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McGraw-Hill Education Specialty Board Review Dermatology A Pictorial Review Ali-FM_00i-xiv.indd 1 9/18/14 3:15 PM Notice Medicine is an ever-changing science. As new research and clinical experience broaden our knowledge, changes in treatment and drug therapy are required. The authors and the publisher of this work have checked with sources believed to be reliable in their efforts to provide information that is complete and generally in accord with the standards accepted at the time of publication. However, in view of the possibility of human error or changes in medical sciences, neither the authors nor the publisher nor any other party who has been involved in the preparation or publication of this work warrants that the information contained herein is in every respect accurate or complete, and they disclaim all responsibility for any errors or omissions or for the results obtained from use of the information contained in this work. Readers are encouraged to confirm the information contained herein with other sources. For example and in particular, readers are advised to check the product information sheet included in the package of each drug they plan to administer to be certain that the information contained in this work is accurate and that changes have not been made in the recommended dose or in the contraindications for administration. This recommendation is of particular importance in connection with new or infrequently used drugs. Ali-FM_00i-xiv.indd 2 9/18/14 3:15 PM McGraw-Hill Education Specialty Board Review Dermatology A Pictorial Review Third Edition Editor Asra Ali, MD Private Practice—Dermatology Houston, Texas New York Chicago San Francisco Athens London Madrid Mexico City Milan New Delhi Singapore Sydney Toronto Ali-FM_00i-xiv.indd 3 9/18/14 3:15 PM Copyright © 2015 by McGraw-Hill Education. All rights reserved. Except as permitted under the United States Copyright Act of 1976, no part of this publication may be reproduced or distributed in any form or by any means, or stored in a database or retrieval system, without the prior written permission of the publisher, with the exception that the program listings may be entered, stored, and executed in a computer system, but they may not be reproduced for publication. ISBN: 978-0-07-179324-7 MHID: 0-07-179324-0 The material in this eBook also appears in the print version of this title: ISBN: 978-0-07-179323-0, MHID: 0-07-179323-2. eBook conversion by codeMantra Version 1.0 All trademarks are trademarks of their respective owners. Rather than put a trademark symbol after every occurrence of a trademarked name, we use names in an editorial fashion only, and to the benefit of the trademark owner, with no intention of infringement of the trademark. Where such designations appear in this book, they have been printed with initial caps. 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THE WORK IS PROVIDED “AS IS.” McGRAW-HILL EDUCATION AND ITS LICENSORS MAKE NO GUARANTEES OR WARRANTIES AS TO THE ACCURACY, ADEQUACY OR COMPLETENESS OF OR RESULTS TO BE OBTAINED FROM USING THE WORK, INCLUDING ANY INFORMATION THAT CAN BE ACCESSED THROUGH THE WORK VIA HYPERLINK OR OTHERWISE, AND EXPRESSLY DISCLAIM ANY WARRANTY, EXPRESS OR IMPLIED, INCLUDING BUT NOT LIMITED TO IMPLIED WARRANTIES OF MERCHANTABILITY OR FITNESS FOR A PARTICULAR PURPOSE. McGraw-Hill Education and its licensors do not warrant or guarantee that the functions contained in the work will meet your requirements or that its operation will be uninterrupted or error free. Neither McGraw-Hill Education nor its licensors shall be liable to you or anyone else for any inaccuracy, error or omission, regardless of cause, in the work or for any damages resulting therefrom. McGraw-Hill Education has no responsibility for the content of any information accessed through the work. Under no circumstances shall McGraw-Hill Education and/or its licensors be liable for any indirect, incidental, special, punitive, consequential or similar damages that result from the use of or inability to use the work, even if any of them has been advised of the possibility of such damages. This limitation of liability shall apply to any claim or cause whatsoever whether such claim or cause arises in contract, tort or otherwise. Contents C o n tr i b u t o r s / v i i pre f ace / x i II C h apter 1 C h apter 9 Hair Findings / 1 PIGMENTARY DISORDERS / 159 Paradi Mirmirani and Jennifer Ahdout Kaveh A. Nezafati, Roger Romero, and Amit G. Pandya C h apter 2 EYE FINDINGS / 19 Mirwat S. Sami and Charles S. Soparkar C h apter 3 NAIL FINDINGS / 39 Ravi Ubriani C h apter 1 0 DISORDERS OF FAT / 175 Hung Doan, Marigdalia K. Ramirez-Fort, Ryan J. Gertz, Andrew J. Thompson, Farhan Khan, and Stephen K. Tyring C h apter 1 1 CUTANEOUS TUMORS / 191 Joy H. Kunishige and Alexander J. Lazar C h apter 4 C h apter 1 2 ORAL PATHOLOGY / 57 MELANOMA AND NON-MELANOMA SKIN CANCER / 213 Kamal Busaidy, Jerry Bouquot, and Laith Mahmood Sumaira Aasi and Michelle Longmire C h apter 5 Genital Dermatology / 85 Libby Edwards C h apter 1 3 Vascular Tumors And Malformations / 225 Denise W. Metry, John C. Browning, and Asra Ali C h apter 6 CONTACT DERMATITIS / 97 C h apter 1 4 Melissa A. Bogle, Giuseppe Militello, and Sharon E Jacob GENODERMATOSIS / 239 C h apter 7 AUTOIMMUNE BULLOUS DISEASES / 113 Whitney A. High Nnenna G. Agim, Joy H. Kunishige, Marzieh Thurber, Adrienne M. Feasel, and Adelaide A. Hebert C h apter 1 5 PEDIATRIC DERMATOLOGY / 269 C h apter 8 Disorders of Cornification, Infiltration, and Inflammation / 129 Pamela Gangar and Rakhshandra Talpur Nnenna G. Agim, John C. Browning, Denise W. Metry, and Adrienne M. Feasel C h apter 1 6 CUTANEOUS INFESTATIONS / 283 Dirk M. Elston, Asra Ali, Melissa A. Bogle, and Alyn D. Hatter Ali-FM_00i-xiv.indd 5 9/18/14 3:15 PM Contributors Sumaira Z. Aasi, MD Jerry E. Bouquot, DDS, MSD, FICD, FACD, FRCM (UK) Professor Department of Dermatology Stanford University Palo Alto, California Chapter 12 Adjunct Professor Department of Diagnostic & Biomedical Sciences University of Texas School of Dentistry Houston, Texas Adjunct Professor Department of Rural Health & Community Dentistry West Virginia University School of Dentistry Morgantown, West Virginia Director of Research The Maxillofacial Center for Education & Research, Morgantown, West Virginia Chapter 4 Nnenna G. Agim, MD Assistant Professor of Dermatology Children’s Medical Center Dallas and University of Texas Southwestern Medical Center Pediatric Dermatology Houston, Texas Chapters 14, 15 Jennifer Ahdout, MD Department of Dermatology UC Irvine Irvine, California Director of Dermatology, Lasers, and Skin Care Spalding Drive Plastic Surgery & Dermatology Beverly Hills, California Chapter 1 Carolyn A. Bangert, MD Associate Professor Department of Dermatology University of Texas Houston, Texas Chapter 22 Melissa A. Bogle Director The Laser & Cosmetic Surgery Center of Houston Houston, Texas Clinical Assistant Professor Department of Dermatology University of Texas M.D. Anderson Cancer Center Houston, Texas Chapters 16, 26 Ali-FM_00i-xiv.indd 7 Kamal Busaidy, BDS, FDSRCS Associate Professor Department of Oral and Maxillofacial Surgery University of Texas-School of Dentistry Houston, Texas Chapter 4 John C. Browning, MD, FAAD, FAAP Assistant Professor of Pediatrics and Dermatology Baylor College of Medicine Houston, Texas Assistant Professor of Pediatrics and Dermatology University of Texas Health Science Center San Antonio, California Chief of Dermatology Children’s Hospital of San Antonio San Antonio, California Chapters 13, 15 Christopher T. Burnett, MD Dermatology Associates of Wisconsin Milwaukee, Wisconsin Chapters 24, 28 Stephanie F. Chan Graduate student in Biostatistics Harvard University Cambridge, Massachusetts Chapter 29 9/18/14 3:15 PM x CONTRIBUTORS Natalia Mendoza, MD Amit G. Pandya, MD Department of Dermatology University of Texas Medical School at Houston Houston, Texas Chapter 17 Department of Dermatology The University of Texas Southwestern Medical Center Dallas, Texas Chapter 9 Denise W. Metry, MD Clinical Assistant Professor FIU Wertheim College of Medicine Miami, Florida Chapter 33 Associate Professor of Dermatology and Pediatrics Texas Children’s Hospital/Baylor College of Medicine Houston, Texas Chapters 13, 15 Jason H. Miller, MD Resident Physician Department of Dermatology University of Texas at Houston Health Science Center M. D. Anderson Cancer Center Houston, Texas Chapters 22, 23 Paradi Mirmirani, MD Permanente Medical Group Vallejo, California University of California San Francisco, California Case Western Reserve University Cleveland, Ohio Chapter 1 Kiran Motaparthi, MD Assistant Professor Department of Dermatology Baylor College of Medicine Houston, Texas Chapter 35 Kaveh A. Nezafati, MD Department of Dermatology The University of Texas Southwestern Medical Center Dallas, Texas Chapter 9 Tri H. Nguyen, MD, FACMS, FAAD, FACPH Texas Surgical Dermatology Houston, Texas Chapter 25 Roberto A. Novoa, MD Resident Department of Dermatology Case Western Reserve University Case Medical Center Cleveland, Ohio Chapter 27 Ali-FM_00i-xiv.indd 10 Gustavo Pantol, MD Giovanni Pellacani, MD Professor Department of Dermatology Medical University of Modena and Reggio Emilia Modena, Italy Chapter 32 Victor G. Prieto, MD, PhD Professor Departments of Pathology and Dermatology The University of Texas M.D. Anderson Cancer Center Houston, Texas Chapter 30 Marigdalia K. Ramirez-Fort, MD Department of Dermatology Tufts Medical Center Boston, Massachusetts Chapters 10, 20, 32, 33 Ronald P. Rapini, MD Chernosky Professor and Chair Department of Dermatology University of Texas Medical School at Houston and M.D. Anderson Cancer Center Houston, Texas Chapter 21 Riva Z. Robinson, MD Resident Physician Department of Preventive Medicine Texas A&M Health Science Center College of Medicine Round Rock, Texas Chapters 20, 33 Roger Romero, MD Department of Dermatology The University of Texas Southwestern Medical Center Dallas, Texas Chapter 9 9/18/14 3:15 PM xi CONTRIBUTORS Mirwat S. Sami, MD Marzieh Thurber, MD Ophthalmic Plastic and Reconstructive Surgeon, Private Practice Houston Oculofacial Plastic Surgery Houston, Texas Department of Head and Neck Surgery, Division of Surgery University of Texas M.D. Anderson Cancer Center Houston, Texas Department of Surgery, Division of Ophthalmology Texas Children’s Hospital Houston, Texas Department of Ophthalmology Houston Methodist Hospital Houston, Texas Chapter 2 Jupiter Medical Center Jupiter, Florida Chapter 14 Charles S. Soparkar, MD, PhD Ophthalmic Plastic and Reconstructive Surgery Private Practice Plastic Eye Surgery Associates Houston, Texas Department of Ophthalmology Baylor College of Medicine Houston, Texas Department of Ophthalmology Weill Cornell Medical College The Methodist Hospital Houston, Texas Chapter 2 Rakhshandra Talpur, MD Senior Research Scientist, Dermatology Research Department of Dermatology University of Texas M.D. Anderson Cancer Center Houston, Texas Chapter 8 Ali-FM_00i-xiv.indd 11 Stephen K. Tyring, MD, PhD Clinical Professor of Dermatology University of Texas Health Science Center Houston, Texas Chapters 10, 17, 20 Ravi Ubriani, MD Assistant Professor of Clinical Dermatology Columbia University New York, New York Chapter 3 Kara E. Walton, MD Assistant Professor Department of Dermatology Medical College of Wisconsin Milwaukee, Wisconsin Chapter 24 Rungsima Wanitphakdeedecha, MD Associate Professor Department of Dermatology Faculty of Medicine Siriraj Hospital Mahidol University Bangkok, Thailand Chapter 25 9/18/14 3:15 PM This page intentionally left blank preface McGraw-Hill  Education Specialty Board Review Dermatology: A Pictorial Review is now in its third edition.   The ever-changing field of dermatology demands constant updating of information. To address this need, the third edition presents many new images as well as a new chapter on confocal microscopy. The goal of this edition is similar to that of the previous two editions of the book. Not only is the book an excellent tool for dermatologyrelated questions on board exams to prepare residents in dermatology, primary care, and other clinical specialties, it will also help practicing dermatologists and other clinicians with their recertification exams.   As an invaluable resource in the clinical setting, the revised and updated new edition of this practical guide provides comprehensive, Ali-FM_00i-xiv.indd 13 yet concise, coverage of the diagnosis and management of common dermatologic disorders as well as some less common but important dermatologic conditions. Each chapter is organized in a readable and helpful format. Principles of diagnosis, differential diagnosis, and considerations for therapy are discussed in clinically related chapters.   There are chapters dedicated to cosmetic and surgical procedures with helpful insights. As a result, the book will be useful to more procedure-focused physicians as well. The questions and answers at the end of each chapter were also updated with new questions in order to make the learning process more interactive. It is hoped that the reader will gain from this edition as much as the editor did in preparing it. 9/18/14 3:15 PM Chapter 1 Hair Findings Paradi Mirmirani and Jennifer Ahdout DEVELOPMENT MICROSCOPIC STRUCTURE (FIG. 1-2) • Follicles form during third month of gestation; form first on head • Lining of follicle = ectodermal origin • Dermal papilla = mesodermal origin • Epidermal invaginations occur at an angle to the surface and over sites of mesenchymal cell collections • Eventually these epidermal cells form a column that surrounds the mesenchymal dermal papilla to form the bulb • The dermal papilla (along with “stem” cells in the bulge) induces hair follicle formation by the overlying epithelium • Additionally, 2 or 3 other collections of cells form along the follicle: • Upper collection becomes the mantle from which the sebaceous gland will develop • Lower swelling becomes the attachment for the arrector pili muscle and where follicle germinal cells reside in telogen phase • If a third collection of cells exists, it is found opposite and superior to the sebaceous gland and develops into the apocrine gland • The hair follicle is arranged in concentric circles (from outer to inner) • Basement membrane (glassy membrane): PAS-positive, acellular; thin during anagen and thickens during catagen • Outer root sheath (ORS): present the length of the follicle; never keratinizes; stays fixed in place • Inner root sheath (IRS): grows toward cell surface and separates from the hair shaft at the level of the sebaceous gland –– Henle layer: one cell thick and first to cornify –– Huxley layer: two cells thick; eosinophilic-staining trichohyalin granules –– Cuticle • Hair shaft: grows toward cell surface; cornifies without trichohyalin or keratohyalin granules • Cuticle: shingle-like hair cells that interlock with cuticle cells of IRS • Cortex: arises from cells in center of hair bulb; disulfide bonds in this region give hair its tensile strength; keratinizes to form shaft; contains pigment of hair • Medulla: contains melanosomes; found only in terminal hairs • Hair cycle: human follicles (Fig. 1-1) cycle in an asynchronous pattern (adjacent hairs in different stages) • Anagen: growth phase, stages I–VI –– Eighty-four percent of hair follicles at any one time; last a few months to 7 years –– Cells in the hair bulb are actively dividing • Catagen: transitional or degenerative stage –– Two percent of hair follicles at any one time –– Last a few days to weeks –– Matrix cells have stopped dividing –– Incomplete keratinization –– Thickened basement membrane (glassy layer) –– Transient, lower portion of follicle is broken down • Telogen: resting phase –– Fourteen percent of hair follicles at any one time –– Lasts about 3 months STRUCTURE (FIG. 1-1) • Longitudinal structure: (superior to inferior) • Permanent portion of the hair follicle –– Infundibulum –– Area of the sebaceous gland –– Isthmus: begins at sebaceous gland and ends at the bulge (site of insertion of arrector pili muscle) –– Area of the bulge: location of follicular stem cells • Transient portion of the hair follicle –– Lower hair follicle –– Hair bulb: contains the matrix, melanocytes; envelopes the dermal papilla; critical line of Auber is at the widest diameter; below this line is the bulk of mitotic activity 1 Ali-Ch01_p0001-018.indd 1 9/15/14 9:47 AM 2 Chapter 1 Hair Findings Hair cycle and anatomy Catagen Telogen Outer root sheath Anagen stage Anagen Infundibulum Epidermis Hair Sebaceous gland Bulge Exogen Bulge Sec Grm Matrix Dermal papilla Bulge Bulge Suprabulbular area Bulb Hair medulla Hair cortex Hair cuticle Companion layer Huxley layer Henle layer Inner root sheath Cuticle Outer root sheath Connective tissue sheath FIGURE 1-1  Hair cycle and anatomy. The hair follicle cycle consists of stages of rest (telogen), hair growth (anagen), follicle regression (catagen), and hair shedding (exogen). The entire lower epithelial structure is formed during anagen and regresses during catagen. The transient portion of the follicle consists of matrix cells in the bulb that generate 7 different cell lineages, 3 in the hair shaft, and 4 in the inner root sheath. (Reprinted with permission from Goldsmith LA et al, Fitzpatrick’s Dermatology in General Medicine, 8th Ed. New York: McGraw-Hill; 2012.) –– “Club hair”; no inner root sheath –– Dermal papilla retracted to higher position in dermis • Hair pigmentation • Pigment comes from melanocytes located in the matrix, above the dermal papilla • Eumelanin: pigment of brown-black hair • Pheomelanin: pigment of blonde-red hair • Loss of melanocytes and decreased melanosomes cause graying of hair—poliosis (can be seen in regrowth of hair after alopecia areata). In youth, catalase breaks down hydrogen peroxide so that the pigmentation of hair is retained. With aging, the protective function of catalase is lost, and hydrogen peroxide builds up and turns hair gray or white. Ali-Ch01_p0001-018.indd 2 • Hair growth • Hair grows approximately 0.35 to 0.37 mm/d • Longer anagen phase = longer hair HAIR DISORDERS Alopecia, Nonscarring Diffuse 1. Telogen effluvium • Hair shedding, often with an acute onset • Reactive process, response to a physical event (surgery, pregnancy, thyroid disease, iron deficiency, high fever), medications (Table 1-1), or severe mental or emotional stress 9/15/14 9:48 AM 3 HAIR DISORDERS FIGURE 1-2  Morphology and fluorescent A B E C microscopy of human hair follicle at distinct hair cycle stages. A–D. Morphology of human hair follicle during telogen (A), late anagen (B), and early and late catagen (C, D). (E) Immunofluorescent visualization of the melanocytes (arrows) in the hair bulb of late anagen hair follicle with anti–melanomaassociated antigen recognized by T cells antibody. (F) Immunofluorescent detection of proliferative marker Ki-67 (arrows) and apoptotic TUNEL+ cells (arrowheads) in early catagen hair follicle. FP = follicular papilla; HM = hair matrix. (Reprinted with permission from Goldsmith LA et al. Fitzpatrick’s Dermatology in General Medicine, 8th Ed. New York: McGraw-Hill; 2012.) D F A large number of hairs shift from anagen to telogen at one time • Telogen hairs move back to anagen in 3 to 4 months following the inciting event; hair density may take 6 to 12 months to return to baseline • The percentage of hairs in telogen rarely goes beyond 50% • Positive pull test: more than 6 telogen hairs • Telogen hairs on hair mount (Fig. 1-3) • Histology: increased number of telogen hairs • Prognosis: recovery is spontaneous and occurs within 6 months if inciting cause is reversed. Regrowing hairs with tapered or pointed hairs can be seen in the recovery phase 2. Loose anagen syndrome • Fair-haired children with thin, sparse, hair; no need for haircuts; easily dislodgable hair • Examination reveals sparse growth of thin, fine hair and diffuse or patchy alopecia • Anagen hairs are easily and painlessly pulled from scalp • Ali-Ch01_p0001-018.indd 3 Diagnosis: epilated hairs are predominantly in anagen phase; hair mount shows distorted anagen bulb, ruffled cuticle (Fig. 1-4) • Histology: premature and abnormal keratinization of the inner root sheath • Improves with age 3. Anagen effluvium (aka anagen arrest) • Hair broken off and not shed • Radiation therapy and chemotherapy agents; occurs 2 to 4 weeks after treatment • Hair shafts are abruptly thinned (Pohl-Pinkus constrictions) and break off at skin surface • Other causes: mercury intoxication, boric acid intoxication, thallium poisoning, colchicine, severe protein deficiency • Histology: normal follicles • Patchy 1. Alopecia areata (Fig. 1-5) • Abrupt onset 9/15/14 9:48 AM 4 Chapter 1 Hair Findings Table 1-1  ommon Medications Causing C Telogen Effluvium Angiotensin-converting enzyme inhibitors (ACEIs) Anticancer Anticoagulation (heparin, coumadin) Anticonvulsant (sodium valproate, carbamazepine) Selective serotonin reuptake inhibitors (SSRIs), tricyclic antidepressants (TCAs), and other psychiatric medications (amitriptyline, doxepin, haloperidol, lithium, haloperidol) FIGURE 1-4  Hair mount showing a dystrophic anagen hair with a ruffled cuticle in a patient with loose anagen syndrome. (Used with permission from Dr. Paradi Mirmirani.) Antigout (probenecid, allopurinol) • Antithyroid (methimazole, propylthiouracil) • β-blockers (propanolol, timolol) Antibiotics (nitrofurantoin, sulfasalazine) Oral contraceptives: containing progestins with high androgen potential Other (amiodarone, indomethacin, vitamin A, oral contraceptives) • • • • Exclamation point hairs which are broken hairs that are tapered at the scalp (Fig. 1-6) Pigmented hair affected first, subsequently gray hair may also be targeted (Fig. 1-7) Peach- or salmon-colored scalp Hair pull test positive for telogen hairs when disease is active • • • • • Follicular damage in anagen; then rapid transformation into telogen Alopecia totalis: total scalp hair loss Alopecia universalis: total scalp and body hair loss Ophiasis: localized hair loss along the periphery of the scalp Nails: pitting, mottled lunula, trachyonychia, or onychomadesis Histology: peribulbar infiltrate of T cells and macrophages (“swarm of bees”) Associations: In the patient: atopic disorders, thyroid disease, vitiligo. In the family: atopic disorders, thyroid disease, vitiligo, diabetes mellitus, pernicious anemia, systemic lupus erythematosus (other autoimmune conditions) FIGURE 1-3  Hair mount showing a telogen hair. (Reprinted with permission from Weedon D, ed. Weedon’s Skin Pathology, 3rd Ed. London: Churchill Livingston Elsevier; 2010.) Ali-Ch01_p0001-018.indd 4 FIGURE 1-5  Patchy alopecia areata. (Used with permission from Dr. Paradi Mirmirani.) 9/15/14 9:48 AM 5 HAIR DISORDERS Treatment: Patchy, or more than 50%: intralesional steroids, minoxidil 5% solution or foam, anthralin, topical steroids. Unresponsive or extensive: topical immunotherapy (squaric acid dibutylester [SADBE] or diphenylcyclopropenone [DPCP]), systemic cortisone (short-term or bridge treatment), psoralen plus ultraviolet A (UV-A), excimer laser. 2. Trichotillomania • Impulse-control disorder • Repeated plucking or pulling of hairs • Confluence of short, sparse hairs within an otherwise normal area of the scalp • Varying lengths of regrowth, “friar tuck” distribution of hair loss (Fig. 1-8) • Regrowing hair is blunt tipped instead of pointed • Eyebrows and upper eyelashes may be affected • Often have other habits: nail biting, skin picking • Histology: pigment casts, increased catagen hairs, trichomalacia • Treatment: psychological intervention and/or psychiatric medication to modify behavior 3. Pityriasis amiantacea (Fig. 1-9) • Thick scale, matted hair • May mimic severe seborrheic dermatitis or psoriasis; however, hair that is involved is easily dislodged on attempts to physically remove the scale • Treatment: keratolytics, corticosteroids, oil, improves with age • FIGURE 1-6  Exclamation point hairs in alopecia areata. (Used with permission from Dr. Paradi Mirmirani.) FIGURE 1-7  Alopecia areata primarily affecting pigmented hairs. (Used with permission from Dr. Paradi Mirmirani.) Ali-Ch01_p0001-018.indd 5 FIGURE 1-8  Trichotillomania. (Used with permission from Dr. Paradi Mirmirani.) 9/15/14 9:48 AM
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