Andrews’ Diseases of the Skin: Clinical Dermatology

1035 Pages·2015·156.13 MB·English

by  William D. James

Andrews’
Diseases

Skin
of
the
CLINICAL DERMATOLOGY
Executive Content Strategist: Russell Gabbedy
Senior Content Development Specialist: Ailsa Laing
Publishing Services Manager: Patricia Tannian
Senior Project Manager: John Casey
Designer: Christian Bilbow
Original cover images: Upper left: Dr. Donald Adler (deceased);
Upper right: Dr. Shyam Verma; Center: Dr. Debabrata Bandyopadhyay;
Lower right: Dr. William D. James.
Andrews’
Diseases
Skin
of
the
CLINICAL DERMATOLOGY
Twelfth Edition
William D. James, MD Timothy G. Berger, MD
Paul R Gross Professor of Dermatology Professor of Clinical Dermatology
Department of Dermatology Executive Vice Chair and Residency
University of Pennsylvania School of Medicine Program Director
Philadelphia, Pennsylvania Chair in Dermatology Medical Student
Education
Dirk M. Elston, MD University of California, San Francisco
San Francisco, California
Professor and Chairman
Department of Dermatology
and Dermatologic Surgery
Medical University of South Carolina
Charleston, South Carolina;
Former Director
Ackerman Academy of Dermatopathology
New York, New York
1600 John F. Kennedy Blvd.
Ste. 1800
Philadelphia, PA 19103-2899
ANDREWS’ DISEASES OF THE SKIN: CLINICAL DERMATOLOGY,
TWELFTH EDITION ISBN: 978-0-323-31967-6
International Edition ISBN: 978-0-323-31968-3
Copyright © 2016 by Elsevier, Inc. All rights reserved.
11th edition © 2011 by Elsevier, Inc.
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Notices
Knowledge and best practice in this field are constantly changing. As new research and
experience broaden our understanding, changes in research methods, professional practices,
or medical treatment may become necessary.
Practitioners and researchers must always rely on their own experience and knowledge
in evaluating and using any information, methods, compounds, or experiments described
herein. In using such information or methods they should be mindful of their own safety
and the safety of others, including parties for whom they have a professional responsibility.
With respect to any drug or pharmaceutical products identified, readers are advised to
check the most current information provided (i) on procedures featured or (ii) by the
manufacturer of each product to be administered, to verify the recommended dose or
formula, the method and duration of administration, and contraindications. It is the
responsibility of practitioners, relying on their own experience and knowledge of their
patients, to make diagnoses, to determine dosages and the best treatment for each
individual patient, and to take all appropriate safety precautions.
To the fullest extent of the law, neither the Publisher nor the authors, contributors, or
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methods, products, instructions, or ideas contained in the material herein.
International Standard Book Number: ISBN: 978-0-323-31967-6
Printed in China
Last digit is the print number: 9 8 7 6 5 4 3 2 1
VIDEO CONTENTS
Videos available at expertconsult.inkling.com
37-1 Shave biopsy
37-2 Punch biopsy
37-3 Narrow hole lipoma excision
37-4 Excision
37-5 Fusiform excision
37-6 Transposition flap
37-7 Split thickness skin graft
37-8 Mohs surgery
38-1 Full face ablative resurfacing
39-1 Filler, lips
39-2 Botulinum toxin, glabella
39-3 Botulinum toxin, frontalis
39-4 Starch iodine test for hyperhidrosis
39-5 Botulinum toxin, hyperhidrosis axilla
39-6 Sclerotherapy
39-7 Foam sclerotherapy
PREFACE AND ACKNOWLEDGMENTS
Andrews’ remains as it was from the beginning: an authored ular investigative techniques, technologic breakthroughs, and
text whose one volume is filled with clinical signs, symptoms, designer therapeutics lead the way in providing advances in
diagnostic tests, and therapeutic pearls. The authors have our specialty. We cover the new understanding following
remained general clinical dermatologists in an era of subspe- from such innovations by discussing the mechanisms at work
cialists in academia. They are committed to keeping Andrews’ in genetic diseases, covering the latest in dermatopathologic
as an excellent tool for anyone who needs help in diagnosing staining and analysis, and enlarging the therapeutic recom-
a patient with a clinical conundrum or treating a patient with mendations to include our expanded therapeutic options, such
a therapeutically challenging disease. as biologic response modifiers and biologically engineered tar-
Andrews’ is primarily intended for the practicing dermatolo- geted medications. We have attempted to define therapeutics
gist. It is meant to be used on the desktop at his or her clinic, in a fashion that emphasizes those interventions with the
giving consistent, concise advice on the whole spectrum of highest level of evidence, but also present less critically inves-
clinical situations faced in the course of a busy workday. While tigated therapeutic options. To care for our patients we need
we have been true to our commitment to a single-volume a large array of options. Not all are fully supported by formal
work, we provide our text in a convenient online format as evidence, yet are helpful to individual patients.
well. Because of its relative brevity but complete coverage of Extensive revisions were necessary to add this wealth of new
our field, many find the text ideal for learning dermatology information. We selectively discarded older concepts. By elimi-
for the first time. It has been a mainstay of the resident yearly nating older, not currently useful information we maintain the
curriculum for many programs. We are hopeful that trainees brief but complete one-volume presentation that we and all
will learn clinical dermatology by studying the clinical descrip- previous authors have emphasized. Additionally, older refer-
tions, disease classifications, and treatment insights that define ences have been updated. The classic early works are not cited;
Andrews’. We believe that students, interns, internists or other instead we have chosen to include only new citations and let
medical specialists, family practitioners, and other health pro- the bibliographies of the current work provide the older refer-
fessionals who desire a comprehensive dermatology textbook ences as you need them. A major effort in this edition was to
will find that ours meets their needs. Long-time dermatolo- reillustrate the text with hundreds of new color images. Many
gists will hopefully discover Andrews’ to be the needed update have been added to the printed text; you will also find a
that satisfies their lifelong learning desires. On our collective number only in the online version. Enjoy! We have looked to
trips around the world, we have been gratified to see our our own collections to accomplish this. These are the result of
international colleagues studying Andrews’. Thousands of many hours of personal effort, the generosity of our patients,
books have been purchased by Chinese and Brazilian derma- and a large number of residents and faculty of the programs
tologists alone. in which we currently work or have worked in the past. Addi-
Many major changes have been made to this edition. Bill tionally, friends and colleagues from all parts of the globe have
James, Tim Berger, and Dirk Elston, three great friends of over allowed us to use their photographs. They have given their
three decades, have worked closely to continue to improve the permission for use of these wonderful educational photos to
quality of our text. The surgical chapters have been updated enhance your understanding of dermatology and how skin
and expanded by Isaac Neuhaus. He has added videos of some diseases affect our patients. We cannot thank them enough.
of the most common procedures, which are available online. All of the authors recognize the importance of our mentors,
We thank him for his continued work to improve this portion teachers, colleagues, residents, and patients in forming our
of our textbook. Robert Micheletti expertly updated Chapters collective expertise in dermatology. Dirk, Tim, and Bill were
29 and 35. He is an internist/dermatologist with superior all trained in military programs, and our indebtedness to this
writing skills whose contributions are most appreciated. We fellowship of clinicians is unbounded. The many institutions
have tried to ensure that each entity is discussed only once, in we have called home, from the East Coast of Walter Reed, the
a complete yet concise manner. In order to do this we have University of Pennsylvania, and Geisinger Medical Center, to
had to make decisions regarding the placement of disease the West Coast of the University of California at San Francisco,
processes in only one site. Clearly, neutrophilic eccrine hidrad- and many in between, such as Brooke in San Antonio and the
enitis, for example, could be presented under drug eruptions, Cleveland Clinic, nurtured us and expanded our horizons.
neutrophilic reactive conditions, infection or cancer-associated Our friendship goes well beyond the limits of our profession;
disease, or with eccrine disorders. The final decisions are a it is wonderful to work with people you not only respect as
team effort and made in the interest of eliminating redun- colleagues, but also enjoy as closely as family. Barbara Lang
dancy. This allows us to present our unified philosophy in and Laura Beckerman provided expert assistance throughout
treating patients in one dense volume. the revision process to Bill and Tim, respectively. We are
Medical science continues to progress at breakneck speed. indebted to their hard work. Finally we are proud to be a part
Our understanding of the etiology of certain conditions has of the Elsevier team and have such professionals as Ailsa
now led us to recategorize well-recognized disease states and Laing, John Casey, and Russell Gabbedy supporting us every
dictated the addition of many newly described entities. Molec- step of the way.
vii
DEDICATION
The authors (left to right): Tim Berger, Bill James, Dirk Elston
For my family, whose love and support sustain me and make me happy.
WDJ
My wife, Jessica, and my children, Olivia and Mateo, who give me the joy and
strength to undertake such a task.
TGB
To my wife and best friend, Kathy, and our wonderful children, Carly and Nate.
DME
viii
CONTRIBUTORS
Isaac M. Neuhaus, MD
Associate Professor
Dermatologic Surgery and Laser Center
University of California, San Francisco
San Francisco, California
Robert G. Micheletti, MD
Assistant Professor of Dermatology and Medicine
University of Pennsylvania
Perelman School of Medicine
Philadelphia, Pennsylvania
ix
Bonus images for this chapter can be found online at 
expertconsult.inkling.com
1 
Skin: Basic Structure and Function
Skin is composed of three layers: the epidermis, dermis, and consist of two subfamilies, acidic and basic. The product of one
subcutaneous fat (panniculus) (Fig. 1-1). The outermost layer, basic and one acidic keratin gene combines to form the mul-
the epidermis, is composed of viable keratinocytes covered by tiple keratins that occur in many tissues. The presence of
a layer of keratin, the stratum corneum. The principal compo- various keratin types is used as a marker for the type and
nent of the dermis is the fibrillar structural protein collagen. degree of differentiation of a population of keratinocytes. Ker-
The dermis lies on the panniculus, which is composed of atins are critical for normal functioning of the epidermis, and
lobules of lipocytes separated by collagenous septa that contain keratin mutations are recognized causes of skin disease. Muta-
the neurovascular bundles. tions in the genes for keratins 5 and 14 are associated with
There is considerable regional variation in the relative thick- epidermolysis bullosa simplex. Keratin 1 and 10 mutations are
ness of these layers. The epidermis is thickest on the palms associated with epidermolytic hyperkeratosis. Mild forms of
and soles, measuring approximately 1.5 mm. It is very thin on this disorder may represent localized or widespread expres-
the eyelid, where it measures less than 0.1 mm. The dermis is sions of mosaicism for these gene mutations.
thickest on the back, where it is 30–40 times as thick as the The epidermis can be divided into the innermost basal layer
overlying epidermis. The amount of subcutaneous fat is gener- (stratum germinativum), the malpighian or prickle layer
ous on the abdomen and buttocks compared with the nose and (stratum spinosum), the granular layer (stratum granulosum),
sternum, where it is meager. and the horny layer (stratum corneum). On the palms and
soles, a pale clear to pink layer, the stratum lucidum, is noted
just above the granular layer. When the skin in other sites is
EPIDERMIS AND ADNEXA scratched or rubbed, the malpighian and granular layers
thicken, a stratum lucidum forms, and the stratum corneum
During the first weeks of life, the fetus is covered by a layer becomes thick and compact. Histones appear to regulate epi-
of nonkeratinizing cuboidal cells called the periderm (Fig. 1-2). dermal differentiation, and histone deacetylation suppresses
Later, the periderm is replaced by a multilayered epidermis. expression of profilaggrin. Slow-cycling stem cells provide a
Adnexal structures, particularly follicles and eccrine sweat reservoir for regeneration of the epidermis. Sites rich in stem
units, originate during the third month of fetal life as down- cells include the deepest portions of the rete, especially on
growths from the developing epidermis. Later, apocrine sweat palmoplantar skin, as well as the hair bulge. Stem cells divide
units develop from the upper portion of the follicular epithe- infrequently in normal skin, but in cell culture they form
lium and sebaceous glands from the midregion of the follicle. active, growing colonies. They can be identified by their high
Adnexal structures appear first in the cephalic portion of the expression of β1-integrins and lack of terminal differentiation
fetus and later in the caudal portions. markers. Stem cells can also be identified by their low levels
The adult epidermis is composed of three basic cell types: of desmosomal proteins, such as desmoglein 3. The basal cells
keratinocytes, melanocytes, and Langerhans cells. An addi- divide, and as their progeny move upward, they flatten and
tional cell, the Merkel cell, can be found in the basal layer of their nucleus disappears. Abnormal keratinization can mani-
the palms and soles, oral and genital mucosa, nail bed, and fest as parakeratosis (retained nuclei), as corps ronds (round,
follicular infundibula. Located directly above the basement clear to pink, abnormally keratinized cells), or as grains (elon-
membrane zone, Merkel cells contain intracytoplasmic dense- gated, basophilic, abnormally keratinized cells).
core neurosecretory-like granules and, through their associa- During keratinization, the keratinocyte first passes through
tion with neurites, act as slow-adapting touch receptors. They a synthetic and then a degradative phase on its way to becom-
have direct connections with adjacent keratinocytes by desmo- ing a horn cell. In the synthetic phase, within its cytoplasm the
somes and contain a paranuclear whorl of intermediate keratin keratinocyte accumulates intermediate filaments composed
filaments. Both polyclonal keratin immunostains and mono- of a fibrous protein, keratin, arranged in an α-helical coiled
clonal immunostaining for keratin 20 stain this whorl of pattern. These tonofilaments are fashioned into bundles, which
keratin filaments in a characteristic paranuclear dot pattern. converge on and terminate at the plasma membrane, where
Merkel cells also label for neuroendocrine markers such as they end in specialized attachment plates called desmosomes.
chromogranin and synaptophysin. The degradative phase of keratinization is characterized by the
disappearance of cell organelles and the consolidation of all
contents into a mixture of filaments and amorphous cell enve-
Keratinocytes lopes. This programmed process of maturation resulting in
death of the cell is called terminal differentiation. Terminal
Keratinocytes, or squamous cells, are the principal cells of the differentiation is also seen in the involuting stage of keratoac-
epidermis. They are of ectodermal origin and have the special- anthomas, where the initial phase of proliferation gives way
ized function of producing keratin, a complex filamentous to terminal keratinization and involution.
protein that not only forms the surface coat (stratum corneum) Premature programmed cell death, or apoptosis, appears in
of the epidermis but also is the structural protein of hair and hematoxylin and eosin (H&E)–stained sections as scattered
nails. Multiple distinct keratin genes have been identified and bright-red cells, some of which may contain small, black
1