-layer lined cysts—outer flat
differentiation. Multiple tumors occur in inherited myoepeithelial cells and inner cuboidal cells.
form. Solitary or few tumors occur sporadically.
Pearly white to skin colored, dome shaped papules Treatment
involving the centro-facial area—eyelids, nose, Topical applications usually do not help. Ablation
nasolabial folds, medial cheeks. using lasers, radiofrequency or excision.
Histopathology Pilomatricoma
Basaloid islands in dermis which may show Uncommon tumor possibly arising from hair matrix.
branching and peripheral palisading. Keratin cysts Common amongst hair follicle tumors. Usually
may be seen lined by basaloid aggregates. over head and neck, and upper extremities.
Solitary deep seated dermal/subcutaneous nodule
Treatment with overlying skin being normal. More than 80%
Ablation with radiofrequency, laser or lesions undergo secondary calcification.
trichloroacetic acid. Excision for larger lesions.
Eccrine Poroma
Uncommon tumors arising from the epidermal part
of the eccrine duct (acrosyringium). Common sites
include the palms and soles. Appear as red to pink
nodules with glazed/moist surface. May mimic
pyogenic granulomas.
Histopathology
Aggregates of cuboidal cells (paler, smaller than
keratinocytes) projecting as columns or chords
from the epidermis into the papillary dermis.
Treatment
Surgical excision.
Histopathology of pilomatricoma: compact aggregate of
Eccrine Hidrocystoma
eosinophilic cells with absent nuclei and smudged cell walls
Uncommon tumor arising from deformed eccrine (ghost cells). Note foreign body type giant cells at the periphery.
sweat glands leading to dilatation of sweat ducts.
Thought to be due to heat exposure due to the
Histopathology
sites of predilection and since they show seasonal
variation. Most common over cheeks and eyelids. Features depend on age of lesion. Well defined
More common in people exposed to heat (cooks, tumor islands with intervening soft tissue stroma.
housewives, etc.). More in summers, may show Two types of cells—compact basaloid aggregates
tendency to resolve spontaneously in winters. at the periphery of lesion or in early lesions;
Multiple 1–3 mm skin colored to bluish papules/ compact eosinophilic aggregates with smudged
papulo-vesicles. Fluid comes out on puncture. cellular outlines and loss of nuclei (mummification
Common differential apocrine hidrocystomas but or ‘ghost cells’). Mummification and calcification
the latter are solitary, bigger (size in centimeters), starts from center of lesions. Foreign body giant
more often bluish, deeper and on cheeks. cells in response to calcification.
Pilomatricoma: solitary skin colored nodule over the upper arm. Eccrine spiradenoma: solitary dermal nodule with reddish blue
Note the whitish hue is due to dystrophic calcification within the hue. Few milia are studded over the nodule.
tumor.
Cylindroma: multiple pinkish nodules and tumors over scalp Hidradenoma: tiny red nodule over forehead.
causing overlying hair loss.
Cutaneous Tumors 347
Treatment Histopathology
Complete surgical excision. ‘Jigsaw puzzle’ appearance on low power—multiple
tumor lobules in dermis which appear to mould/fit
Eccrine Spiradenoma into each other’s shape. Prominent hyaline pink
thickened basement membrane around tumor
Extremely uncommon tumor arising from sweat
nodules which comprise of two types of cells (dark
glands. Solitary, painful dermal nodules over
at the periphery and pale at the center), ductal
trunk and upper limbs. Overlap may occur with
differentiation may be seen.
cylindromas both clinically and histologically.
Histopathology
Well defined dermal nodules as islands and chords
of basaloid cells. Two types of cells—dark cells
(with hyperchromatic nuclei) present at periphery
of islands and pale staining nuceli containing cells
in the center. Many duct like structures seen in
the nodules and sometimes with cystic spaces
and vascular channels. Lymphocytic infiltration of
tumor lobules may be seen.
Treatment
Excision or ablation using radiofrequency or
lasers. Multiple or large tumors require excision
and grafting. Topical salicylic acid tried anecdotally
in multiple lesions.
Histopathology of eccrine spiradenoma: tumor nodule of
basaloid cells with numerous duct-like structures, dilated capil-
laries and some lymphocytic infiltration. Hidradenoma
Rare tumor of sweat gland origin. Now reclassified
Treatment as either having apocrine or poroid differentiation.
Surgical excision. Solitary slow growing nodule occurring over scalp,
face and trunk. More common in females.
Cylindroma
Uncommon tumor of uncertain origin. Frequently Histopathology
multiple, pink-red tumors or raised nodules over Lobulated tumor masses in dermis composed of
scalp causing hair loss, may cause discomfort due to two types of cells—darker elongated cells at the
large size. May be inherited in autosomal dominant periphery and light/clear cells towards the center.
manner (due to mutation in CYLD tumor suppressor Stroma vascular with ectatic vessels surrounded
gene) as part of Brooke Spiegler syndrome. by a sleeve of sclerotic collagen.
Keloid: after piercing of upper ear rim Keloid: red brown horizontal plaque on presternal area (classic
location).
Keloid: Massive tumor-like keloid over shoulder. Multiple leiomyomas: grouped firm brown nodules and plaques
in a segmental distribution over back.
Cutaneous Tumors 349
Treatment
Prevention better than cure. Post operative
intralesional (IL) steroid injections in patients
with keloidal tendency. Combination therapy
better than monotherapy. Intralesional steroids,
5 fluorouracil, bleomycin (tattooing), cryosurgery.
Histopathology of Hidradenoma: tumor mass comprised of Combination of surgical excison/laser ablation
darker cells at periphery and pale clear cells towards the center. with intralesional steroids/localized radiotherapy.
Stroma showing numerous dilated capillaries. Maintenance with compression therapy. Other
(Courtesy: Dr Sudheer Arava, Assistant Professor of Pathology, modalities with weaker evidence: topical
AIIMS, New Delhi)
imiquimod, silicone, onion products.
Treatment
Complete excision. Leiomyoma
Uncommon tumor of smooth muscles. Based on
DERMAL TUMORS origin, 3 types: piloleimyoma (from arrector pili),
angioleiomyoma (from venous walls) and genital
Keloid (from mammillary, dartos, vulvar muscles). Pilar
Common. Hyperplasia of fibrous tissue, history of most common, can be multiple and familial. Multiple
trauma may or may not be there. Constitutional leiomyomatosis has mutation in fumarate hydratase
Epidermal cyst: nodule with central greenish hue and punctum. Epidermal cyst: multiple whitesh nodules on scrotum.
Cutaneous Tumors 351
Histopathology
Cyst wall made of stratified squamous epithelium
with epidermal keratinization (keratin formation
through presence of granular layer). Cyst contains
laminated keratin. Older cyst may show fibrosis of
wall, calcification/cholesterol crystals in cavity.
Treatment
Surgical excision. Or laser ablation. Palliative
pain relieving therapies include calcium channel
blockers (nifedipine, amlodipine, etc), gabapentin,
nitroglycerin, phenoxybenzamine, intralesional
botulinum toxin, etc.
Lipoma
Common. Single or multiple. Freely movable Histopathology of epidermoid cyst: wall showing stratified
(slipping) subcutaneous masses of varying size. squamous epithelium with granular layer and lumen containing
Distributed over the neck, arms, shoulders, back. laminated keratin.
Trichilemmal cyst: solitary well-defined nodule over scalp (with Milia en plaque: grouped milia over an inflamed plaque behind
overlying hair shaved) the ear
Secondary milia: in a case of epidermolysis bullosa acquisita Cherry angioma: over chest
Cutaneous Tumors 353
Treatment Milia
Mostly left alone. If symptomatic or disfiguring, Tiny epidermal cysts, 1 to 2 mm dome-shaped white
complete elliptical excision including overlying papules. May be congenital/inherited or acquired.
skin with punctum. Recurrence common if wall Scattered or grouped. Primary or secondary
portion left. Some authors tried aspiration followed (following healing of dermatoses or trauma).
by intralesional sclerosant injection with success. Secondary causes include epidermolysis bullosa,
porphyrias, linear IgA disease, etc. Common sites
Trichilemmal Cyst (Pilar Cyst) of primary lesions cheeks, eyelids. Variants include
eruptive milia, milia en plaque (common behind
Probable origin from isthmic region of follicle. Firm,
ears), etc.
well circumscribed nodules predominantly present
on scalp. Majority have multiple lesions. Usually
Histopathology
asympatomatic, occasionally secondary infection
or rupture occurs. Proliferating trichilemmal cyst Identical to epidermal cysts.
may mimic malignancy.
Treatment
Histopathology Congenital subside on their own, acquired
Lining composed of startified squamous epithelium removed by needle/blade/electrodessication.
without granular layer. Plump, pale columnar
keratinocytes at inner lining have interdigitating BENIGN VASCULAR TUMORS
edge with the compact eosinophilic keratin filling
the lumen. Cherry Angiomas
(Campbell de Morgan Spots)
Common. Benign. Innocuous. Elderly or middle
aged adults. Pinhead to slightly larger nodules.
Verrucous surface. Purple color. May persist or
resolve.
Treatment Treatment
Complete surgical excision. Best left alone.
Pyogenic granuloma: solitary bright red moist pedunculated Pyogenic granuloma: a pedunculated vascular lesion on scalp.
papule (side of neck) with acanthotic skin collar at base Has a collarette at base.
Bowen’s disease: psoriasiform plaque with atrophy. Note reni- Bowen’s disease: crusted inflamed plaque with renifrom borders.
form notch.
Cutaneous Tumors 355
Histopathology Histopathology
Lobular aggregate of proliferating capillaries, more Hyperkeratosis, parakeratosis, acanthosis with
compact in deeper dermis and loose in upper broad rete ridges. Full thickness dysplasia
dermis. Thin slit like spaces in place of lumina when (disorganization of epidermal cells). Characteristic
compact. Thinned out epidermis and margins of dyskeratotic and vacuolated cells. Basement
lesion show collarette of epidermis in the form of membrane intact. Dermis shows heavy
elongated rete ridges or sweat ducts. inflammatory infiltrate.
Treatment
Electrocauterization or surgical excision.
Recurrence likely if deeper portion left.
Actinic keratosis: rough, scaly, keratotic papules over dorsum of Arsenical keratosis: punctate keratosis of palms.
hand in an albino man.
Cutaneous Tumors 357
Treatment
Stopping further exposure. Systemic retinoids
for prevention of malignancies. Specific lesions
treated with topical 5 FU or ablative or surgical
therapies.
PUVA Keratosis
Chronic exposure to PUVA. Uncertain etiology but
possibly to UVA induced mutation in p53 or H-ras.
Histopathology of erythroplasia of Queyrat: bowenoid picture.
Keratotic papules and plaques at sites of UVA
exposure. Malignant transformation into SCC may
Actinic Keratoses occur. Virtually unknown in Indian skin.
Sun induced. Common in whites, particularly
blue eyed, red haired elderly individuals. Also in Lentigo Maligna (Melanotic Freckle of
patients with xeroderma pigmentosum or albinism. Hutchinson)
Irregular, keratotic papules with surrounding Uncommon. Elderly individuals affected. Light
erythema on the face, dorsa of hands or other light exposed areas, most frequently the face. Solitary
exposed parts. large, pigmented macule with irregular margins.
Arsenical keratosis and cutaneous: horns over the natal cleft in PUVA keratosis: yellowish keratotic papule over vitiligo lesion
chronic arsenic poisoning. chronically exposed to PUVA.
Lentigo maligna: flat, irregular dark brown macule. Bowenoid papulosis: pink barely elevated papules over glans
penis and coronal sulcus.
Cutaneous Tumors 359
Histopathology
Nests of atypical melanocytes at the dermo-
epidermal interface. Inflammatory infiltrate in the
dermis.
Treatment
Surgical excision if feasible. Electrodessication or
other destructive measures in carefully selected
patients. Imiquimod 5% cream also effective.
Alternatively careful follow-up for development of
any suspicious change.
Bowenoid Papulosis
Histopathology of Paget’s disease: typical Paget’s cells (large
Pigmented or pinkish plane topped or verrucous round cells with large nucleus and vacuolated cytoplasm),
papules involving the genitalia and showing arranged lalong the basal epidermal layer and focally scattered
changes of carcinoma in situ (Bowen’s) on [CK 20 stain].
histopathology. Commonly caused by high risk Courtesy: Dr Sarita Singh, Gynecologist, Safdarjung Hospital,
human papilloma viruses types 16 and 18. Delhi and Dr Meeta Singh, Assistant Professor of Pathology,
MAMC, Delhi.
Superficial basal cell carcinoma: irregulalry arcuate plaque with Pigmented basal cell carcinoma: pigmented scaly plaques over
thin edge and scaly papule at the margins. forehead with atrophic center.
Cutaneous Tumors 361
Histopathology
Characteristic multilobular mass of basaloid cells
(cuboidal cells with deeply basophilic nuclei)
lying in the dermis. May maintain continuity with
the epidermis. Peripheral cells palisade with
clear space between the tumor mass and the
surrounding fibrotic stroma (artefactual clefting).
Much present in the stroma. Some differentiation
towards adnexal structures. Tumor cells may
contain melanin or dendritic melanocytes.
Histopathology of pigmented basal cell carcinoma: melanin
and dendritic melanocytes (note fine dndritic processes) within
a basaloid cell tumor aggregate.
Treatment
Several modalities available. Choice depends
on clinical type, size of tumor and site involved.
Moh’s micrographic surgery ideal. Standard
treatment is excision with 2–4 mm free margin.
About 1 cm margin required for tumors >2 cm
in size. Other modalities include curettage and
electrodessication, cryosurgery, radiotherapy,
topical 5 fluorouracil, topical photodynamic
therapy. Imiquimod, 5% cream in superficial type
gives cure with excellent esthetic results. Recently
Histopathology of superficial basal cell carcinoma: basaloid vismodegib, a hedgehog pathway inhibitor, FDA
cell nests extending in form of bulbous protrusion from the epi- approved for metastatic basal cell carcinoma.
dermis into the papillary dermis with artefactual clefting.
Nodulo-ulcerative basal cell carcinoma (rodent ulcer): plaque Morpheaform basal cell carcinoma: small crusted pigmented
destryoing the ear helix. papule in the nasolabial fold with a large area of scarring and
depigmentation around it.
Squamous cell carcinoma: large verrucous ulcerated plaque Marjolin ulcer (squamous cell carcinoma in scar): indurated
over thigh. non-healing ulcer on a chronic burn scar.
Cutaneous Tumors 363
Malignant melanoma: in transit metastasis from a foot lesion Malignant melanoma, acral lentiginous type: ulcerated nodule
with lymph node mass in right inguinal region. in web space.
Cutaneous Tumors 365
Mycosis fungoides, patch/plaque stage: few moist eczema- Mycosis fungoides, tumor d’ emblee: multiple nodules and
tous plaques over background of patches and post inflammatory tumors developing on trunk and neck, de novo.
hyperpigmentation.
Cutaneous Tumors 367
Sezary syndrome: infiltrated skin, forearm, nodule in the elbow. Langerhans’ cell histiocytosis: papules, many purpuric, distrib-
uted in seborrhiec distribution. Abdominal distension due to
hepatosplenomegaly.
Cutaneous Tumors 369
Treatment of CTCL
Rosai Dorfman syndrome: yellow to erythematous papules, Generalized eruptive histiocytosis: tiny erythematous papules
nodules and plaques in genital area. scattered over face.
Cutaneous Tumors 371
Mastocytosis: irregular dark brown pigmented macules. Darier sign: erythema and urtication in a patient of urticaria
pigmentosa.
Cutaneous Tumors 373
Histopathology
Dense dermal monomorphous histiocytic infiltrate
with foam cells; Touton giant cells and foreign body
giant cells typical.
Treatment
Reassurance and regular ophthalmological
examination. Ocular and visceral involvement:
treat with oral steroids.
Diffuse cutaneous mastocytosis, pseudoxanthomatous type: Metastatic tumor: stony hard nodules on lower abdomen; pri-
diffuse facial infiltration along with waxy yellow plaques and mary tumor in the breast.
nodules.
Cutaneous Tumors 375
Treatment Histopathology
Juvenile xanthogranulomas and Rosai Demonstration of mast cells—‘Fried egg’
Dorfman self limiting. For others, treatment appearance round central nucleus and surrounding
usually unsuccessful. Systemic steroids and amphophilic granular cytoplasm. Number of cells
immunosupressants (methotrexate, azathioprine, dependent on subtype. Upper dermal infiltrate in
chlorambucil, cyclophosphamide, melphalan) urticaria pigmentosa and sheets of mast cells in
used with variable response. Destructive mastocytoma and diffuse cutaneous variants. More
therapies for cosmetic relief. compact and deeper aggregates in mastocytoma
as compared to diffuse cutaneous variant. Toludine
Mastocytosis blue stain and CD117 immunostains for mast cells.
Uncommon proliferative disorder of mast cells.
Generally benign. Onset, infancy or early childhood.
Generalized cutaneous (urticaria pigmentosa),
diffuse cutaneous or systemic forms. Occasionally
solitary tumor mastocytoma. Rarely malignant.
Urticaria Pigmentosa
Commonest form. Onset: infancy or childhood.
Yellowish brown or brown-black illdefined macules
or plaques that urticate easily Darier's sign. Bullous
lesions in some. Self limiting. Pigment fades
around puberty. Diffuse cutaneous form: diffuse
Histopathology of mastocytosis: sheets of mast cells in diffuse
infiltration of the skin. Yellowish discoloration, cutaneous variant, staining strongly with toludine blue.
doughy feel. May have associated systemic
involvement: hepatosplenomegaly, osseous Treatment
lesions, hematological abnormalities.
Frequently no treatment required. Oral
Histopathology antihistaminics for symptomatic relief. For
persistent lesions: topical/intralesional steroids,
Biopsied with care to avoid degranulation. Sheets of calcineurin inhibitors, excision of mastocytomas,
mast cells in dermis which stain metachromatically oral psoralen plus ultraviolet therapy, orals
with toluidine blue or Giemsa stain. steroids and mast cell stabilizers. Avoidance of
vigorous exertion/emotions, food items like raw
Treatment
fish/peanut/cheese/alcohol/hot beverages, etc.
Excise solitary tumors. For generalized or diffuse
cutaneous forms, use antihistamines combined
METASTATIC MALIGNANT TUMORS
with H2 antagonists. PUVA helpful in some
patients. Systemic steroids, if bullous lesions. Uncommon. Single or multiple. Rather late
lymphogenous or hematogenous spread. Primary
Diffuse Cutaneous Mastocytosis site variable: breast, prostate, lungs, kidney,
Exclusively seen in infants. Thick skin with peau-d- stomach, intestine, liver, bone. Or cutaneous
orange appearance, yellowish brown discoloration. malignant melanoma. Commonly localized to
Hemorrhagic bullae develop in early phase, diffuse scalp, trunk. Asymptomatic. Skin colored or
skin thickening persists. erythematous, firm nodules or plaques.
Mastocytomas Course
Usually in infants, over extremities. More commonly Generally unfavorable. Occasional cure on removal
solitary. of primary and metastasis.
19 Sexually
Transmitted
Diseases
Primary syphilis: solitary indurated ulcer. Primary syphilis: indurated, ulcerated, clean ulcer.
Secondary syphilis: lichenoid papules and plaques. Secondary syphilis: nodular lesion. Rare symmetrical pattern.
Sexually Transmitted Diseases 379
Infective disorders, in which sexual transmission is Divided into early and late syphilis. Or primary,
epidemiologically important. The old term venereal secondary, latent and tertiary stages. Early syphilis
diseases (VD) included syphilis, gonorrhea, comprises primary, secondary and early (<1 year)
chancroid, donovanosis and lymphogranuloma latent stages. Late syphilis, late latent (>1 year)
venereum (LGV)—the infamous five! The scope and tertiary stages. Various stages, overlapping
enlarged to include others such as herpes genitalis, and arbitrary.
genital molluscum contagiosum, anogenital warts,
genital candidiasis, hepatitis B, pediculosis pubis, Primary Syphilis
scabies and the latest and most dreaded of them The first manifestation, the chancre: the lesion at the
all—acquired immunodeficiency syndrome (AIDS). point of entry of organisms. Solitary, asymptomatic,
Laxity in sexual standards, permissiveness non-tender, indurated, erosive papulo-nodule or a
of the society in general, easy access to sex, shallow ulcer—moist, smooth, non-bleeding with
economic independence and breakdown of family sharply defined border. Genital, extragenital or
structure have all helped promote the spread perianal location. Glans or shaft of penis, prepuce,
of sexually transmitted diseases (STDs). Males coronal sulcus, cervix, clitoris, vagina and vulva—
report more frequently and earlier. Females, may frequently affected. Extragenital sites, lip, areola of
be asymptomatic or have lesions on concealed breast, finger. Self healing—with a hyperpigmented
genital parts. Heterosexual or homosexual macule or a superficial scar within 3–6 weeks.
contact—genital, anogenital or orogenital—causal. Unilateral or bilateral regional lymphadenopathy:
small, rubbery, discrete, firm.
SYPHILIS
The classical sexually transmitted disease. Secondary Syphilis
Ancient, fascinating, capricious. Causative Systemic disease. Spirochetemia present.
organism—Treponema pallidum—subsp. pallidum Develops 6 weeks to 6 months after onset of
a spirochete, 5–15 µ 0.1–0.2 µ. Characteristic primary syphilis—may overlap with healing primary
undulating to-and-fro and bending movements sore. Generalized, bilateral, symmetrical rash—
with little translational motion. Unable to grow on macular, papular, papulosquamous or psoriasiform.
artificial media. Grown in rabbit testicles. And nodular (often asymmetrical). Never vesicular.
Incubation period—long and variable: 9–90 Asymptomatic. Mucocutaneous lesions, moist
days, generally 3–5 weeks. Estimated rate of papules. Intertriginious, perianal and genital lesions
transmission from infected partner for a single often florid and vegetative—condylomata lata.
sexual exposure about 30%. Mucosal and mucocutaneous lesions, particularly
infectious—buccal, labial, faucial, tonsillar.
Generalized lymphadenopathy—painless, discrete,
non-tender. ‘Moth-eaten’ alopecia over scalp with
or without telogen effluvium. Systemic symptoms—
malaise, fever, bone pains, headaches. Treponemal
and non-treponemal serological tests for syphilis
invariably positive. Secondary stage lasts 1–3
months. Relapses infrequent—generally within
6 months; not beyond 2 years. Transplacental
transmission to fetus significant.
Latent Syphilis
No clinical or laboratory (radiological and CSF
Treponema pallidum: delicate spirochetes under dark ground examination included) evidence of syphilis, except
microscopy. a positive blood serology.
Secondary syphilis: pigmented palmar scaly macules. Secondary syphilis: characteristic ‘split papules’.
Secondary syphilis: erosive mucosal lesions. JH reaction to penicillin in secondary syphilis: bright red plaques
over sole at baseline (top) and after (bottom) oral steroid therapy.
Sexually Transmitted Diseases 381
Arbitrarily divided into potentially infectious: Early syphilis: benzathine penicillin, total of
early (less than 1 year) or late (more than 1 year) 2.4 million IU—1.2 in each buttock. Or procaine
post infection stages. benzyl penicillin, 1.2 million IU, once daily for
10 days.
Tertiary Syphilis Late syphilis: 2.4 million IU of benzathine
Classified as benign: involves the skin, penicillin weekly for 3 successive weeks. For neuro-
subcutaneous tissue, bones or joints. Or neuro- syphilis, aqueous benzyl penicillin, 3–4 million IU,
syphilis. Or cardiovascular syphilis (indicating intravenously (IV), every 4 hours for 14 days.
respectively involvement of the nervous or cardio- Congenital syphilis: treat syphilitic mother at
vascular systems). Develop 5, 10 or 20 years after earliest to prevent congenital syphilis. Procaine
infection, respectively. penicillin 50,000 IU/kg daily for 10 days or aqueous
Characteristic lesion, gumma—a granulomatous benzyl penicillin 1 lakh IU/kg in two divided
lesion rich in lymphocytes and plasma cells and doses, daily for 10 days. The infant may be treated
associated with endarteritis. Treponemes not with a single dose of benzathine penicillin G
demonstrable. Serological tests for syphilis almost 50,000 IU/kg single dose IM of the baby is normal
always positive in blood. clinically, with a non-treponemal titer <4 fold of
Mucocutaneous gummata: asymptomatic mother’s titer and the mother has been treated
nodules or plaques. Asymmetrical. Arranged in adequately with penicillin regimen for syphilis
polycyclic and arcuate configuration; variable in during pregnancy.
size and number. Break down into indolent ulcers— In penicillin sensitive individuals: tetracycline or
heal with atrophic scars. Mucosal involvement erythromycin, a total dose of 30 g (2 g a day for
common—gumma of palatal bone and overlying 15 days) or doxycycline 100 mg twice a day for
mucosa-perforation of the hard palate; ‘eating’ 15 days for early and late benign syphilis. For
away of uvula or depression of bridge of the nose. neurosyphilis 60 g (2 g per day for 30 days).
Tetracyclines should not be used in pregnant
Diagnosis women or in children under 8 years.
Primary and secondary stages, diagnosed most Jarisch-Herxheimer reaction: develops within a
specifically by demonstration of Treponema few hours of administration of penicillin or other
pallidum on dark ground microscopy from ulcer treponemicidal drugs. More common in early
transudate or lymph node aspirate. If negative, syphilis, more serious in late. Fever, malaise, body
syphilitic serology along with clinical features aches. Also exacerbation of syphilitic lesions.
of presumptive value. Latent syphilis shows Useful as presumptive evidence of syphilis.
serological positivity in blood alone (no other
evidence of syphilis). GONORRHEA
Late syphilis, by quasi-specific histology of
syphilis together with positive serology in blood Extremely common. Almost always sexual
(and CSF in neurosyphilis). transmission, rarely accidental. Caused by
Neisseria gonorrhoea—an intracellular Gram
Treatment negative diplococcus. Incubation period 2–7 days,
occasionally less or more. Males present with acute
Penicillin, always the drug of choice. 0.1 μg/mL
urethritis, females asymptomatic. Or with cervicitis.
effective treponemicidal serum levels. Parenteral
Acute urethritis: copious, purulent or
route preferred because of compliance and
mucopurulent discharge with dysuria and burning
consistent absorption. To be administered after
micturition in males. Some males and most females
intradermal sensitivity testing before each dose.
asymptomatic.
Condylomata lata: flat papules and plaques, vulva. Frankly purulent urethral discharge in gonococcal urethritis.
NON-GONOCOCCAL URETHRITIS
Extremely common; commoner in the West,
particularly in Whites. Basically, urethritis (in males)
that is not gonococcal. Causative organisms:
Chlamydia trachomatis, Mycoplasma genitalium; less Histopathology of Neisseria gonorrhoea: Gram negative pre-
dominantly intracellular ocucci in purulent urethral discharge.
important Ureaplasma urealyticum, and Trichomonas
vaginalis. Incubation period: 2–3 weeks. Mild dysuria
and mucopurulent discharge. Females: symptomatic
sterile pyuria (urethral syndrome). Reiter’s syndrome,
a serious but rare complication.
Diagnosis: essential prerequisite demonstration
of polymorphonuclear leukocytes and absence of
N. gonorrhoeae in the urethral smear. Culture and
immunoconfirmation of antigen.
Treatment
Doxycycline,100 mg orally, twice daily for 7 days or
azithromycin 1 g orally, single dose. Erythromycin
base/stearate, 500 mg orally, 4 times a day for
7 days in pregnant females. Treat sexual partner(s).
Condom, a good prophylactic measure against
Histopathology of Chlamydia trachomatis: inclusion bodies
gonococcal and non-gonococcal urethritis. stained with iodine.
Chancroid, phagedenic ulcer: most of the shaft of penis Chancroid: with bubo. Inflammatory inguinal lymphadenopathy.
destroyed.
Donovanosis: clean shiny beefy red lesions with a pearly border. Donovanosis: multiple, clean fleshy lesions.
Sexually Transmitted Diseases 385
CHANCROID
Common in tropics and subtropics. Bacterial
infection; autoinoculable. Causative organism:
Haemophilus ducreyi, Gram-negative bacillus.
Histopathology of donovanosis: Giemsa stained crushed tissue
Characteristic chain formation. Uncircumcised preparation showing intracytoplasmic and extracellular bipolar
males with poor personal hygiene more frequently (safety pin appearance) staining Calymmatobacterium granulo-
affected. Incubation period: 2–6 days. Multiple, matis (Donovan bodies); top (10x), bottom (40x)
superficial, dirty sloughed ulcers with ragged
margins; bleed easily. Coronal sulcus, prepuce,
shaft and glans penis affected. Secondary
infection with fusospirochetal organisms results Treatment
in rapidly destructive phagedenic ulcer. Inguinal Azithromycin, 1 g orally, single dose. Or ceftriaxone
lymphadenitis, 1–2 weeks later; unilateral, painful, 250 mg, IM single dose. Or ciprofloxacin 500 mg,
fluctuant; may rupture. twice a day for 3 days. Sexual partners should
Diagnosis: difficult to confirm. Demonstration of also be treated to avoid relapses. Fluctuant
H. ducreyi in a Gram stained smear—bacilli arranged lymph nodes should be aspirated, through non-
in ‘a school of fish’ configuration, not specific or dependent normal skin.
sensitive. Culture of fastidious bacillus difficult.
Lymphogranuloma venereum: typical groove sign due to Mixed infection: lymphogranuloma venereum and syphilis.
enlargement of femoral and inguinal lymph nodes.
Vesicular primary herpes genitalis in a male. Primary ulcerative herpes genitalis in a female.
Sexually Transmitted Diseases 387
Condyloma acuminata: blocking the introitus in a HIV negative Umblicated pearly white papules of molluscum over inner thighs
female. and perineum.
Sexually Transmitted Diseases 389
twice daily or valacyclovir 500 mg once daily period of 12–16 weeks. Podophyllin 25% solution
for at least one year. In immunocompromised— in benzoin tincture (more suitable for warts on
acyclovir 400–800 mg twice daily. Or famciclovir moist partially keratinized areas), physician
250 mg twice daily. Or valacyclovir 500 mg twice applied strictly to the lesion with the surrounding
daily. skin protected by petrolatum. Wash off after 2–4
For neonates and HIV-coinfection: acyclovir hours; repeat once a week. Podophyllotoxin,
5 mg/kg, IV 8 hourly for 7–10 days. a patient—applied alternative (currently not
available in market). If no response in 4–6
GENITAL WARTS weeks, treat with cryotherapy, electric cautery
or carbon dioxide laser. Intralesional interferon
Common, amongst sexually active population. therapy has variable efficacy. Other therapies
Caused by human papilloma virus (HPV) particularly include topical cidofovir, sinecatechins (green
HPV 6 and HPV 11. Polymorphic. Several clinical tea extract), topical trichloro/bichloroacetic acid,
types including papular, common wart, condyloma cryotherapy. Intralesional immunotherapy with
acuminata and plane wart. Condyloma acuminata Mw (new name Mycobacterium indicus pranii)
term restricted to cauliflower-like warts on moist vaccine quite effective. First dose is immunizing
surfaces. Coronal sulcus, frenulum, prepucial inner and given intradermally in shoulder, then
surface or urethral meatus, perianal region. And 2 weekly intralesional injections given till resolution
vaginal wall and vulva generally affected. Giant [usually after 3–4 intralesional (I/L) injections].
condylomas during pregnancy may interfere with
vaginal delivery. Giant and cervical condylomata
may be coinfected with high risk HPVs (like 16,
GENITAL MOLLUSCUM CONTAGIOSUM
18, 33, etc.), prone to malignant transformation. Incubation period 2–3 months. By skin to skin
Common verruca vulgaris type present on the non- contact during sex. Pearly white umblicated
moist sites—shaft of penis, vulva. monomorphic papules involving pubic/inguinal
area, inner thighs, vulva, scrotum (more than penis).
Treatment May resolve spontaneously or get secondarily
Imiquimod 5% cream treatment of choice. infected or eczematized. Treated by extirpation/
Applied overnight on every alternate day for a curettage, radiofrequency, chemical cautery (TCA/
BCA, silver nitrate), topical retinoic acid.
20 HIV Infection and
Acquired
Immunodeficiency
Syndrome
Course and stages of HIV infection.
Rash of acute retroviral syndrome: a maculopapular rash is Persistent generalized lymphadenopathy in an HIV-positive
seen in 70% of patients, but is often disregarded. patient: enlarged inguinal lymph nodes in a clinically otherwise
asymptomatic but serologically HIV-positive patient.
HIV Infection and Acquired Immunodeficiency Syndrome 393
EPIDEMIOLOGY
Geographic Distribution
zzGlobally,number of people living with HIV and
AIDS (PLHA) variable.
zzPrevalence of HIV/AIDS in high risk goups
higher.
Number of PLHAs and prevalence of HIV infection
PLHA in millions Prevalence (%)
Global1 34.0 (2011) 0.8
India2 2.08 (2011) 0.29 Structure of HIV.
Males having sex with males (MSM) 7.4% Pol: coding for reverse transcriptase (RT),
Herpes zoster in an HIV-positive patient: multidermatomal and Molluscum contagiosum in an HIV-positive patient: multiple
bilateral involvement with dissemination. Such widespread distri- lesions, some large. Multiple lesions occur when CD4 counts
bution suggestive of HIV-positive state. Herpes zoster often seen < 250/mm3.
in early symptomatic HIV infection.
HIV Infection and Acquired Immunodeficiency Syndrome 395
Oral hairy leukoplakia in an HIV-positive patient: due to Pyogenic infections in an HIV-positive patient: cutaneous
Epstein Barr virus. Manifests as asymptomatic, white plaques with pyogenic (especially Staphylococcus aureus) infections common.
characteristic vertical corrugations on lateral aspect of tongue. Sometimes resistant to treatment.
HIV Infection and Acquired Immunodeficiency Syndrome 397
zzViral replication: integrated viral DNA may lie cells. Antibodies against HIV absent (patient
dormant (in latent HIV infection). Or replicate seronegative, albeit falsely) but highly infectious
(as infection progresses). During viral replication (window period).6
integrated DNA provirus transcribed into zzAfter 3–12 weeks, patient mounts serological
mRNA. May be spliced (into smaller pieces) or response and viral load decreases.
remain unspliced. Both move into cytoplasm → zzClinical manifestations depend on effects on
translated proteins. immune system:
¾¾Spliced viral RNA → translated into regulatory ¾¾In early period of immune destruction, clinical
proteins Tat and Rev. latency, i.e. no clinical symptoms, but serological
¾¾Unspliced RNA → translated into structural positivity, viral load low and CD4+ counts normal.
proteins Gag and Env which help in packag- ¾¾Later, as immunosuppression progresses,
ing of new virus particles. patient becomes symptomatic. Viral load
zzAssembly and release: occurs on plasma increases and CD4+ count decreases.
membrane of host cell.
¾¾Components of HIV move towards plasma CLINICAL MANIFESTATIONS
membrane. And virion buds develop from
host cell.
Course
¾¾Maturation of virion occurs either in forming zzPrimary HIV infection at the end of which
buds or in the immature virion after it buds off acute retroviral syndrome occurs (indicating
from the host cell. seroconversion) 2–4 weeks after infection→ 2–10
¾¾During maturation, HIV proteases cleave poly- years of asymptomatic period → symptomatic
proteins into individual functional HIV proteins phases (early, late and advanced) → death in 1–3
(a step inhibited by protease inhibitors). years (without ART).
¾¾On maturation, virion able to infect another cell.
Stages of Disease
Host’s Response zzAcute retroviral syndrome (ARS):
zzInitially,though some virions are killed, HIV ¾¾Incidence: up to 50–90% develop ARS.
continues to multiply rapidly, infecting more CD4 Commoner if infection sexually acquired.
Relationship of HIV load (red), HIV antibodies (green) and CD4 counts (blue) as HIV
infection progresses.
6
Window period: Variable; shorter for transfusion acquired infection. Diagnosis of HIV infection during window period by detection of
p24 antigen. Or using nucleic acid-based assays.
Bacillary angiomatosis in an HIV-positive patient: multiple Candidiasis in an HIV-positive patient: most common cutane-
(sometimes solitary) pyogenic granuloma-like nodules. Local ous infection. Often severe, extensive and indicative of disease
lymphadenopathy frequent. progression.
Oral candidiasis in an HIV-positive patient: extensive, recurrent Dermatophyte infection in an HIV-positive patient: tinea corpo-
oropharyngeal infections. Associated dysphagia a clue to esopha- ris, multiple extensive, recurrent lesions.
geal involvement, an AIDS defining criteria.
HIV Infection and Acquired Immunodeficiency Syndrome 399
Cryptococcosis in an HIV-positive patient: multiple skin colored Histoplasmosis: well-defined erythematous scaly nodule which
papules with umbilication, resembling molluscum contagiosum on biopsy revealed histoplasmosis.
on the face. Confirmed on biopsy.
HIV Infection and Acquired Immunodeficiency Syndrome 401
Bacterial infections
zzPyogenic infections: cutaneous and systemic
pyogenic (especially Staphylococcus aureus)
infections common. Sometimes resistant to
treatment.
zzTuberculosis: (And ? leprosy) get reactivated.
zzBacillary angiomatosis: Bartonella henselae
infection. Typical pyogenic granuloma-like Potassium hydroxide mount: from an HIV-positive patient
papules and nodules. Or Kaposi’s sarcoma-like showing multiple mites.
Histoplasmosis in an HIV-positive patient: multiple crusted Scabies in an HIV-positive patient: may manifest as classic disease.
nodules and plaques. Diagnosis often made histolopathologically. Or as crusted (Norwegian) scabies presenting as hyperkeratotic,
crusted plaques or as erythroderma. Often not diagnosed clinically.
Crusted scabies in an HIV-positive patient: presenting as hyper- Insect bite hypersensitivity in an HIV-positive patient: excori-
keratotic, crusted plaques. Often diagnosed on scraping or biopsy. ated papules on exposed parts-extremities and face. Presence of
such lesions in adults warrants HIV testing.
HIV Infection and Acquired Immunodeficiency Syndrome 403
Eosinophilic folliculitis in an HIV-positive patient: severely Eosinophilic folliculitis in an HIV-positive patient: severely
itchy, chronic follicular papules and pustules on the face. itchy, chronic follicular papules and pustules in neck, upper trunk
Eosinophilic folliculitis in the spectrum of pruritic papular erup- and face. Treated with topical steroids and phototherapy.
tion of HIV disease.
HIV Infection and Acquired Immunodeficiency Syndrome 405
Keratoderma blenorrhagicum in an HIV-positive patient: ery- Circinate balanitis in an HIV-positive patient: moist annular
thematous hyperkeratotic plaques on palms (and soles). plaques with serpiginous margin.
HIV Infection and Acquired Immunodeficiency Syndrome 407
Acquired ichthyosis in an HIV-positive patient: lesions of Oral aphthae: ulcers with central necrotic slough and halo of
acquired ichthyosis resemble ichthyosis vulgaris. Appearance of erythema. Occur when CD4+ count < 100/mm3.
ichthyosis in later life warrants search for an underlying disease
including an HIV infection. Ichthyosis in HIV-infected generally
occurs in advanced HIV infection.
HIV Infection and Acquired Immunodeficiency Syndrome 409
Serological Tests
Two types of serological tests for HIV infection:
zzScreening tests.
zzSupplemental test.
Screening tests
zzFeatures: rapid, inexpensive. Highly sensitive,
may not be very specific (i.e. false positives
Genital herpes in an HIV-positive patient: extensive polycyclic Anogenital warts in an HIV-positive patient: large lesions of
lesions extending from genital area onto lower abdomen. condyloma acuminata and smaller lesions of bowenoid papulosis
(marked).
HIV Infection and Acquired Immunodeficiency Syndrome 411
Classification of ART
NRTI NtRTI NNRTI Protease inhibitor Fusion inhibitor Integrase CCR-5 inhibitor
inhibitor
Zidovudine (AZT)* Tenofovir (TDF)* Nevirapine (NVP)* Indinavir (INV)* Enfuvirtide (T-20) Raltegravir Maraviroc
Didanosine (ddI)* Efavirenz (EFV)* Ritonavir (RTV)*
Zalcitabine (ddC)* Delaviridine (DLV) Saquinavir (SQV)*
Stavudine (d4T)* Etravirine Nelfinavir (NFV)*
Lamivudine (3TC)* Lopinavir (LPV)*
Abacavir (ABC)* Atazanavir (ATV)*
Emtricitabine (FTC) Darunavir
*Available in developing countries
Morbilliform eruption: generalized erythematous, maculopap- Drug hypersensitivity reaction: generalized erythematous mac-
ular eruption due to nevirapine. Similar rash can develop with ulopapular eruption with peripheral edema due to nevirapine.
abacavir. Abacavir can also cause similar reactions.
Acute generalized exanthematous pustulosis: widespread Toxic epidermal necrolysis: large areas of sheets of dusky red
eruption of sterile pustules due to nelfinavir. skin which denude to reveal erosions. Due to nevirapine can also
develop with abacavir.
HIV Infection and Acquired Immunodeficiency Syndrome 413
sexual practices including consistent and cor- NACO 2007 guidelines for starting ART
rect use of condoms, avoiding casual sex and (modified by 2011 memorandum)
intravenous drug use. Clinical scenario CD4 test not CD4 test available
¾¾Supporting of those afflicted directly and indi- (WHO clinical stage) available
rectly by HIV: Asymptomatic Do not treat
zzTypes: (1) Treat if CD4 < 350
¾¾Pre-test counseling: involves providing basic Mild symptoms Do not treat cells/mm3
information on HIV/AIDS. And risk assess- (2)
ment of those being HIV tested. Advanced symptoms Treat
¾¾Post-test counseling: depends on result: (3) Treat irrespective of
If negative: basic knowledge on HIV reit- Severe/advanced Treat CD4 count
erated to assist client to adopt behaviors symptoms
that reduce future risk of getting infected. A (4)
repeat test is recommended after 12 weeks. Follow-up counseling: to re-emphasize adop-
If positive test: client assisted to cope with tion of safe behaviors to prevent transmis-
result. And helped to access treatment and sion of HIV infection. And assists in estab-
care. And supported to disclose HIV status lishing linkages and referrals to services for
to partner. care and support including ART, nutrition,
WHO 2013 guidelines for starting ART in HIV-positive patients home-based care and legal support.
Population Recommendation
Which antiretroviral therapy to start when
Initiate ART if CD4 cell count ≤500 cells/
Target population WHO, 2013 NACO, 2007
mm3
guidelines guidelines
• As a priority, initiate ART in all individuals
with severe/advanced HIV disease (WHO clini- HIV + ARV- TDF + 3TC (or FTC) 3TC + AZT/
Adults and cal stage 3 or 4) or CD4 count ≤350 cells/mm3 naive adults and + EFV d4T + NVP/EFV
adolescents adolescents
Initiate ART regardless of WHO clinical
(≥ 10 years) stage or CD4 cell count HIV + pregnant TDF + 3TC (or FTC) AZT + 3TC +
• Active TB disease women + EFV NVP
• HBV coinfection with severe chronic liver HIV/TB coinfection TDF + 3TC (or FTC) AZT/D4T+
disease + EFV 3TC+EFV/NVP
• Pregnant and breastfeeding women with
HIV/HBV coinfection TDF + 3TC (or FTC) AZT/+
HIV
+ EFV 3TC+EFV/NVP
• HIV-positive individual in a serodiscordant
partnership (to reduce HIV transmission risk) Abbreviations: 3TC: lamivudine; ARV: antiretroviral (drug); AZT: zidovu-
dine; d4t: stavudine; EFV: efavirenz; FTC: emtricitabine; NVP: nevirapine;
Children ≥5 Initiate ART if CD4 cell count ≤500 cells/mm 3
TDF: tenofovir disoproxil fumarate
years old • As a priority, initiate ART in all children with
severe/advanced HIV disease (WHO clinical stage
3 or 4) or CD4 count ≥350 cells/mm3 Antiretroviral Therapy
Initiate ART regardless of CD4 cell count
• WHO clinical stage 3 or 4
Antiretroviral Drugs
• Active TB disease zzObjective of therapy: antiretroviral treatment
Children 1–5 Initiate ART in all regardless of WHO clinical (ART) suppresses disease, does not eradicate
years old stage or CD4 cell count virus. So reduces morbidity and mortality but
• As a priority, initiate ART in all HIV-infected chil-
dren 1–2 years old or with severe/advanced HIV does not cure.
disease (WHO clinical stage 3 or 4) or with CD4 zzClassification: ART broadly classified by phase
count ≤750 cells/mm3 or <25%, whichever is lower of retrovirus life-cycle inhibited:
Infant <1 Initial ART in all infants regardless of WHO ¾¾Reverse transcriptase inhibitors:
years old clinical stage or CD4 cell count Nucleoside reverse transcriptase inhibi-
WHO 2013 ART guidelines; Source: apps.who.int/iris/bitstream/10665/ tors (NRTI): compete with natural deoxynu-
85321/1/9789241505727_eng.pdf cleotides8 for incorporation into viral DNA
8
NRTIs: Are analogs of naturally occurring deoxynucleotides which are needed to synthesize HIV DNA.
Nail pigmentation: bluish-black nail pigmentation due to zido- Mucosal pigmentation: bluish-black mucosal pigmentation due
vudine. Pigmentation of nail may appear as early as 1 month after to zidovudine. Zidovudine typically causes pigmentation of lateral
inititation of zidovudine. aspect of tongue.
Fixed drug eruption on glans: well-defined erythematous Facial lipoatrophy: irreversible loss of subcutaneous fat began
plaque on the glans which developed after taking cotrimaxozole 6 months after starting therapy with zidovudine (also seen with
for prophylaxis for pneumocytis pneumonia. stavudine).
HIV Infection and Acquired Immunodeficiency Syndrome 415
chain but form defective DNA which cannot ¾¾Abacavir (ABC): severe hypersensitivity reac-
extend DNA chain resulting in chain termi- tion not uncommon.
nation. ¾¾Protease inhibitors: dyslipidemia (high total
Nucleotide reverse transcriptase inhibitors and LDL cholestrol, decreased HDL cholestrol
(NtRTI): act as NRTI. and elevated triglycerides).
Non-nucleoside reverse transcriptase
inhibitors (NNRTI): act as non-competitive Indications
inhibitors of reverse transcriptase (by bind- ART to be initiated only if indicated, not only on
ing to it). basis of presence of HIV infection.
¾¾Protease inhibitors (PI): inhibit protease which
cleaves polyproteins for final assembly of new Drug Regimens
HIV virions.
zzART given as Highly Active Antiretroviral Therapy
¾¾Fusion inhibitors (FI): prevents fusion of viral
envelope with cell membrane of target cells. (HAART), a combination of 3 drugs:
¾¾Integrase inhibitors (II): inhibit enzyme inte-
grase, which integrates HIV DNA into infected Monitoring of HAART
cell DNA. Real challenge to monitor efficacy of ART because
zzSide effects: several side effects described with of immunological and virological discordancy. And
ART. Common ones include: to diagnose resistance to initiate. 2nd line therapy.
¾¾Zidovudine (AZT): anemia, (hemoglobin mon- zzViral load estimation.
itoring recommended) and lipoatrophy. ¾¾Most sensitive marker of efficacy. But resource
¾¾Stavudine (d4T): peripheral neuropathy, lipo- intensive.
dystrophy, dyslipidemia lactic acidosis. ¾¾Recommended to measure every 3–6 months.
¾¾Lamivudine (3TC): relatively safe, but drug ¾¾Expressed as RNA copies.
most prone to HIV developing resistance. zzSurrogate markers: used individually or in
¾¾Tenofovir (TDF): nephropathy (so renal func- combination but poorly sensitive.
tion monitoring). ¾¾Clinical improvement.
¾¾Nevirapine (NVP): drug eruptions (maculo- ¾¾CD4 counts.
papular eruptions, Steven-Johnson syndrome-
toxic epidermal necrolysis), hepatotoxicity.
Drugs for Opportunistic Infections
¾¾Efavirenz (EFV): gastrointestinal side effects,
hepatotoxicity, nephrotoxicity.? Safety in preg- Various regimens recommended for prophylaxis
nancy. and therapy of opportunistic infections.
Management of opportunistic infections in HIV patients
Treatment (daily) Prophylaxis
Infections Indications Drug regimen (daily)
Pneumocystis jiroveci TMP (15 mg/kg) + SMZ* CD4+ < 200/mm 3
TMP (2.5 mg/kg) + SMZ
(75 mg/kg) × 3 weeks (12.5 mg/kg)
Toxoplasma encephalitis Sulfadiazine (4–8 g) + Pyrimethamine Toxoplasma seropositive Sulfadiazine (2-4 g) +
(200–400 mg) × 6 weeks CD4+ < 100/mm3 Pyrimethamine (100 mg)
Herpes Acyclovir 400 mg × 5 times × 2 weeks > 6 recurrences/year Acyclovir 400 mg bid
simplex virus
Herpes zoster Acyclovir 800 mg × 5 times × 2 weeks
M. tuberculosis Standard therapy. Look for MDR
tuberculosis. ATT initiated before HAART
Candidiasis Fluconazole 100–200 mg Fluconazole 150 mg weekly
daily × 3 weeks
*TMP-SMZ: trimethoprim-sulfamethoxazole
21 Miscellaneous
and Rare
Dermatoses
Orofacial granulomatosis: asymmetric thickening of the lower Orofacial granulomatosis: massive upper lip swelling, mild
lip with ulceration over its inner aspect. lower lip swelling and diffuse indurated swelling over lower face.
Melkersson Rosenthal syndrome: triad of facial palsy (right Lupus miliaris disseminatus faciei: inflammatory papules and
side), furrowed tongue and labial swelling. nodules in centrofacial distribution. Prominent eyelid lesions.
Miscellaneous and Rare Dermatoses 419
Treatment
None proven completely effective. Based on
literature, topical or systemic glucocorticoids, oral
clofazimine, thalidomide and azathioprine have
been tried.
Sweet’s syndrome: edematous, erythematous plaque on cheek Sweet’s syndrome: shiny erythematous plaque with slightly bos-
with pseudovesicular appearance (note tiny pustules) at the selated surface.
margins.
Miscellaneous and Rare Dermatoses 421
Treatment
Removal/excision of the foreign material. Topical/
intralesional steroids may help.
NEUTROPHILIC DERMATOSES
Scleromyxedema: grouped waxy papules over lower back and Scleromyxedema: waxy papules and plaques over nape of neck
gluteal area, many showing linear configuration. and upper back. Patchy hair loss over occipital scalp secondary
to papular lesions is seen.
Miscellaneous and Rare Dermatoses 423
Angiolymphoid hyperplasia: asymptomatic, erythematous pap- Angiolymphoid hyperplasia: deep seated swelling over the
ules and nodules on pinna—a characteristic site. frontal scalp. Reddish papules and nodules over and around the
swellings.
Miscellaneous and Rare Dermatoses 425
Subcutaneous nodules also recently reported. widely seperated collagen bundles. Empty spaces
Associated underlying paraproteinemia in 80–90% contain mucin that stain positive with alcian blue.
cases. Cases without paraproteinemia labelled
atypical. Treatment
Streptococcal-associated cases self-limiting,
Histopathology resolve over months to years. Diabetes associated:
Triad of fibroblast proliferation, collagen deposition refractory to therapy. Paraproteinemia-associated:
and abundant dermal mucin (upper and mid extracorporeal photopheresis. PUVA therapy and
reticular). cyclosporine effective in some. Physiotherapy to
prevent limitation in movement in all.
Treatment
Difficult and disappointing, variable
clinical response. Systemic steroids and
immunosuppressants like cyclophosphamide,
melphalan (more commonly used) may help
but have serious toxicities over long term use.
Anecdotally isotretinoin tried. Other but expensive
alternatives—plasmapharesis, intravenous
immunoglobulins and stem cell transplantation.
Scleredema
Rare. Sudden onset symmetric induration of skin
usually preceded by prodrome of fever, body
aches. Onset from neck, upper back and shoulders,
progress to face and arms later. Abnormality only
felt on palpation, otherwise skin appears normal. Histopathology of scleredema: square shaped biopsy, thick-
Difficulty in mouth and eye closure, joint movement, ened collagen bundles with spaces between the bundles. Inset—
rarely dysphagia may occur. Four types: idiopathic, abundant bright blue mucin (alcian blue-PAS stain)
poststreptococcal, type II diabetes-associated and
paraproteinemia-associated.
CUTANEOUS PSEUDOLYMPHOMAS
Histopathology Skin disorders that mimic lymphomas
Square shaped biopsy (normal tapering shape lost histopathologically in form of dense lymphoid
due to diffuse dermal thickening). Thick dermis with aggregates.
Jessner’s lymphocytic infiltrate: erythematous plaques with Jessner’s lymphocytic infiltrate: erythematous plaques over ‘V’
confluent papules at the periphery, many showing annular area of upper chest.
configuration.
Miscellaneous and Rare Dermatoses 427
Histopathology
Dilated vascular channels, lined by plump,
vacuolated endothelial cells which resemble
epithelioid cells. Perivascular moderate to dense
Histopathology of angiolymphoid hyperplasia with eosino-
nodular infiltrate of lymphocytes, eosinophils and philia: numerous capillaries with slit like lumen lined by plump
plasma cells. Variable vascular and lymphoid endothelial cells. Nodular aggregate of lymphocytes and numer-
components; some have more vascular and some ous eosinophils.
have more lymphoid findings.
Cutaneous Lymphoid Hyperplasia
Treatment (Syn—Lymphocytoma Cutis, Spiegler
Difficult, recurrent condition. Medical therapies Fendt Sarcoid)
usually fail. Topical/Intralesional steroids may Hypersensitivity response to various stimuli like insect
give symptomatic relief. Electrosurgery, lasers, bite, vaccination, gold body piercing, tattoos, etc.
cryosurgery, radiotherapy tried with some success.
Complete surgical excision treatment of choice if Clinical Manifestation
feasible. Recently intralesional radiofrequency
described with good relief in solitary lesion. Mildly firm to firm solitary nodule or tumor,
occasionally papules or plaques. Sometimes
Elastosis perforans serpigenosa: keratotic papules arranged in Plica polonica: scalp hair entangled into multiple plaits and an
serpigenous and arcuate manner on neck irreversible mass.
Courtesy: Dr Geeti Khullar, Department of Dermatology, PGI
Chandigarh.
Miscellaneous and Rare Dermatoses 429
Papulonecrotic tuberculide of glans: necrotic ulcer with bridg- Zoon’s balanitis: solitary glistening red plaque over the dorsum
ing scars on the glans. of glans penis. Telangiectasias visible over the plaque (‘cayenne
pepper spots’)
Miscellaneous and Rare Dermatoses 431
Treatment
If underlying disease present, control of that
improves pruritus and perforating process.
Otherwise treatment relied on keratolytics like
urea-lactic acid combinations, topical retinoids,
oral doxycycline, UVB, PUVA, etc.
Exogenous ochronosis: violaceous blotchy/reticulate pigmenta- Pentazocine abuse: multiple crusted ulcers along veins with
tion over the cheek and forehead. Note, the epidermal wrinkling non-pitting edema of hands (puffy-hand syndrome).
and shininess, suggestive of triple combination induced atrophy.
Miscellaneous and Rare Dermatoses 433
Erythromelalgia: prominent redness of both feet (evident by Flagellate pigmentation: caused by bleomycin.
area of blanching produced by pressure from thumb).
Miscellaneous and Rare Dermatoses 435
Muir Torre syndrome: innumerable discrete greasy umblicated Amyloidosis cutis dyschromica: rippled hyperpigmentation
papules (sebaceous adenomas) over the neck and chest. over photo-exposed area of chest. Punctate hypopgimented
macules also present.
Miscellaneous and Rare Dermatoses 437
Treatment Treatment
Early stage: topical salicylic acid ointment Mainly preventive. Screening for internal
and intralesional steroid. Late stage: surgical malignancy in view of large number of truncal
amputation. Antibiotics for secondary infections. sebaceous tumors. Oral isotretinoin (@0.8 mg/
kg/day) may prevent development of cutaneous
Muir Torre Syndrome malignancy.
Rare syndromic association between skin
neoplasia (sebaceous adenomas, sebaceous AMYLOIDOSIS CUTIS DYSCHROMICA
carcinomas, keratoacanthoma) with internal
Familial pigmentary disorder of unknown cause
malignancies (colorectal, small bowel, endometrial
and pathogenesis.
or urothelial). Autosomal dominant inheritance,
Characterized by reticular hyperpigmentation
defect in MSH2 and MLH1 genes. Associated
studded with punctate hypopigmented macules
with HNPCC (hereditary non-polyposis colorectal
distributed extensively, onset usually before
cancer). Skin lesions precede or succeed internal
puberty, no or little itch, and focal papillary dermal
malignancy. Sebaceous adenomas appear as
amyloid deposition. Rarely papules may also
raised umblicated paules or nodules. In MTS, trunk
occur. Considered as differential diagnosis for
more commonly involved. Sebaceous carcinomas
reticulate pigmentary disorders and dyschromatotic
involve eyelids commonly.
disorders.
Histopathology
Treatment
Multilobular, lobules well-defined, variable
No definitive treatment available. Photo-
numbers of small, basophilic, sebaceous matrix
protection, topical keratolytics, CO2 laser, oral
cells peripherally and larger mature sebaceous
retinoids may help.
cells.
22 Therapeutics in
Dermatology
of water from skin surface. Used with benefit in propionibacterium acnes, anaerobes. Used for
exudative lesions. impetigo and secondarily infected eczema.
In decreasing order of acuteness of inflammation,
the bases may be selected in the following order: Antifungal Agents
Clear lotions—shake lotions—creams pastes Several agents. Some specific for dermatophytic
—ointments. or candidal infections, others cover both as well
as Pityrosporum orbiculare (pityriasis versicolor).
ACTIVE SUBSTANCES Advisable to use all topical antifungal agents for
May have specific, quasi-specific or symptomatic 2–3 weeks after clinical and mycological cure.
zzAzoles: Broad spectrum fungistatic agents—
action. Commonly employed active substances
are the following as mentioned below. inhibit growth through action on cell wall.
Effective against dermatophytes, candida
Antibacterial Agents and P orbiculare. Also effective in erythrasma.
Miconazole nitrate 2%, clotrimazole 1%,
Wide choice—from soaps and antiseptics to econazole 1% and ketoconazole 2%. Newer
antibiotics. Soaps act by being anionic detergents; congeners-sertaconazole 2% cream and
potassium permanganate and hydrogen luliconazole 1% cream. Sertaconazole has
peroxide by being oxidizing agents; antibiotics by greater reservoir effect in startum corneum than
bacteriostatic or bactericidal actions. Commonly older azoles. Good activity against tinea, candida
used antibacterials are: triphenylmethane dyes and also Gram positive bacteria. Luliconazole
(0.5–2% brilliant green and 0.5–1% gentian violet), 1% cream. Once a day therapy for 2 weeks for
3% hydrogen peroxide, 0.5–2% silver nitrate; tinea cruris, corporis and pedis. Advantage of
10% hexachlorophene. Commonly used topical single application.
antibiotics—neomycin, polymyxin band bacitracin. zzAllylamines and benzylamines: Terbinafine
Lotion, gel or cream bases preferred. Ointments 1%, naftifine 1% and butenafine 1%. Fungicidal
avoided as they cause occlusion—hence spread agents—inhibit squalene epoxidase. Effective
infection. Agents such as nitrofurazone, penicillin, against dermatophytes, pityrosporum and
sulphonamides, streptomycin and salicylanilides candida (c.f. oral terbinafine effective only
best avoided because of their potential to sensitize. against dermatophytes).
Mupirocin, 2% ointment or cream and fusidic zzOthers: Tolnaftate 1%: effective against
acid 2% cream have various indications. Mupirocin dermatophytes but not against candida.
bactericidal. Spectrum-staphylococcal species and Haloprogin: 1% cream/solution: effective
beta hemolytic streptococci. Increasing resistance against dermatophytes and P orbiculare and
amongst methicillin resistant Staphylococcus aureus possibly candida. Ciclopirox olamine 1%
effective in impetigo, folliculitis and infected eczema. cream: broad spectrum fungistatic agent
Not to be used for prolonged periods—results in effective against dermatophytes, candida
development of resistance. Fusidic acid bacteriostatic and P orbiculare. Also available as 8% nail
but broader spectrum of activity-all bacteria as in case lacquer. Amorolifine 5% nail lacquer. Both
of mupirocin plus MRSA, neisseria and bacteroides. nail lacquers effective as monotherapy for
Can penetrate intact skin and crusts. Useful in distal onychomycosis and superficial white
furuncles, impetigo. Also erythrasma. onychomycosis. Also as adjuvant therapy with
New antibiotics-nadifloxacin 1% cream and systemic antifungal agents. Amorolfine now
retapamulin 1% ointment. Nadifloxacin bactericidal, available as 0.25% cream, used as once a day
used for treatment of acne but also for infections. application for tinea. Nystatin, amphotericin B
Broad spectrum activity—Gram positive, MRSA, and gentian violet creams or lotions effective
Gram negative and anaerobic bacteria. No cross against candida only. Selenium sulphide
resistance with other quinolones. Retapamulin (1.25–2.5%) effective in pityriasis versicolor.
bacteriostatic. Spectrum—Gram positives, MRSA,
Whitfield’s ointment (6% benzoic acid and effect. 5%—used for scabies. Safe in children.
3% salicylic acid) combines fungistatic and May be used in pregnant women.
keratolytic action. Now obsolete. zzGamma benzene hexachloride (GBH): 1%
cream or lotion. Effective against scabies,
Antiviral Agents pediculosis pubis and capitis. Toxic. Not to be
Idoxuridine (5-iodo 2-deoxyuridine; IDU): 0.1–0.5%, used in children <5 years and pregnant women.
zzBenzyl benzoate: 25% emulsion. Effective
useful in herpetic keratitis. For herpes genitalis,
5–15% IDU with dimethylsulfoxide (DMSO) applied against scabies. Less so against pediculosis
early in course of disease shortens the duration of pubis and capitis.
zzCrotamiton: 10% lotion or cream. Effective in
attack in some patients. Allergic contact dermatitis
may occur. scabies. Also mild antipruritic. Useful in children.
zzDDT: 5% powder—for disinfection of clothes in
zzAcyclovir: 5% cream; virostatic-inhibits DNA
synthesis through activation of thymidine kinase. pediculosis corporis.
zzSpinosad: 0.9% suspension-for pediculosis
Topical application gives only symptomatic relief
and perhaps shortens the course. Not very capitis. Found to be more effective than
useful. permethrin 1%.
zzBenzyl alcohol: 5% lotion-pediculosis capitis.
zzPodophyllin resin: 10–25% in compound
zzIvermectin: 0.5% lotion-pedicluosis capitis. Also
tincture of benzoin. Cytotoxic effect; useful
in anogenital warts, as physician-applied used as oral preparation for scabies (@200 mg/kg,
therapy. Adjoining skin should be protected with 2 doses 2 weeks apart).
petrolatum. Podophyllotoxin (0.5%)—patient-
applied therapy, twice daily for 3 consecutive Anti-inflammatory Agents
days a week. Both can cause irritation. Not be Topical corticosteroids—most potent anti-
used in pregnancy. inflammatory preparations. Tars, shake lotions,
zzImiquimod: 5% cream; an immunomodulator. emollients such as oils and creams—moderate
Used in anogenital warts, molluscum anti-inflammatory agents. Topical calcineurin
contagiosum. Thrice a week; wash after 6–10 inhibitors also commonly used, efficacy equivalent
hours after application. Available as single use to lower-mid potent steroid.
sachets. zzCorticosteroids: Choice of corticosteroid
zz5-flurouracil: 5% cream; cytotoxic agent. Used depends upon acuteness of inflammation,
more frequently for treating solar keratoses, site(s) affected, age, expected duration of use
Bowen’s disease and some basal cell and cost of preparation. Available as creams,
carcinomas. ointments, lotions or gels. Concentration
zzSinecatechin: 15% ointment; reduces expression and potency of corticosteroids varies widely.
of human papilloma virus oncoproteins E6 and Steroids dispensed in ointment base generally
E7. FDA approved for external anogenital warts. more potent than same concentration in
zzCidofovir: 1% gel; inhibits viral polymerases. cream base. Occlusion and combination with
Broad spectrum activity, used in resistant genital keratolytics enhance absorption and improve
herpes, resistant molluscum contagiosum, ano- effectiveness. Combination with antibiotics and
genital warts. other antibacterials and/or antifungals available.
Combination with tars may, however, disturb
Antiparasitic Agents pharmaceutical stability.
zzPermethrin: 1% cream, lotion. Treatment of The following preparations are commonly
choice in pediculosis capitis–safe, has residual available for therapy (in decreasing order of
potency).
Classification of some common topical steroids according burning, local infections (viral, fungal). Therapy
to potency under occlusion may provide enhanced effect.
S.No Potency Examples Preparation
Keratolytic Agents
1 Superpotent Clobetasol Oint/Cr
propionate 0.05% Oint/Cr Act by dehiscing or removing keratin:
Halobetasol Oint zzHydroxy acids: alpha (glycolic acid, lactic acid,
propionate 0.05% pyruvic acid), beta (slaicylic acid). Act by dissolving
Betamethasone
demosomal junctions, causing desquamation.
dipropionate 0.05%
Long term use leads to thickening of dermis.
2 Potent Betamethasone Cr Glycolic acid (20–70%) and salicyclic acid (2–10%)
dipropionate 0.05% Oint
Fluocinonide 0.05% Oint
used commonly in chemical peels for pigmentary
Mometasone disorders and acne scars. Higher concentrations
furoate 0.1% of salicylic acid used for corns and callosities.
3 Upper Betamethasone Lotion zzUrea 20–40%: as aqueous solution or ointment
midstrength dipropionate 0.05% Oint base, dissolves hydrogen bonds and epidermal
Fluticasone Oint keratin.
propionate 0.005% zzResorcinol: a phenol derivative, occasionally
Triamcinolone employed in 1% concentration in acne
acetonide 0.1%
preparations.
zzRetinoic acid: 0.025, 0.05 and 0.1%, is a weak
keratolytic agent.
Side effects: Local atrophy of the skin and
striae distensae (particularly in the folds of Keratoplastic Agents
skin). Prolonged use of potent fluorinated Restore abnormal keratinization process to
corticosteroid preparations on the face may normalcy. Useful in psoriasis and subacute
result in perioral dermatitis; and in children eczemas. Several agents available:
systemic toxicity due to efficient percutaneous zzTars: distillation products of organic substances.
absorption. Pustular psoriasis—after withdrawal Crude coal tar supposed to be therapeutically
in patients of psoriasis. Under occlusion, may superior to wood tars, bituminous tars and
cause folliculitis. Indian skin tolerates topical petroleum tars. Active constituent(s) unknown.
corticosteroids rather well. Exact mode of action ill-understood. Weak
zzTopical calcineurin inhibitors: Most preferred phototoxic action; UVA and/or UVB perhaps
anti-inflammtory agents for facial application augment its therapeutic value. Crude coal tar,
or long term use, because of good safety coal tar solution (liquor carbonis detergens) or
profile. Bind to calcineurin, inhibit synthesis of ichthammol can be incorporated into ointments,
interleukin 2, effectively inhibiting activation and creams, shampoos, in concentrations varying
proliferation of T lymphocytes. Tacrolimus 0.03 between 1 and 10%. Side effects-staining of
and 0.1% ointments; and Pimecrolimus 1% clothes, follicultis, erythema, contact dermatitis,
cream. etc. Liposomal preparations available, reduce
Indications similar except pimecrolimus irritation and staining side effects.
preferred over facial lesions, expensive and safer zzAnthralin (1,8 dihydroxyanthranol): a
in infants. Common indications of tacrolimus- synthetic substance prepared from anthracene.
atopic dermatitis, vitiligo, psoriasis, lichen Useful in psoriasis. Mode of action uncertain.
planus, rosacea, morphea/lichen sclerosus. Applied locally as a paste in concentrations
Common indications of pimecrolimus-vitiligo, of 0.5–1% and washed off after 4–6 hours to
psoriasis, lichen planus, atopic dermatitis, lichen prevent irritation. ‘Short contact’ therapy involves
sclerosus. Side effects include local irritation, application of 2–5% for 12–2 hours. Claimed
to give better results and cause less irritation. since percutaneous absorption can result in
Disadvantages: irritates skin and stains clothes. nephrotoxicity. Local anesthetics and local
Inactivation of dithranol zinc paste—prevented antihistamines, good antipruritics but have a high
by addition of salicylic acid. contact sensitizing potential and so should not
zzRetinoic acid (vitamin A acid, tretinoin): be used. Crotamiton (10%) is a good antipruritic,
has ‘regulatory’ effect on epidermal cell besides being scabicidal.
differentiation. Most often used in acne in
concentrations of 0.025, 0.05%, or 0.1%. Sunscreens
Comedonic acne responds best. Also useful Protect from light. Two broad categories recognized:
in Darier’s disease, verrucous epidermal nevi, organic (earlier designated as chemical)
ichthyosiform dermatoses and palmoplantar sunscreens. Or inorganic (earlier designated as
keratoderma. Therapeutic value in psoriasis physical) sunscreens.
debatable. Contraindicated in pregnancy. zzOrganic sunscreens: act by absorption of
specific wavebands of light. Para-aminobenzoic
Depigmenting Agents acid (PABA) or one of its esters absorbs UVB
zzHydroquinone (2–5%): has antityrosinase <320 nm). Only infrequently used now due to
activity and toxic effect on melanocytes. An sensitizing potential. Benzophenones absorb
effective and safe remedy in treatment of UV and partially visible light. Dibenzoylmethanes
melasma. (e.g. avobenzone) absorb UVA while cinnamates
zzMonobenzyl ether of hydroquinone (MBH): are UVB absorbers.
a melanocyte poison, could result in mottled zzInorganic sunscreens: act by physically
depigmentation—often irreversible. MBH 20%, deflecting light and thus acting as barriers (sun
however, profitably employed in the treatment blocks). Titanium dioxide, zinc oxide, kaolin, talc
of residual pigmented areas in widespread deflect light or simply impose a physical barrier.
vitiligo to achieve uniform white color. May need Effect not restricted to any specific waveband,
maintenance applications especially on photo- though shorter wavelengths get scattered better.
exposed areas. However, difficult to procure. Also widely used in cosmetics.
zzAzelaic acid (10–20% cream): proven useful zzSPF: is a measure of protection provided by
but mechanism uncertain. Possibly by inhibiting sunscreen (primarily against UVB). It is given by
microbial respiratory chain (useful as anti- the ratio of the minimum erythema dose (MED)
acne medication) and inhibits tyrosinase (anti- of the skin protected by sun screen to MED of
melasma medication). the unprotected skin. Being a ratio, it has no unit.
zzKojic acid 2% cream: moderately effective in
pigmentation disorders. Mechanism same as Astringents
azelaic acid. Arbutin 3% questionable efficacy. Precipitate surface proteins and reduce oozing.
Useful in exudative conditions. Liquor aluminum
Antipruritic Agents acetate 0.52%; silver nitrate 0.05–2%.
Relieve itching by a variety of mechanisms:
by blocking the nerve impulses as with local Caustics
anesthetics, chloretone (1–2%) and phenol (1–2%). Act by chemical destruction of lesions; useful in
Or by change of itch to some other sensation— removal of growths, xanthelasma, warts, Bowen’s
cold as with menthol (0.01–1%) and calamine disease. Phenol 80% w/v solution of water and
lotion (cooling by evaporation)—or warmth as with trichloroacetic acid are potent caustics. High
camphor (1–2%). Or by ill-understood mechanisms concentrations of silver nitrate (10–20%) or low
as in case of corticosteroids. Antipruritics can concentrations of trichloroacetic acid (5%) are mild
be incorporated in lotions, creams, ointments or caustics and used for destruction of epidermis or
pastes. Phenol avoided on extensive surfaces exuberant granulation tissue.
weekly for 15–18 weeks. Candidiasis and administered in fruit juice, thrice daily and
pityriasis versicolor–150 mg single dose. Weekly gradually build it up to 15 drops three times a
dose useful in recurrent infections. day or till signs of iodism (lacrymation, epiphora)
zzKetoconazole: a water soluble imidazole. Well appear.
absorbed orally (c.f. miconazole). Effective
against dermatophytes, candida, Cryptococcus Antileprosy Therapy
and coccidioidomyces but used infrequently due For purpose of therapy, leprosy patients classified
to hepatotoxicity. Dose: 200–400 mg/day. into paucibacil/ary (less than 6 lesions and 2 nerve
zzGriseofulvin: a penicillium derived fungistatic
trunks) or multibacillary (more than 5 lesions and 1
antibiotic effective against only dermatophytic nerve trunk):
species. With availability of other drugs, no zzDapsone (diaminodiphenyl sulphone, DDS):
longer a drug of choice. Dose: 10 mg/kg/day, primary drug in all but dapsone-resistant forms
preferably given, for better absorption, after a of leprosy. Bacteriostatic. Dose: 1–2 mg/kg/day
fatty meal. Length of treatment depends upon administered as a single daily oral dose. Given
site of infection. Thus for T. corporis, 3–4 weeks for 6 months in paucibacillary and 1 year (or more
and for T. capitis 6–8 weeks. T. unguium takes in highly bacillated patients) in multibacillary
3–6 months for finger nails and 6-18 months leprosy. Hemolytic anemia, a universal but
for toe nails. Drug interactions seen with mild side effect. Serious toxicity rare—includes
barbiturates and coumarin anticogulants. Toxicity psychosis, hepatitis.
generally mild-headaches, gastrointestinal zzRifampicin: highly bactericidal morphological
upsets; occasionally severe: photosensitivity, index (MI) and perhaps viability of bacteria
precipitation of porphyria cutanea tarda or reduced to zero within 4 weeks. Dose: single
acute intermittent porphyria and systemic lupus supervised 600 mg (or 450 mg for patients
erythematosus. Resistance to drug uncommon. weighing 45 kg or less) once a month for
zzNystatin: a polyene antibiotic. Not absorbed
6 months in paucibacillary and for 1 year (or more
from the gut. Oral administration useful in
in highly bacillated patients) in multibacillary
buccal, esophageal or intestinal candidiasis
leprosy. Empty stomach aids absorption. Always
and in cutaneous or other forms of candidiasis.
warn patient regarding changed color of urine.
Tablets of 500,000 units or oral suspension
Hepatotoxic, nephrotoxic and causes ‘flu-like’
containing 100,000 units per ml given 3–4 times
syndrome.
a day. Of little value in the treatment of systemic
zzClofazimine: a fat soluble imino-phenazine
candidiasis.
compound. Bacteriostatic and anti-inflammatory.
zzAmphotericin B: a polyene antibiotic effective
Employed in addition to rifampicin and dapsone in
against number of deep mycotic infections.
MDT of multibacillary leprosy. Dose: 50 mg/ day
Administered intravenously (absorption by oral
unsupervised and 300 mg once a month under
route poor) in gradually increasing doses. Start
supervision given for 12 months (or longer in
with 1–5 mg/day and raise up to 0.65 mg/kg/day.
highly bacillated patients). Useful also in the
Several side effects, including serious ones:
treatment of type II reactions in leprosy; dose:
headaches, chills, hemolytic anemia, phlebitis,
200–300 mg/day. Toxicity: reddish discoloration of
anorexia, impaired renal function—reversible or
skin and ichthyosiform change; skin discoloration
permanent and fatal.
may be objectionable and unacceptable.
zzFlucytosine: 5-fluorocytosine. Antifungal
activity additive to that of amphotericin B. All medicines started concurrently and discontinued
Mild to moderate toxicity-anemia, leukopenia, after stipulated periods. Patients followed up for
headaches, hallucinations and vertigo. 2 years in paucibacillary and 5 years in multibacillary
zzPotassium iodide: almost specific for disease. In dapsone resistant cases, several
sporotrichosis and subcutaneous phycomycosis. alternatives available: minocycline, ofloxacin and
Start with 5 drops of saturated solution, clarithromycin.
zzTreatment of reactions in leprosy: non-steroidal Dose: varies depending on the nature, extent
anti-inflammatory agents (NSAIDs) useful in and severity of disease. Alternate day therapy
mild to moderate type I or type II reactions. In preferred (patient’s condition permitting) when
severe type I reactions, corticosteroids may be long term corticosteroid treatment is required.
used, particularly in impending nerve palsies. In Large parenteral or oral bolus ‘pulse’ therapy
type II reactions, corticosteroids better avoided, indicated in conditions needing long term therapy.
as patients are likely to become steroid- Possibly induces remission faster; toxic effects
dependent. Use thalidomide instead—is almost similar, maybe less. Immunosuppressives,
as good. Notoriously teratogenic, hence avoided such as cyclophosphamide , methotrexate or
in females of child-bearing age. Intradermal azathioprine may be used as adjuvants- steroid
Mw vaccine may be used as adjunct in the sparing. Adverse effects: multiple and sometimes
management of type II lepra reaction. serious. Cutaneous: acne, hypertrichosis, striae,
susceptibility to pyococcal or fungal infections.
Antileishmanial Agents Systemic: hypertension, diabetes, reactivation of
Several choices available; none entirely tuberculosis.
satisfactory. Pentavalent antimonial, sodium
stibogluconate, 330 mg/mL (equivalent to 100 mg Immunosuppressive and Cytotoxic Agents
of pentavalent antimony). Dose: 10 mg/kg/day. Used generally in psoriasis, pemphigus, mycosis
Average course: 6–10 injections for cutaneous fungoides and histiocytosis. Different modes of
leishmaniasis and 90–120 injections for post kala action; used alone. Or in combinations. May have
azar dermal leishmaniasis. Reasonable results. steroid sparing effect. Some of the commonly used
Rifampicin, metronidazole give inconsistent ones are:
results. Ketoconazole 200–400 mg single daily zzMethotrexate: an antimitotic agent. Acts by
dose, seems a promising alternative or additive. substrate competition for dihydrofolate reductase.
Systemic and local chloroquine therapy needs Useful in erythrodermic, pustular, extensive
further evaluation. Miltefosine, 2.5 mg/kg/day for plaque psoriasis and psoriatic arthropathy.
28 days—a promising new oral therapy. Also used as an adjuvant to corticosteroids
in pemphigus and dermatomyositis. Dose:
Corticosteroids 0.2–0.4 mg/kg once a week. Toxicity: bone-
Revolutionary drugs in dermatologic therapeutics. marrow depression, hepatocellular damage and
Widely used in variety of dermatoses with high cirrhosis, mucosal ulcerations, gastrointestinal
morbidity or high mortality. Often a life saving haemorrhages. A close watch on liver functions
measure. Common indications: autoimmune bullous required; periodic liver biopsies needed once the
diseases (pemphigus, pemphigoid), collagen cumulative dose exceeds 1.5 g. Concomitant
vascular diseases (systemic lupus erythematosus, therapy with folic acid reduces toxicity.
zzAzathioprine: a 6-mercaptopurine derivative.
dermatomyositis, systemic sclerosis). Or
disseminated or severe dermatitis (air borne Used in pemphigus, systemic lupus
contact dermatitis). Or angioedema associated erythematosus, air borne contact dermatitis
with respiratory symptoms. Or cutaneous T cell and allergic vasculitis. Also an adjuvant in
lymphoma. Or severe drug eruptions. Role of dermatomyositis and pyoderma gangrenosum.
corticosteroids in Stevens Johnsons syndrome Dose: 3 mg/kg/day. Toxicity: bone-marrow
and toxic epidermal necrolysis is controversial- depression, hepatocellular damage and
many patients receive systemic steroids. Use infections.
in psoriasis (except impetigo herpetiformis) and zzCyclophosphamide: an alkylating agent.
atopic dermatitis is best avoided. Action: anti- Used in mycosis fungoides, pemphigus and
inflammatory, immuno-suppressive, antipruritic, mUltisystem histiocytosis. Dose: 5–10 mg/kg
keratoplastic or other ill-understood mechanisms. bolus dose monthly followed by more conservative
daily dose of 1–2 mg/kg/day. Toxicity: alopecia fungoides and histiocytosis. Dose: vinblastine:
(anagen effluvium), bonemarrow depression, 0.1 mg/kg/week with a weekly increment of
liver damage, hemorrhagic cystitis. Regular 0.05 mg until a remission is induced or
monitoring with blood counts and urine leukopenic response (commonest toxicity) is
examination. caused. Vincristine: 0.015–0.05 mg/kg or 2 mg/
zzMycophenolate Mofetil: It is a prodrug, inhibits sqm. Toxicity: neuropathy.
inosine monophosphate dehydrogenase
reducing purine synthesis and eventually Biological Agents
decreased T and B lymphocytes. Dosage is Substances derived from living organisms.
25–50 mg/kg/day, higher dose required for Of several types: fusion proteins (alefacept,
antibody mediated diseases. Not FDA approved etanercept, abatacept), monoclonal antibodies
for any dermatological indication, but may be (infliximab, adalimumab, rituximab, ustekinumab),
used wherever steroid sparing effect is needed. recombinant cytokines (Il-4, IL-10, IL-11, etc.) and
Commonly associated GI intolerance. immunotoxins (denileukin deftitox). Classification
zzCyclosporine: Works by inhibiting calcineurin, and indications of common biologics given in Table.
resulting in stoppage of IL-2 synthesis and
effectively, inhibition of T cell activation and Agents Causing Pigmentation
proliferation. Approved use in psoriasis and
atopic dermatitis. Widely used wherever steroid zzPsoralens (+ UVA = PUVA): Three chemicals:
sparing effect required. Dosage 2.5–5 mg/kg/day. 5-methoxypsoralen, 8-methoxypsoralen and
Step up regimen in stable cases and step down trimethoxypsoralen. All act as pigmentogenic
used in crisis or widespread disease. Common agents through their phototoxic potential, hence
side effects hypertension, hyperkalemia, need UVA (action spectrum of 320–400 nm
deranged renal function (urea, creatinine) and with peak at 360 nm) following application/
dyslipidemias. Not to be co-administred with ingestion. Improvement occurs with perifollicular
phototherapy as risk of cutaneous malignancy pigmentation in most but not all patients. Non-
may increase. hairy regions slow to repigment. Also effective in
zzVinca alkaloids: vincristine and vinblastine.
psoriasis due to antimitotic potential because of
Used intravenously in the treatment of mycosis formation of photoadducts of psolarens with DNA.
zzSystemic therapy: used for treatment of carbuncles need to be carefully incised and
extensive vitiligo, psoriasis, atopic dermatitis drained.
and plaque stage of mycosis fungoides. zzAmpicillin + clavulanic acid. Or cloxacillin (2 g/
8-methoxypsoralen (0.6 mg/kg) ingested day) in divided doses orally. Parenteral therapy
on alternate days—daily treatment causes may be needed in case of multiple carbuncles
cumulative phototoxicity. Followed 1–2 hours and if patient debilitated or immunosuppressed.
later, by gradually increasing exposures to To be continued for at least 2–3 days after
sunlight (PUVA sol) or to UVA sourced from subsidence of inflammation.
special chambers. Topical therapy: useful in
treatment of localised lesions of vitiligo and Dermatophytic Infections
palmoplantar psoriasis. Psora len cream, Tinea cruris/extensive T. corporis/T. faciei: any of
ointment or alcoholic solution is applied, on the following used:
alternate days, followed 30 minutes later, by zzAllylamines: terbinafine 1%, naftifine 1%.
gradually increasing exposures to sunlight Applied for 1–2 weeks.
or UVA. Side effects include mild to severe zzImidazole preparations: miconazole nitrate 2%,
phototoxicity. clotrimazole 1%, econazole 1%, ketoconazole,
2%. Applied twice a day for 4 weeks.
PRESCRIPTIONS FOR COMMON zzTolnaftate 1%: Applied twice a day for 4 weeks.
DERMATOSES zzHaloprogin 1%: Applied twice a day for 4 weeks.
Extensive T. corporis/recurrent T. cruris/T.pedis:
Superficial Bacterial Infections any of the following used:
zzTerbinafine: 250 mg daily for 2 weeks.
zzRemove crusts with soap and water washes.
zzItraconazole 200–400 mg daily for 1–2 weeks.
Or weak Condy’s lotion (1 in 8000–10,000
potassium permanganate aqueous solution) T. capitis: depending on type:
zzNon-inflammatory variety: terbinafine: 3–5 mg/
compresses or soaks.
zzTopical creams (not ointments) containing an
kg mg daily for 6 weeks.
zzInflammatory variety: itraconazole 5–10 mg/kg
antibiotic such as mupirocin or fucidic acid.
zzFor widespread infection: use systemic antibiotics
daily for 6 weeks.
like erythromycin (has advantage of covering T. ungiuim/onychomycosis: any of the following
both Staphylococcus and Streptococcus. used:
zzTerbinafine: 250 mg daily for 8 (finger nails)–12
zzIf staphylococcal infection suspected: preferably
use a penicillinase-resistant penicillin, such as (toe nails) weeks.
zzItraconazole 200 mg daily for 1 week every
cloxacillin or flucloxacillin
zzIn staphylococcal scalded skin syndrome (SSSS),
month for 12 weeks.
zzWhen distal 1/2 of single nail plate involved: topical
aggressive treatment, often with parenteral
antibiotics (cloxacillin. Or other penicillins in amorolifine 5% lacquer once a week or ciclopirox
combination with β lactamase inhibitors— olamine 8% lacquer daily for 6–9 months.
clavulanic acid or sulbactum) are used. In all types of dermatophytic infection, the area
should be kept dry. In case of recurrent infection,
Deep Bacterial Infections look for a reservoir of infection e.g., T. ungiuim.
zzRemove slough/crusts with 3–5% hydrogen Pityriasis Versicolor
peroxide or weak potassium permanganate
zzAlmost all topical anti-dermatophytic applications
solution.
zzAppropriate supportive treatment such as rest
are effective. Alternatively 2.5% selenium
and analgesics should be given. sulphide suspension may be used—apply at the
zzSimple furuncles—treated with moist or dry
time of bath, leave for 5–10 minutes and rinse
heat. Fluctuant furuncles or those ‘pointing’ and off. Or apply once or twice a week at bed time
and wash off the following morning. Frequent or zzIn case a bath is taken, re-apply medication soon
prolonged applications may cause local irritation. after.
zzKeep body dry. zzRoutine washing or cleaning of personal
garments and bed clothes. Expose to sunlight
Candidal Infections for 4–8 hours before laundering.
zzAll members of the family to be treated—even
zzFor localized infections: imidazole preparations:
miconazole nitrate 2%, clotrimazole 1%, those asymptomatic—at the same time.
econazole 1%, ketoconazole, 2%. Applied twice
a day for 4 weeks. Or nystatin cream or powder Pediculosis
(100,000 units/g). Pediculosis capitis
zzFor recurrent/extensive infections: oral treatment zzAny of the following may be used:
with itraconazole 200 mg daily for 1 week; ¾¾Permethrin 1% lotion left on the scalp for 8–12
fluconazole 150 mg once a week for 2 weeks. In hours is the treatment of choice because of its
recurrent/extensive infections—rule out diabetes safety profile and residual effect. Or shampoo
mellitus. Or an immunosuppressed state. left on, for 5–10 minutes.
zzKeep area dry. ¾¾Gamma benzene hexachloride 1%, rubbed
thoroughly and left overnight under a shower
Scabies cap; followed by shampoo; repeated after 7–10
Any of the following may be used: days. Alternatively, the hair may be washed
zzPermethrin 5% cream: single application for with gamma benzene hexachloride shampoo,
8–12 hours, after bath. Can be used in children to be repeated, if necessary, after a week.
(including neonates) and pregnant women. ¾¾Malathion 0.5% applied for 12 hours. Has
zzGamma benzene hexachloride 1% lotion/ residual effect which prevents reinfection for
cream: single application of 8–12 hours, after 6 weeks.
bath—but not hot water bath. Do not use in zzAll close contacts: to be treated at the same
children below 5 years of age. Nor in pregnant time. And combs and head wear treated with
women. lysol or pediculocidal agents.
zzBenzyl benzoate 10–25% emulsion: 3
Pediculosis pubis
applications at intervals of 8–12 hours. Lower
concentration in children and pregnant women. Single or two weekly applications of one of the
zzCrotamiton 10%: twice daily application for
above preparations. Important to treat sexual
1 week. Preferred treatment in children. partners. If eye brows/lashes involved: apply white
zzFor pruritis: antihistamines to be given for 1–2
petrolatum and remove nits and adult individually.
weeks, as itching persists for several days. Pediculous corporis
Crotamiton can also be prescribed for the same Important: improvement in personal hygiene-
period. regular soap and water baths. Disinfestation of
zzFor nodular and eczematised lesions: topical clothes with 10% DDT powder. Or gamma benzene
steroid in addition. hexachloride. Or simply hot iron.
zzFor secondary infection: a course of systemic
antibiotics—azithromycin for 3 days. Acute Exudative Dermatitis
Following instructions: should be carefully adhered
zzCondy’s lotion compresses/soaks/bath 2–3
to:
zzAll medications to be applied, after soap and
times a day.
zzTopical application of 1% aluminium acetate
water scrub bath, all over the body, below the
neck. Special care to be given to genitals and or silver nitrate—both are astringents and so
subungual region. reduce exudation.
Psoriasis of scalp: betamethasone dipropionate zzCyclosporine can also be used (@2.5–5 mg/
lotion. Or a steroid foam preparation to be used at kg/day). Usually given as step down regimen-
night. Shampoo next morning. started at full dose (5 mg/kg/day), gradually
tapered to 2.5 mg/kg/day over several weeks,
Psoriasis involving 10–50% BSA followed by either maintanence dose or stopping
Phototherapy/photochemotherapy treatment of and switching over to conventional therapies. Not
choice. Phototherapy involves irradiation of skin with recommended to combined with phototherapy
sunlamps that emit UVB, while photochemotherapy or methotrexate. Monitor for deranged renal
(PUVA) involves administration of psoralen, function, dyselectrolytemia, hypertension and
followed 2–3 hrs later, by exposure to black light dyslipidemia.
lamps (that emit UVA). Or sunlight (PUVA sol). zzCare, often inpatient, required in patients with
Chronic phototoxicity: photo-oncholysis in patient on long- Chronic phototoxicity: thickening and scaling over a vitiligo
term PUVA therapy. patch after long-term oral PUVA treatment.
Phototesting
Exposure of the skin to incremental doses of UV
or visible radiation followed by recording of skin
responses. Uses:
zzDiagnostic: in suspected photosensitivity,
calculation of minimal erythema dose (MED),
photoprovocation.
zzTherapeutic: MED/minimal phototoxic dose
Phototesting template: opaque template with coverable
(MPD) calculation for appropriate phototherapy/ windows.
photochemotherapy.
THERAPEUTIC PHOTOMEDICINE
Principle
Phototherapy (using UVB-290–320 nm; UVA1-
340–400 nm) and photochemotherapy (using
psoralens with UVA, 320–400 nm, so termed
PUVA) well-established treatment modalities in
dermatology. Main use in psoriasis and vitiligo.
Application broadened in recent years. Mechanism
of action shown in table. Further, the mechanisms
common to both UVA and UVB include-increased
regulatory T-cells and reduced effector T-cells,
locally increased levels of IL-10, decreased
keratinocyte expression of CD-54 (ICAM-1) which
Courtesy: Dr. Prakash Khute, Department of Dermatology, AIIMS, reduces T-cell binding to keratinocytes. These
New Delhi.
Photochemotherapy (PUVA and PUVA sol)
PUVA
Method: systemic, psoralens (8-methoxypsoralen
at 0.4–0.6 mg/kg, trimethylpsoralen at 0.6–1.2 mg/kg
and 5-methoxypsoralen at 1.2–1.8 mg/kg)
administered 1 Y2-2 hours before UVA exposure,
in especially designed chambers fitted with
fluorescent tubes. Systemic psoralens generally
prescribed when disease extent is >10% body
surface area. Topical, psoralens applied locally
(solution, ointment, cream or bath) Yz hour before
UVA exposure. For both-starting dose of UVA, 70%
Contraindications: pregnancy, children (< ease and uniformity of application, and less UVA/
12-years), photosensitivity, severe cardiac, hepatic sun-exposure dose required.
or renal disease, history of skin cancers, aphakia or
cataracts and immunosuppressed states. Turban PUVA/PUVA Sol
Complications: systemic PUVA—nausea, photo- Modification in which a cloth is soaked in diluted
toxicity (pruritus, erythema, edema and blistering), 8-MOP solution and worn as a turban on scalp
skin thickening and scaling (esp in vitiligo patches followed by sun-exposure/UVA. Helpful in case of
which tend to become plaques with chronic scalp psoriasis, may help scalp vitiligo.
exposure), prematurely aged skin and cutaneous
malignancies (no risk reported in skin types 4–6, no Turbo PUVA
Indian reports till date) and cataracts. Topical PUVA:
phototoxicity, photoallergic contact dermatitis, Modification of oral PUVA studied for psoriasis.
hyperpigmentation, rarely hypertrichosis. Sunless tanning agent dihydroxyacetone (DHA)
is applied all over body. And usual course of oral
PUVA sol (psoralens + solar exposure) PUVA started. DHA was noted to peel off later
Method: Direct sunlight used instead of UVA from the normal skin than psoriatic plaques, hence
chamber. Systemic therapy, affected parts exposed protecting the normal skin. Thus UVA could be
to direct sunlight (best between 11 AM and 2 PM), given at high doses leading to quicker remission
2 hours after ingestion of psoralens (dose, vide and better skin tolerance than in those patients
supra). Initial exposure 2 minutes, increased who did not use DHA.
gradually to maximum of 15 minutes (as tolerated).
Topical therapy, psoralens (0.01–0.1% solution TARGETED PHOTOTHERAPY
of 8-MOP; usually 1% solution is diluted 30–40
times in propylene glycol) applied Yz hour before Implies the use of various technologies which
sun exposure—for 1 minute initially, gradually deliver the phototherapy only to the lesion and
increasing to 5 minutes (as tolerated). Repeat sparing the uninvolved skin. It includes: excimer
every alternate day. laser, photodynamic therapy, intense pulse light
Indications: as for PUVA therapy. Effective, therapy, low-level laser and light emitting diode
cheap, home-based ecofriendly treatment, if therapy.
patient can expose conveniently. When compared
with PUVA for psoriasis, it is more cost-effective Excimer Laser
with almost similar efficacy. When compared to Delivers a specific wavelength (308 nm) of UVB
PUVA for vitiligo, it is slower in repigmenting, less radiation. Focused excimer laser/ light over
efficacious and produces less improvement in localized lesions. Xenon chloride is lasing medium.
quality of life parameters. Usual spot size of 1x1 cm, energy of around 3
mj/cm2 per pulse at frequency of up to 200 Hz.
Bath PUVA and Bath Suit PUVA/PUVA Sol Able to treat small lesions or large lesions over
A variation in topical PUVA/PUVA sol. In bath PUVA, multiple sittings. Found useful in both vitiligo and
solution prepared by mixing 50 mL of psoralen psoriasis, in previously resistant lesions as well.
solution (8-MOP) with 100 liters of water. Patient Tested useful in other dermatoses like oral lichen
soaks in this solution for 15 minutes followed planus, alopecia areata, topic dermatitis, mycosis
by sun-exposure or UVA chamber. In bath suit, fungoides, etc. But lesions at poorly responsive
0.8 mL of 8-MOP solution added to 2 liters of water sites do not respond as well. Fairly expensive
and bathing suit soaked in it. After squeezing out machine and not so impressive response. Only
excess water, suit is worn for 15 minutes, then it advantage is sparing of normal skin thus reducing
is removed and body is exposed to sun or UVA. long-term risk of malignancy in fairer skin types
Advantages—no systemic psoralen side effects, 1–3. Side effects include-local erythema, crusting,
blistering.
Linear morphea with hemifacial atrophy, at baseline. Linear morphea with hemifacial atrophy, 7 days after autologous
fat injection. Note filling of the forehead defetcs and fullness of
the medial right cheek which was depressed at baseline.
Cosmetic Dermatology 467
Vitiligo: pre-treatment photograph. Vitiligo: after treatment with combination of blister and punch
grafting.
Cosmetic Dermatology 469
Mark Rubin’s classification of peeling agents based on depth and examples of agents achieving the depth
Depth of penetration Chemical peeling agents
Glycolic acid 20–50%
Salicyclic acid 20–30%
Very superficial/stratum corneum exfoliation Resorcinol 20–30% (5–10 min)
Jessner’s solution (1–3 coats)
Trichloroacetic acid 10% (1 coat)
Phenol 88%
Glycolic Acid 70% (5–30 min)
Medium-depth/papillary dermal necrosis Trichloroacetic acid 35% in combination with:
Glycolic acid 50–70%
Solid CO2
Jessner’s solution
Neurofibroma, right lower eyelid: pedunculated lesion, Neurofibroma, right lower eyelid: 10 days after complete
0.8–1 cm sized. excision.
Cosmetic Dermatology 471
Final outcome 3 months after surgery. Island pedicle flap sutured in place.
Cosmetic Dermatology 473
do not give permanent results. Follow-up laser Methods: excision, dermabrasion, infrared
sittings always needed every 3–6 months. coagulation, salabrasion, grafting, cryosurgery
and Moh’s surgery.
Vitiligo Surgery Liposuction
Variety of surgical procedures conducted for
Safe and effective technique to improve body’s
vitiligo patches on cosmetically concerning areas. contours and rearrange distribution of adipose
Common ones include-micro-punch grafting, tissue without altering skin surface. Tumescent
suction blister grafting, non-cultured epidermal technique: infiltration of large volumes of
cell suspension, ultra thin split thickness skin vasoconstrictive anesthetic solution and removing
grafting. A new technique of follicular outer root adipose tissue through tiny holes using cannulas.
sheath cellular suspension has been developed Indications: lipomas, lipodystrophy and axillary
with encouraging results. Surgery performed only hyperhidrosis. Not a treatment for obesity.
if lesions have been stable for at least 1 year. Complications: ecchymosis, hematoma and
infection.
Tattooing and its Removal
Indications for tattooing: Reconstructive Surgical Pearls
Medical: camouflaging burns by micro- Aim to produce esthetically pleasing ideal scar
pigmentation. Producing permanent dots for that blends imperceptibly with natural creases and
radiation therapy. folds. Techniques: placing subcuticular sutures,
Non-medical indications: esthetic reasons, punch and blister grafting, reconstruction using
peer pressure, to provoke and enhance sexuality. flaps (e.g. island pedicle flap) and scar revisions
Dermatologists approached for tattoo removal. using W-plasty, Z-plasty and V-V plasty.
25 Lasers in
Dermatology
Fractional CO2 laser (10600 nm). Long pulsed diode laser (810 nm).
Q switched Nd:YAG laser (1064 nm). Pulse dye laser (595 nm).
CLINICAL TERMS
Macule: a circumscribed area of change of color,
not raised above or depressed below the surface
of adjoining skin
umbilicated papules
lichenoid papules
(note Wickham’s
depigmented and brownish striae on the larger
hyperpigmented macules lesion)
erythematous macule
edematous plaque
hemorrhagic crust honey-colored crust
Vesicle: a small, circumscribed lesion elevated Scale: a sheet of adherent epidermal cells
above the surface of skin containing clear fluid
Ulceration: a break in the continuity of skin Eschar: Thick adherent crust covering an
underlying ulcer.
Chromomycosis 34f, 35, 37f Cubital fossae 102f Darier’s disease 290f, 291, 291f
etiology 35 Cushing’s syndrome 200f, 202f, De Sanctis-Cacchione syndrome
Cicatricial alopecia 24f, 174f, 294f 203 309
secondary to discoid lupus Cutaneous amyloidosis, primary 185 Decubitus ulcer 252f, 253
erythematosus 224 Cutaneous horn 342f, 343 Deep necrotic ulcers 382f
Cicatricial alopecias 271 Cutaneous infiltration, mild diffuse De-novo-tumor D’emblee 367
classification of 273t 56f Dense mixed infiltrate of
Cidofovir 442 Cutaneous larva migrans 95f eosinophils 91f
Circinate balanitis 148f Cutaneous LE, chronic 223 Dense nodular lymphoid aggregate
in HIV-positive 406f Cutaneous leishmaniasis 91, 91f 429f
Cladophialophora 35 acute 88f, 89 Depigmented irregular edged
Clindamycin 267 chronic 88f, 89 macule 336f
Clofazimine 69, 446 Cutaneous lymphoid hyperplasia Depigmented scar 106f
Cobblestone appearance 304f 426f, 427, 429f Dermabrasion, indications of 471
Cold urticaria 121 Cutaneous manifestations 399 Dermal leishmanoid 89
Cold, severe 243 Cutaneous mastocytosis, diffuse Dermal melanocytic nevus 334f
Collagen sclerosis 231f 375 Dermal mucin, abundant bright
Collagenosis, reactive perforating Cutaneous mucinoses 423 blue 423f
428f, 429 classification of 423t Dermal tumors 341, 349
Collodion baby 285 Cutaneous myiasis 93, 93f Dermatitis 99, 109
Comedo 481 Cutaneous pseudolymphomas 425 acute exudative 450
formation 257 classification of 425t artefacta 114f, 117
Comedone 257 Cutaneous T cell lymphoma 189, contact 111f
closed 257 365 herpetiformis 177, 178f, 179f
open 481f Cutaneous tuberculosis herpetiformis 179f
Condyloma acuminata 43, 44f, and true tuberculides 79 infective 113
388f
epidemiology 73 occupational 109
in male 388f
etiology 73 Dermatology
Condylomata lata 379, 382f
investigations 77 common lasers used in 478t
Conidiobolus coronatus 33
treatment of 79 premalignant conditions in 355t
Connective tissue, disorders of 303
Cutaneous tumors 339 Dermatomyositis 236f, 237, 238f,
Contact leukoderma 210f, 212f
Cutaneous vasculitis 130f, 131 239t
Contact, urticarial 121
Cutaneous xanthomatoses 183 Dermatophyte infection in HIV-
Cornoid lamella 289, 289f
Cutis hyperelastica 305 positive 398f
Corticosteroids 442, 447, 452
Cutis verticis gyrata 313, 333 Dermatophyte, culture of 25f
Corynebacterium minutissimum 11
of scalp 314f Dermatophytic infection 403, 449
Corynebacterium spp 13
Cyclophosphamide 447 in HIV-positive 400f
Cosmetic 109
dermatitis, pigmented 153 Cyclosporine 251, 448 Dermatophytoses 19
dermatology 465 Cylindroma 347, 347f etiology 19
Cough 197 Cyst, epidermal inclusion 351 treatment 25
Cowden disease 303, 304f Cysticercosis 95 Dermatosis papulosa nigra 340f,
Crab louse 87f of skin 94f 341
Creams pastes 441 Cysticercus cellulosae 95 Dermis sparing epidermis 429f
Creeping eruption 95, 96f Cytopathology 51 Dermographism 121
Crotamiton 442, 450 Dermoscopy 45
Crusted ulcers, multiple 432f D in molluscum contagiosum 45f
Cryoglobulinemic vasculitis 245 Diabetes
Cryptococcosis 36f, 37 Dactylolysis spontanea 435 insipidus 371
Cryptococcus neoformans 37, 39f Dapsone 69, 446 mellitus 269
culture of 37f Darier sign 372f Diabetic bulla 198, 199
Diabetic dermopathy 198, 199 herpeticum 49, 50f Epithelioma cuniculatum 364
Diabetic shin spots 199 infective 112f Eroded dermatitis 96f
Diaminodiphenyl sulphone 446 Eczematoid dermatitis, infectious Eruptive histiocytosis 370f, 373
Diaper dermatitis 115 113 Eruptive xanthomas 182f, 183
Diffuse and limited systemic Edematous lesion with vesiculation Erysipelas 7
sclerosis, difference 6f Erythema ab igne 243, 244f
between 233t Edematous nodules 88f Erythema and
Discoid lupus erythematosus 223 Edematous plaque 482f edema of upper and lower
chronic 222, 222f, 225f Ehlers-Danlos syndrome 304f, 305 eyelids 236f
lichen planus overlap 224f EIA, principle of 407f peripheral vesiculation 168f
plaque on buccal mucosa 224f Elastosis perforans serpigenosa Erythema annulare centrifugum
with squamous cell carcinoma 428f 126f, 127
224f Elejalde syndrome 311, 312f Erythema chronicum migrans 126f,
Dome-shaped papules 481f Emulsions 440 129
Donovan bodies 385f Endogenous infection 73 Erythema dose, minimal 444, 457
Donovanosis 384f, 385f, 387, 405 Endothelin receptor antagonist 235 Erythema dyschromicum perstans
Dowling Degos disease 218f, 219, Enzyme immunoassay 409 153, 215, 216f
219f Eosinophilic cells 345f Erythema elevatum diutinum 132f,
Dracunculosis 95, 96f Eosinophilic folliculitis 403 133
Drug eruption 128f, 129 in HIV-positive 404f Erythema gyratum repens 127
fixed 129, 130f, 131, 414f Eosinophilic intracytoplasmic bodies Erythema induratum 77
photosensitive 129 47f Erythema marginatum rheumaticum
Drug hypersensitivity reaction 412 Epidermal atrophy 235f 128f, 129
Drug induced lupus 229 Epidermal layers, upper 291f Erythema multiforme 49, 122f, 123,
Drug regimens 415 Epidermal necrolysis 125t 123t, 124f, 125f, 170f
Epidermal origin, cysts of 351 Erythema nodosum 77, 78f, 79f
Drug-induced acne 258f
Epidermis, atrophic 65f leprosum 63, 68, 68f
Dry discoid
Epidermis, atrophy of 225f, 247 necroticans 68f
dermatitis 105
Epidermis, degeneration of 227f like lesion 134f
eczema 102
Epidermodysplasia verruciformis Erythematous border 286f
Dyskeratosis congenita 309, 310f
43, 46f, 313, 396 Erythematous halo, peripheral 130f
Dyskeratotic 355
Epidermoid cyst 351, 351f Erythematous hyperkeratotic
cells 291f, 293f, 355f
Epidermolysis bullosa 292f, 293, plaques on palms 406f
Dyspnea 197
294 Erythematous macule 481f
Dystopia canthorum 308f
acquisita 352f Erythematous nodule 8f, 88f, 426f
E dominant dystrophic 294f, 295 Erythematous papules 78f, 424f
dystrophica 295f Erythematous papulovesicles 106f
Ear 263 junctional 295 Erythematous plaque 172f
EB pruriginosa 295, 296f recessive dystrophic 297 on cheek 370f
Eccrine gland 257 simplex 292f, 293, 295f Erythematous shiny nodules over
Eccrine hidrocystoma 344f types of 295t cheek and nose 426f
Eccrine poroma 344f, 345 Epidermolytic hyperkeratosis 284f, Erythematous small papules 142f
Eccrine spiradenoma 346f, 347, 285f Erythematous verrucous plaques
347f Epidermolytic ichthyosis 282f, 285 222f
near outer canthus 302f Epidermolytic verrucous epidermal Erythemo-telangiectatic rosacea
Ecthyma 2f, 3, 5 nevus 326f 262f
etiology 3 Epidermophyton 17, 19 Erythrasma 12f
hemorrhagic crusts 2f Epidermotropism 369 Erythroderma 116f, 117, 117t, 129,
Ectodermal dysplasias 297 Episodes, primary 49 143
Eczema 99 Epitheliod cells 65f Erythrodermic psoriasis 141
craquelé 115 Epithelioid hemangioma 427 Erythrodontia 190f, 193
lamellar 282, 285 Intravenous drug users 393 Keratotic nodules 114f
linearis circumflexa 286f Irritant contact dermatitis 106, 107 Keratotic papules 356f, 428f
nigra 285 Island pedicle flap sutured in place over nape of neck 312f
vulgaris 282f, 283 472f Keratotic plaque over capillary
Idiopathic photodermatosis, Isoniazid 79 malformation, left leg 322f
treatment of 249 Isonicotinic acid hydrazide 205 Keratotic plugs 225f, 235f
Idoxuridine 442 Isotretinoin 217, 451 Keratotic protruberance in neck 44f
IgA bullous dermatosis, linear 177 Itchy 327 Keratotic umbilicated papules on
IgA pemphigus 165, 167, 168f Itraconazole 25, 445 dorsa of hands 428f
IgE syndrome, hyper 313, 314f Ivermectin 442 Ketoconazole 446
Imiquimod 442 Kimura’s disease 427t
Immunofluorescence of bullous J Kindler syndrome 297
pemphigoid, direct 171f Klebsiella granulomatis 387
Immunofluorescence, direct 225 Jarisch-Herxheimer reaction 381 Klippel-Trenaunay syndrome 323
Impetigo contagiosa 2f, 3, 3f Jessner’s lymphocytic infiltrate Koebner’s isomorphic phenomenon
etiology 3 426, 426f, 429 139, 149
investigations 3 Jewels appearance, cluster of 175 Koebner’s phenomenon 148f
Incontinentia pigmenti 304f, 305, Jigsaw puzzle appearance 347f Koenen’s tumors 299
306f Job syndrome 313 Koilonychia 277
bullous stage 305f Junctional nevi 333 Kojic acid 444
Increased pigmentation, disorders Juvenile Koplik’s spots 47
of 213 and adult dermatomyositis, Kwashiorkor 202f, 203
Infantile 101 differences between 239t Kyphoscoliosis 298f
capillary hemangioma 319, 319f dermatomyositis 236f Kyrle’s disease 429
hemangioma 318f elastomas 331
and vascular malformations, xanthogranuloma 370f, 373
L
differences between 321f
superficial type 318f K Labia minoria 234f
seborrheic dermatitis 105 Kaposi’s sarcoma 403f, 405, 408f Lacazia loboi 37
Inflammatory swellings, multiple 22f Kaposi’s varicelliform eruption 49 Lamivudine 415
Ingenol mebutate 445 Keloid 259, 348f, 349 Langerhans’ cell 373f
Inguinal lymphadenopathy 384f over shoulder, massive tumor- histiocytosis 368f, 371, 371f,
Inherited tumors 297 like 348f 373f
Insect bite 95 Keloidal and ulcerative plaques 36f Laser surgery, principles of 476
hypersensitivity 94f, 403 Keloidal blastomycosis 37 Laser therapy
in HIV-positive 402f Keloidal morphea 232f in dermatology 479t
Intracytoplasmic molluscum bodies Keloids and hypertrophic scars, indications for 478
47f differences between 349t Late symptomatic disease 399
Intradermal nevus 333f Keratinizing disorders 283 LCH, treatment of 373
Intraepidermal clefts, irregular 293f Keratoacanthoma 341, 343f, 437 Leaf macules 299
Intraepidermal lymphocytic Keratoderma blenorrhagicum 148f Leiomyoma 349
aggregates 369f in HIV-positive 406f Leishmaniasis 89, 403
Intraepidermal neutrophilic Keratodermic 157 causative agents of 89t
IgA dermatosis 165 Keratosis follicularis 291 disseminated 89
pustule 145f Keratosis lichenoides chronica recidivans 89
Intraepidermal neutrophilic type 165 154f, 155 Leishmanin test 91
Intra-epidermal split 291f Keratosis of palms, punctate 356f Le-non-specific skin disease 223t
Intra-epidermal spongiotic vesicles Keratosis, arsenical 356f, 357 Lentigines 331
containing eosinophils Keratotic follicular papules 156f, Lentigo maligna 357
305f 202f melanoma 358f, 365
Staphylococcal scalded skin Swarm of Bees’ appearance 269 Tinea unguium 21, 24f, 276f
syndrome 4f, 5, 7, 445 Sweet’s syndrome 420f, 421, 421f, Tissue diseases, connective 221
Staphylococcus aureus 3, 275, 422f TNM staging of mycosis fungoides
396f Sycosis barbae 6f 367t
Startified squamous epithelial lining Symblepheron 174f Toe nail 278f
353f Syphilis 379, 405, 410f ingrowing 252f, 253
Startum corneum 365f congenital 381 Tolnaftate 449
Stasis eczema 107 early 381 Topical calcineurin inhibitors 443
Stasis ulcer 106f late 379, 381 Topical PUVA 452
Stavudine 415 primary 378f, 379 Topical therapy 87, 103, 117, 440
Steatocystoma 303f secondary 378f, 379, 380f Touton giant cells 185, 185f
multiplex 302f, 303 tertiary 381 Toxic epidermal necrolysis 124,
Stratified squamous epithelium Syringocystadenoma papilliferum 126f, 127f, 178f, 226f, 412f
351f 328f, 329 Transglutaminase, epidermal 177
Stratum corneum 25f, 85f Syringomas 343, 343f, 344 Treponema pallidum 379, 379f,
Strawberry hemangioma 319 Systemic amyloidosis, primary 381
Strep pyogenes 3, 7, 9 186f, 187, 188f Tretinoin 259, 267
Streptococcal infections 11, 445 Systemic lupus erythematosus Trichilemmal cyst 352f, 353, 353f
Streptococcus pyogenes 3 227f Trichoepithelioma 301, 301f, 302,
Striate palmoplantar keratoderma 344f, 345
286f T Trichomonas vaginalis 383
Sturge-Weber syndrome 318f Trichophyton 17, 19
Subacute cutaneous lupus T. cruris 25 concentricum 19
erythematosus 228f, 229 T. pedis 25 Trichorrhexis nodosa 430f, 431
Subcorneal pustular T. vaginalis 407 Trichotillomania 268f, 269, 270
dermatosis 165 Taenia solium 95 Trigeminal zoster 52f
type with hypopyon 168f Target cells, entry into 395 Trigger, identification of 125
Subcorneal split containing Telangiectasia 483 Tripe palms 189
polymorphonuclear Telangiectatic blood vessels 200f Trophic ulcer 64f
leukocytes 3f Telangiectatic nevus port wine stain Tuberculin skin test 77f
Subcutaneous phycomycosis 33f 321 Tuberculin test 77
Subcutaneous zygomycosis 32f, Telogen effluvium 271 Tuberculoid curvilinear granuloma
33 Temperature change, disorders to 65f
Subcutis tumors 341 243 Tuberculoid granulomas 67f
Subepidermal bulla 171f Tendinous xanthoma 183, 184f Tuberculoid leprosy 57, 59, 60f,
Succulent centrofacial nodules 90f Tenofovir 415 65, 65f
Sudden friction, acute 243 Tense bulla 176f, 482f borderline 67f
Sunburn 245 Terbinafine 25, 445 Tuberculosis 401
Superficial basal cell carcinoma Tetracyclines 265, 275 verrucosa cutis 74f, 75, 76f
361f Textile dermatitis 109 Tuberculous gumma 73, 75, 76f
Superficial folliculitis 7 Tgases 177 Tuberous sclerosis 299, 300f, 301f
Superficial spreading melanoma Tinea barbae 21, 24f Tuberous xanthomas 183, 184f
365 Tinea capitis 21, 22f-24f, 25 Tumor 482
Suppress hematopoiesis 195 Tinea corporis 18f, 19, 20f, 398f Tumor nodule of basaloid cells
Suprabasal intraepidermal split with Tinea cruris 19 347f
acantholysis 167f Tinea faciei 18f, 19 Tumor syndromes 297
Suprapapillary thinning of Tinea imbricata 18f, 19 Tumors, benign
epidermis 143f Tinea incognito 19, 20f, 400f appendageal 343
Surrogate markers 415 Tinea manuum 20f, 21 vascular 353
Sutton’s nevus 211 Tinea pedis 20f, 21, 22f, 25f Tumors, epidermal 341