Dermatopathology: A volume in the Foundations in Diagnostic Pathology Series, Expert Consult - Online and Print

690 Pages·2009·76.71 MB·English

by  Klaus J. Busam MD

Copyright © 2010 by Saunders, an imprint of Elsevier Inc.
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Library of Congress Cataloging-in-Publication Data
Dermatopathology / edited by Klaus J. Busam. – 1st ed.
p. ; cm. – ( Foundations in diagnostic pathology)
Includes bibliographical references.
ISBN 978-0-443-06654-2
1. Skin–Diseases. 2. Skin–Cancer. I. Busam, Klaus J. II. Title. III. Series: Foundations in diagnostic
pathology.
[DNLM: 1. Skin Diseases–pathology. 2. Skin Neoplasms–pathology. WR 140 D4351705 2010]
RL96.D478 2010
616.5¢07–dc22
2009010247
Publishing Director: William R. Schmitt
Developmental Editor: Christine Abshire
Publishing Services Manager: Anitha Raj
Project Manager: Sruthi Viswam
Design Direction: Steven Stave
Printed in the United States of America
Last digit is the print number 9 8 7 6 5 4 3 2 1
This book is dedicated to my wife and children.
To Vinita—for your tireless love and support.
You put up with a lot!
To Jonathan and Sophia—for the joy of your company.
Foreword
The study and practice of anatomic pathology are both impressive, including many authors who have contrib-
exciting and overwhelming. Surgical pathology, with all uted significantly to the dermatopathology literature.
of the subspecialties it encompasses, and cytopathology More important, however, the authors are all practic-
have become increasingly complex and sophisticated, ing dermatopathologists with an enormous collective
and it is not possible for any individual to master the experience in the field.
skills and knowledge required to perform all of these As with all of the other editions in the Foundations
tasks at the highest level. Simply being able to make a in Diagnostic Pathology series, the information in the
correct diagnosis is challenging enough, but the standard current text is presented in a uniform and organized
of care has far surpassed merely providing a diagnosis. fashion, covering the essential clinical and pathologic
Pathologists are now asked to provide large amounts features of each entity, accompanied by a large number
of ancillary information, both diagnostic and prognos- of practical tables and high-quality photomicrographs.
tic, often on small amounts of tissue, a task that can be Dermatopathology in particular lends itself to an algo-
daunting even to the most experienced pathologist. rithmic approach to diagnosis, on which this book
Although large general surgical pathology textbooks consistently capitalizes. When applicable, the authors
are useful resources, they by necessity could not possibly integrate modern ancillary diagnostic techniques, includ-
cover many of the aspects that pathologists need to know ing immunohistochemistry, immunofluorescence, elec-
and include in their reports. Accordingly, the concept tron microscopy, and molecular diagnostics.
behind Foundations in Diagnostic Pathology was born. The book is organized into four major sections,
This series is designed to cover the major areas of surgi- including histology of the skin, non-neoplastic derma-
cal pathology and cytopathology, and each edition in the tologic disorders, and, of course, tumors and pseudotu-
series is focused on one major topic. The goal of every mors of the skin and subcutis. The final unique section
book in this series is to provide the essential information summarizes the salient features of the pathologic evalu-
that any pathologist, whether general or subspecialized, ation of sections derived from Mohs surgery.
in training or in practice, will find useful in the evalua- I wish to extend my heartfelt appreciation to Dr. Busam,
tion of virtually any type of specimen encountered. who took on this project despite innumerable other
Dr. Klaus Busam, a world-renowned dermatopathol- professional and personal responsibilities. As usual he
ogist at Memorial Sloan-Kettering Cancer Center, has provided enormous energy and wisdom in completing
edited a superb state-of-the-art book covering the essen- this endeavor. I would also like to extend my sincere
tial aspects of dermatopathology. This book is geared appreciation to the many authors who have taken the
not only to dermatopathologists or those who have a time from their busy lives to provide their expertise.
particular interest in this topic, but also to general sur- It is my hope you find this volume of Foundations in
gical pathologists who encounter skin specimens as Diagnostic Pathology to be a valuable help in your daily
part of their daily practice. The list of contributors is practice of dermatopathology.
John R. Goldblum, md
vii
Preface
When I was first approached with the idea of editing Minimizing references to save space for text and illustra-
this book, I hesitated, wondering whether it was worth tions seemed justifiable in an era when ready access to
publishing another textbook on dermatopathology, a the Internet permits quick retrieval of specialized arti-
field with a number of excellent books already avail- cles and new publications are added to the literature at
able, including comprehensive reference texts as well as a rapid pace. Controversial issues could not be discussed
basic introductions. On the other hand, pathology and in detail, but we tried to offer a balanced view and prac-
dermatology residents have been telling me that they tical guide to deal with ambiguity and complexity.
miss a textbook that covers more than the very basics, As editor I would like to thank all the contributors
but avoids an exhaustive attempt to review all that is who made this book possible. It is the result of team-
known in the field, and is compact enough so that they work. I would also like to thank many individuals at
can read it from start to finish during their residency Memorial Sloan-Kettering Cancer Center who provided
training. Such an introductory to midlevel book is what direct or indirect help for this book, including Jennifer
the Foundation in Diagnostic Pathology series attempts Nobrega, Jason Bini, Allyne Manzo, Kin Kong, and
to provide. We wanted to offer a no-nonsense practical Drs. Melissa Pulitzer, Ruby Delgado, and Bijal Amin.
book with emphasis on the clinical and histologic fea- I am also grateful to Dr. Marc Rosenblum, Chair of Pat h o -
tures required to make a diagnosis. In an effort to keep logy, and Dr. Allan Halpern, Chief of Dermatology, for a
the book compact, compromises were necessary. A num- supportive professional environment. Special thanks
ber of clinical issues or disease entities had to be left out go to members of the Dermatology Service for providing
or could be mentioned only briefly. This affected in par- clinical images and/or valuable comments, in particular
ticular rare congenital diseases and entities for which Drs. Kishwer Nehal, Patricia Myskowski, and Ashfaq
a skin biopsy provides only limited diagnostic value. Marghoob. Finally I want to express my great apprecia-
References were cut to a minimum, offering mainly tion for the support and patience I received from the
review articles or specialized books for the reader will- publisher, especially the help from William Schmitt,
ing to engage in a more in-depth exploration of a subject. Christine Abshire, and Sruthi Viswam.
ix
Contributors
Bijal Amin, MD Maxwell A. Fung, MD
Fellow in Dermatopathology Associate Professor of Clinical Dermatology and Pathology
Department of Pathology Department of Dermatology
Memorial Sloan-Kettering Cancer Center University of California Davis
New York, New York Sacramento, California
Wilma F. Bergfeld, MD, FAAD John R. Goldblum, MD
Co-Director, Dermatopathology Professor of Pathology
Departments of Dermatology and Pathology Department of Anatomic Pathology
Senior Staff, Department of Dermatology Cleveland Clinic Lerner College of Medicine
Cleveland Clinic Chairman, Department of Anatomic Pathology
Cleveland, Ohio Cleveland Clinic
Cleveland, Ohio
Steven D. Billings, MD
Associate Professor Jacqueline Granese, MD, FCAP
Department of Anatomic Pathology Dermatopathologist
Cleveland Clinic Lerner College of Medicine Director of Anatomic and Clinical Pathology
Co-director, Dermatopathology Section Departments of Pathology and Dermatology
Anatomic Pathology and Dermatology Graves Gilbert Clinic
Cleveland Clinic Bowling Green, Kentucky
Cleveland, Ohio
Henry Haskell, MD
J. Andrew Carlson, MD, FRCPC
Clinical Assistant Professor
Professor Department of Dermatology
Department of Pathology University of Oklahoma
Albany Medical College Oklahoma City, Oklahoma
Albany, New York Pathologist
Regional Medical Laboratory
Julide Tok Celebi, MD
Pathologist
Associate Professor of Clinical Dermatology
Department of Pathology
Department of Dermatology
St. John’s Medical Center
Columbia University
Tulsa, Oklahoma
New York, New York
Helen M. Chen, MD, PhD Jacqueline M. Junkins-Hopkins, MD
Clinical Assistant Professor Associate Professor in Dermatology
Department of Dermatology Department of Dermatology
University of Illinois and Chicago Johns Hopkins University
Chicago, Illinois Director, Division of Dermatopathology
Department of Dermatology
Loren E. Clarke, MD
Johns Hopkins Hospital
Assistant Professor Baltimore, Maryland
Departments of Pathology and Dermatology
Penn State College of Medicine Neil Scott McNutt, MD
Pathologist/Dermatologist Senior Research Associate
Departments of Pathology and Dermatology Laboratory of Investigative Dermatology
The Penn State Milton S. Hershey Medical Center The Rockefeller University
Hershey, Pennsylvania Consultant in Pathology
Department of Pathology
Anca G. Prundeanu Croitoru, MD
Memorial Sloan-Kettering Cancer Center
Dermatopathologist New York, New York
CBLPATH
Rye Brook, New York Michael E. Ming, MD, MSCE
Assistant Professor
Douglas R. Fullen, MD
Department of Dermatology
Associate Professor
University of Pennsylvania
Department of Pathology and Dermatology
Philadelphia, Pennsylvania
University of Michigan
Ann Arbor, Michigan
xi
xii L ist of Contributors
Rajmohan Murali, MBBS, FRCPA Richard A Scolyer, BMedSci, MBBS, MD, FRCPA,
Clinical Senior Lecturer FRCPath, MIAC
Discipline of Pathology Clinical Professor
The University of Sydney Discipline of Pathology
Staff Specialist The University of Sydney
Department of Anatomical Pathology Senior Staff Specialist
Royal Prince Alfred Hospital Department of Anatomical Pathology
Sydney, NSW, Australia Royal Prince Alfred Hospital
Consultant Pathologist Sydney, NSW, Australia
Melanoma Institute Australia (incorporating the Sydney Consultant Pathologist
Melanoma Unit) Melanoma Institute Australia (incorporating the Sydney
North Sydney, NSW, Australia Melanoma Unit)
North Sydney, NSW, Australia
Patricia L. Myskowski, MD
Professor Klaus Sellheyer, MD
Department of Dermatology Cleveland Clinic Foundation
Weill Cornell Medical College Department of Dermatology
Attending Physician, Dermatology Service Cleveland, Ohio
Medicine Nelson Dermatopathology Associates
Memorial Sloan-Kettering Cancer Center Atlanta, Georgia
Attending Physician
Christopher R. Shea, MD
Dermatology
Professor, Department of Medicine; Chief, Section of
New York-Presbyterian Hospital
Dermatology; Director, Residency and Dermatopathology
New York, New York
Fellowship Programs,
Kishwer S. Nehal, MD Department of Medicine, Section of Dermatology
Associate Professor of Dermatology The University of Chicago, The University of Chicago
Department of Dermatology Medical Center
Weill Cornell Medical College Chicago, Illinois
Associate Attending Physician
John F. Thompson, MD, FRACS, FACS
Dermatology/Medicine
Professor of Melanoma and Surgical Oncology
Memorial Sloan-Kettering Cancer Center
Faculty of Medicine
New York, New York
The University of Sydney
Victor G. Prieto, MD, PhD Consultant Surgeon
Royal Prince Alfred and Mater Hospitals
Professor
Sydney, NSW, Australia
Departments of Pathology and Dermatology
Executive Director
University of Texas M.D. Anderson Cancer Center and
Melanoma Institute Australia (incorporating the Sydney
Laboratory Medicine
Melanoma Unit)
Houston, Texas
North Sydney, NSW, Australia
Melissa Pulitzer, MD
Marie Tudisco, Phd, HT(ASCP)
Assistant Attending Pathologist
Histotechnologist
Department of Pathology,
Department of Dermatology
Memorial Sloan-Kettering Cancer Center
Memorial Sloan-Kettering Cancer Center
New York, New York
New York, New York
Marcia Ramos-e-Silva, MD, PhD Ravi Ubriani, MD
Associate Professor and Head Assistant Professor of Clinical Dermatology
Sector of Dermatology Department of Dermatology
Federal University of Rio de Janeiro Columbia University
Rio de Janeiro, Brazil New York, New York
Franco Rongioletti, MD Angela J. Wyatt, MD
Professor of Dermatology Clinical Assistant Professor
Section of Dermatology and Dermatopathology Department of Dermatology
University of Genoa Baylor College of Medicine
Genoa, Italy Physician Dermatology
Memorial Hermann Southwest Hospital and St. luke’s
Episcopal Hospital
Houstan, Texas
Table of Contents

Part I: Non-neoplastic Skin Disorders
Section 1:Inflammatory Reactions
Chapter 1. Spongiotic Dermatitis
Chapter 2. Psoriasiform Dermatitis
Chapter 3. Interface Dermatitis
Chapter 4. Perivascular Dermatitis
Chapter 5. Nodular and Diffuse Infiltrates
Chapter 6. Vesicular Dermatitis
Chapter 7. Vasculitis
Chapter 8. Panniculitis
Section 2: Infections
Chapter 9. Bacterial Infections
Chapter 10. Fungal Infections
Chapter 11. Viral Infections
Chapter 12. Other Infections
Section 3:Miscellaneous Non-Neoplastic Conditions
Chapter 13. Genodermatoses
Chapter 14. Disorders of Pigmentation
Chapter 15. Deposition Disorders
Chapter 16. Alopecia
Part II: Cutaneous Neoplasms
Chapter 17. Epidermal Tumors
Chapter 18. Sebaceous Tumors
Chapter 19. Follicular Tumors
Chapter 20. Sweat Gland Tumors
Chapter 21. Melanocytic Tumors
Chapter 22. Soft Tissue Tumors
Chapter 23. Hematolymphoid Neoplasms
Chapter 24. Metastases
<PII>, <AID>
i
seCtion
Histology of tHe skin
Henry Haskell
Skin consists of epidermis and dermis (Fig. I-1). Beneath esses provide contact points for desmosomes, which are
the dermis lies the subcutis (or hypodermis). the ultrastructural basis for the tight binding of kerati-
nocytes to one another. These processes may be difficult
to visualize in normal skin, but they become more evi-
dent by intercellular edema (also known as spongiosis).
EpidErmis The stratum granulosum, or granular cell layer, is
named for the irregular, darkly basophilic keratohy-
alin granules that accumulate in the cytoplasm of
The epidermis is a stratified squamous epithelium that the cells in this layer as they flatten and mature. At
consists mainly of keratinocytes, with an admixture of the superficial edge of the stratum granulosum, pro-
melanocytes, Langerhans, and Merkel cells. Of these, grammed cell death occurs.
keratinocytes are by far the most numerous, making up On most parts of the body, the skin possesses a single
the bulk of the epidermis and giving it its characteris- layer of dead (but functional) cells, the stratum corneum,
tic microscopic appearance. The epidermis is typically consisting mostly of keratin. The cells in this layer are
divided into four layers (see Fig. I-1): sometimes called corneocytes, to distinguish them from
the living cells in the layers below. The stratum corneum
1. Stratum basale (SB)
varies widely in thickness according to site: in glabrous
2. Stratum spinosum (SS)
skin, it is only a few cell layers thick, and forms a char-
3. Stratum granulosum (SG)
acteristic “basket weave” pattern; whereas at acral sites,
4. Stratum corneum (SC)
it is both thicker and more compact (Fig. I-2). Where
The stratum basale (or stratum germinativum), also the stratum corneum is particularly thick, another layer,
known as the basal cell layer, consists of a single layer the so-called stratum lucidum, may be present between
of cuboidal keratinocytes that lie atop the basement it and the stratum granulosum. The stratum lucidum
membrane (see the following) and are connected to it differs from the stratum corneum only by a pale eosino-
by numerous hemidesmosomes. At this depth, keratino- philic appearance and higher lipid content. Although
cytes have abundant eosinophilic cytoplasm and ovoid pyknotic nuclei may appear in the stratum corneum or
nuclei. As its name implies, division of keratinocytes stratum lucidum in pathologic processes, in normal skin
occurs primarily in the stratum germinativum, although the keratinocytes of both these layers are anucleate.
mitotic activity is occasionally seen in the lower part of Keratinocytes stain positively for high molecular
the stratum spinosum, in particular the cell layer imme- weight cytokeratins such as 34betaE12, but negatively
diately above the stratum basale, the parabasal layer. In for Cam 5.2.
the process of self-renewal, these cells gradually ascend
into the upper layers and are replaced.
Melanocytes
The stratum spinosum, also known as the spinous cell
layer, is named after the spinous processes (or “prick-
les”), which connect the keratinocytes in this layer and Melanocytes are found along the dermoepidermal
the stratum basale to one another. These spinous proc- junction as well as within hair follicles (Fig. I-3).
1
2 Dermatopathology
They are responsible for the production and secretion
SC of melanin pigment. Histologically the cells are char-
SG acterized by small, dark, ovoid nuclei and scant, clear
Epidermis SS
cytoplasm. Depending on anatomic site they number
Papillary SB from one per ten to one per five basal keratinocytes,
Dermis with higher concentrations on the face and genitalia.
Although melanocytes produce melanin, pigment is
not normally visible in their cytoplasm, as it is rapidly
Reticular secreted through their network of dendritic processes
Dermis
and taken up by basal keratinocytes, where it is stored
and gradually broken down. Although the amount of
melanin produced and stored varies between darker-
Figure i-1 and lighter-skinned individuals, the number of mel-
Normal skin with epidermis, papillary and reticular dermis. the epidermis anocytes does not.
consists of stratum basale (SB), stratum spinosum (SS), stratum granulo-
Normal intraepidermal melanocytes stain immuno-
sum (Sg), and stratum corneum (SC).
histochemically best for tyrosinase, Melan-A/Mart-1,
and microphthalmia transcription factor. HMB-45 may
decorate some normal melanocytes, but it is not a sensi-
tive reagent for visualizing normal resting melanocytes.
S-100 protein stains normal intraepidermal melano-
cytes, but it is neither very sensitive nor specific. It also
stains Langerhans cells.
langerhans cells
The Langerhans cell is a dendritic cell that functions
in antigen presentation, and travels between the skin
and draining lymph nodes. In routine tissue sections
of uninflamed skin, these cells are difficult to iden-
tify on hematoxylin and eosin (H&E)–stained sec-
tions. They are best seen on immunostains for S-100
protein and/or CD1a, which highlight their charac-
Figure i-2 teristic location above the stratum basale (Fig. I-4).
Normal acral skin with thick compact stratum corneum and acrosyringeal
duct (arrow).
Figure i-4
Figure i-3 langerhans cells are immunoreactive for CD1a and recognized as dendritic
melanocytes (arrow) are present at the dermoepidermal junction. cells within the spinous cell layer.