An atlas of hair pathology with clinical correlations

226 Pages·2012·37.9 MB·English

by  Cowper

An Atlas of
Hair Pathology with
Clinical Correlations
Second Edition
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An Atlas of Hair Pathology with
Clinical Correlations

An Atlas of Hair Pathology with
Clinical Correlations
Second Edition
Leonard C. Sperling,
MD
Professor of Dermatology and Pathology
Chair of Dermatology at the Uniformed Services University
Bethesda, Maryland, USA
Shawn E. Cowper,
MD
Associate Professor of Dermatology and Pathology
Yale University
New Haven, Connecticut, USA
Eleanor A. Knopp,
MD
Fellow in Dermatopathology and Clinical Dermatologist
Yale University
New Haven, Connecticut, USA
This edition published in 2012 by Informa Healthcare, 119 Farringdon Road, London EC1R 3DA, UK.
Simultaneously published in the USA by Informa Healthcare, 52 Vanderbilt Avenue, 7th Floor, New York, NY 10017, USA.
First edition published in 2003 by The Parthenon Publishing Group.
Informa Healthcare is a trading division of Informa UK Ltd. Registered Office: Informa House, 30–32 Mortimer Street, W1W 7RE.
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Contents
Preface vi 30. Erosive pustular dermatosis 171
31. Brocq’s alopecia (pseudopelade of Brocq) and
1. Using this book 1 end-stage cicatricial alopecia 174
2. Specimen acquisition, handling and processing 3 32. Aplasia cutis congenita of the scalp 178
3. Normal hair anatomy and architecture 5 33. Tinea capitis 181
4. Evaluating and describing transverse 34. Trichodysplasia of immunosuppression 190
(horizontal) sections 17
35. Chemotherapy-induced alopecia 194
5. Classification of hair disease 26
36. Overview of hair shaft disorders 198
6. Distinctive or critical histological features
and associated diseases 28
Glossary 209
7. Clinical correlation 35
Index 213
8. Senescent alopecia (“senile alopecia” or
“senescent balding”) 40
9. Androgenetic alopecia 42
10. Telogen effluvium 53
11. Trichotillomania 60
12. Traction alopecia 67
13. Postoperative (pressure-induced) alopecia 72
14. Temporal triangular alopecia 75
15. Alopecia areata 79
16. Psoriatic alopecia 96
17. Tumor necrosis factor-alpha inhibitor associated
psoriasiform alopecia (drug-induced psoriasiform
alopecia) 102
18. Syphilitic alopecia 108
19. Noncicatricial alopecia from systemic
lupus erythematosus 111
20. Loose anagen hair syndrome 115
21. Central, centrifugal, cicatricial alopecia 120
22. Acne keloidis (folliculitis keloidalis) 126
23. Folliculitis decalvans 131
24. Tufted folliculitis 134
25. Lichen planopilaris 137
26. Frontal fibrosing alopecia 150
27. Fibrosing alopecia in a pattern distribution 154
28. Chronic cutaneous lupus erythematosus 158
29. Dissecting cellulitis of the scalp (perifolliculitis
capitis abscedens et suffodiens) 166
v
Preface
This second edition differs from the first in several ways. There of course Joanne and Laura are always there for me. They
are now three authors, instead of one, which has allowed for new make the effort worthwhile.
approaches and a more critical and synergistic presentation of
shawn
the material. Every section has been a group effort, with careful
editorial inputs from all three of us. In this way, poor-quality My interest in alopecia comes from “hands on” experience in
images and confusing or nebulous text have been excluded (we the alopecia clinic at UCSF, where I worked with Vera Price
hope), and innovative ideas have had a chance to take root and and her fellow, Paradi Mirmarani. This was bolstered by the
undergo refinement. The images, which are truly the heart and excellent dermatopathology training I received from my fel-
soul of the text, are a dramatic improvement from the first edi- lowship mentors Tim McCalmont and Phil Leboit.
tion, thanks to improved cameras and technology. Several addi- Besides these early influences in my academic career, I would
tional sections have been added to capture the latest thinking in like to thank Len, whose work I admired long before I finally
nomenclature as well as some of the less common diseases that got to work with him in 2006. He is a patient and unflappable
are entering the mainstream of clinical and pathological practice. teacher, and a pragmatic practitioner of evidence-based medi-
What has not changed is the purpose of the book, which is cine. I want to thank him not only for his instruction and
intended to serve as a primer, an atlas, and a reference text. As example, but also for inviting me to be a part of this project—
a primer, the book reviews very basic information, including a true team effort created over many months of authoring,
hair anatomy and the “nuts and bolts” of processing and evalu- imaging, editing, revising, and re-revising.
ating specimens. The authors assume that the reader knows Our coauthor, Eleanor, is among the kindest and smartest
very little about hair disease or hair pathology, and so a step- people I have ever met, and I know she will have a truly great
by-step approach is utilized. As an atlas, the book is rich in career. It has been a pleasure working with her as she made her
photographs demonstrating both basic and advanced histo- way through Yale over the years, culminating in the successful
logical features of hair disease. As a reference, the book includes completion of her fellowship in dermatopathology virtually
the most up-to-date information about the pathology of hair coincident with her co-authorship of this second edition.
disease, presented with a synopsis format. Basic clinical fea- My wife Carolyn, and our children Nathaniel, Benjamin,
tures are reviewed to provide clinicopathological correlation. and Emma all deserve medals for their patience while this edi-
We expect that dermatopathologists will be the most enthu- tion came together. I love you all.
siastic audience for this book. However, many general patholo- I would like to thank our dermatopathology fellow, Ashley
gists and dermatologists were avid users of the first edition, Mason, for taking on the proofreading of this text in addition
and we expect the same to be true of the second. to her many other duties. In addition, thanks to Carol Hribko,
whose expertise with image editing and careful proofreading
len
brought this edition to a new standard of excellence. Lastly, I
The science of hair pathology has come a long way since Dr. thank my partners at Yale for their support of this effort.
Headington’s seminal article on the subject (Headington J.
eleanor
Transverse microscopic anatomy of the human scalp. Arch
Dermatol 1984;120:449–56). We still owe Dr. Headington a There are many minds, eyes, and hands that helped create this
debt of gratitude; and many of his keen observations are uti- work. To all who contributed ideas, research, images, and time:
lized in this text. I have been fortunate to follow the footsteps thank you.
of many pioneers in the field of hair disease, such as David Len has a knack for making difficult things simple. His
Whiting, Vera Price, Wilma Bergfeld, Al Solomon, and Rodney knowledge is profound, his thinking is clear, and lucky for the
Dawber. David Whiting, in particular, has been a selfless men- rest of us, he is open and generous with his insights. He has
tor and “cheerleader” for much of my work. I have also received been an incomparable teacher, mentor, and friend, and I am
support and encouragement from many other leaders in the grateful to him for inviting me to contribute to this project.
field of hair disease, such as Jerry Shapiro, George Cotsarelis, Shawn is a remarkable physician, an innovator with exper-
Kurt Stenn, Elise Olsen, Amy McMichael, Rodney Sinclair, tise in diverse areas of dermatopathology, and a phenomenal
Lynne Goldberg, Antonella Tosti, Catherine Stefanato, Laila El teacher. Our collaborative “hair service” was a highlight of my
Shabrawi-Caelen, and Jeffrey Miller. dermatopathology education. I feel incredibly fortunate to
The confidence to move forward with an ambitious project have trained with Shawn, to count him as a friend, and to have
such as this text is due, in part, to the encouragement I have worked together on this project.
received from my academic idols and mentors: William D. James, I would like to offer my heartfelt gratitude to Jennifer
Stephen I. Katz, John Stanley, Kim Yancey, George Lupton, Jeffrey McNiff, Earl Glusac, and the whole team of amazing dermato-
Callen, Jennifer McNiff, and Larry Laughlin, among others. pathologists at Yale: Rossitza Lazova, Tony Subtil, Christine Ko,
My co-authors, Shawn Cowper and Eleanor Knopp, are sim- Anjela Galan, and Marcus Bosenberg—each has had a pro-
ply brilliant and have brought so much to this endeavor. And found impact on me. I am enormously thankful for the
vi
vii
PREFACE
instruction, guidance, and mentorship from Richard Edelson, I send my love and deepest appreciation to my family,
Robert Tigelaar, and my Yale Dermatology family. including my father, Robert Knopp, MD, who is missed every
I am grateful to the North American Hair Research Society moment.
and the Women’s Dermatologic Society for facilitating my ear- My copy of the first edition of the atlas is well worn and
liest experiences studying under and learning from Len coming apart at the seams—may this edition fare as well in
through their mentorship programs. They worked! your hands!

1
Using this book
the “trichopathologist’s” field guide Section I: Acquisition
Within the medical subspecialty of dermatopathology, there Before the study of biopsy specimens, one must understand
is yet another smaller world inhabited by those who special- how to sample and process the biopsy in order to maximize
ize in (and struggle with) the challenge of diagnosing alope- the diagnostic yield. This is the focus of chapter 2.
cia. These ultraspecialists lack an offi cial label. One might
consider calling them “alopecists” but according to the Section II: Context
Oxford English Dictionary (OED), that label has already Before beginning an evaluation of what is abnormal about a
been taken to describe those who “undertake to cure or pre- specimen, one must fi rst understand normal cycling and hair
vent baldness.” “Trichologist” might be a better term, and the follicular anatomy. Chapters 3 and 4 cover this in detail,
OED informs us that a trichologist is one “who is versed in including a discussion and rationale for our advocacy of hori-
the study of the structure, functions, and diseases of the hair.” zontal (transverse) sectioning of scalp biopsies.
Indeed this is a broader, more inclusive term, but appears to
be already in use. The term also tends to describe a nonphysi- Section III: Walking
cian paramedical expert who completes a two-year certifi ca- We must “walk before we can run.” Chapter 5 sketches out a
tion course and is subsequently recognized as a trichologist broad concept of classes of alopecia that will allow the student
after passing a certifying exam. of trichopathology to establish a foundation of important
Practitioners (both novice and expert) of the histopathologic concepts. Chapter 6 discusses a specifi c methodology that
evaluation of alopecia need their own custom label. For the walks the student through the fi rst steps of identifying salient
expediency of this introductory chapter, we will use the term (and sometimes specifi c) histologic features, and narrowing
“trichopathologist.” Trichopathologists are engaged in the fi rst the broad fi eld of alopecia into several clear and smaller groups
steps necessary for the successful work of the alopecist, namely, that one can study in more specifi c detail.
ensuring an accurate diagnosis. Successful trichopathologists
need to understand the three-dimensional anatomy of the hair Section IV: Correlation
follicular unit and associated supporting structures. They The student of trichopathology will quickly learn that the
should understand something of the clinical presentation of number of histologic features one may encounter in a case of
various alopecias, as well as have a thorough understanding of alopecia is not broad. Making sense of these features often
the nature of the hair follicle cycle, the impact of alopecic states requires clinical input. If the clinician provides no better infor-
on the shape of the hair shaft, and on the results of some easily mation than “alopecia” on the requisition form, the patholo-
performed clinical tests. Also, trichopathologists should under- gist and the clinician will probably fi nd the diagnostic endeavor
stand the use of ancillary testing and something about the less than satisfactory. Good clinical input may provide enough
appropriate treatment of various alopecias to provide guidance information to render a specifi c diagnosis when histologic fea-
to their alopecist colleagues. tures are borderline, or favor a diagnosis when histologic fea-
Our intent is that this book will serve not only as a fi eld tures are nonspecifi c. Chapter 7 includes a discussion about
guide to the “budding” trichopathologists, but also as a frame- specifi c clinical features that can provide the correlation
work for the seasoned trichopathologists who may be encoun- needed for a fruitful diagnostic collaboration.
tering an unusual feature or alopecia variant for the fi rst time.
Whether our readers ultimately come to refer to themselves as Section V: Running
“trichopathologists” is yet to be determined. We do hope, how- After walking through the basics of Section III and embracing
ever, that the following chapters will inspire a new generation any clinical input provided, the trichopathologist should be
of dermatopathologists to take the next step and think of able to “run” to one of several diagnoses. Section V constitutes
themselves as trichopathologists. chapters 8–35. The chapters in this section are focused on a
specifi c diagnosis. Each diagnosis has a paragraph of introduc-
using the guide
tion, sometimes containing information about the known or
This book is organized into several sections (each comprising presumed cause of the disease. This is followed by paragraphs
one or more chapters) meant to systematically lead the diag- detailing the clinical fi ndings (including a brief summary of
nostician to a specifi c diagnosis. Sections I–IV provide the treatment options) and the histological fi ndings. Special diag-
fundamental skills needed to tackle the evaluation of any sort nostic techniques (e.g., direct immunofl uorescence, special
of alopecia. Section V provides a detailed discussion of many stains) are also discussed in this section. A boxed summary
specifi c diagnoses, as well as a quick reference box at the end of (toward the end of each diagnosis chapter) contains critical
the chapter with the most salient features and critical facts. and salient clinical features, followed by histologic fi ndings.
Section VI is an atlas of hair shaft disorders. The last section, The fi rst set of histologic fi ndings (denoted by ) is consid-
Section VII, is a comprehensive glossary of terminology and ered the minimal criteria needed to establish a particular diag-
an in-depth index of the entire work. nosis. These are followed by round bullets () that denote
1