JAAD

Journal of the
American
Academy of

Dermatology
www.jaad.org March 2018 / Volume 78 / Number 3

CONTENTS
Continuing 433 Merkel cell carcinoma: An update and review
Medical Pathogenesis, diagnosis, and staging
Kathleen Coggshall, MD, Tiffany L. Tello, MD, Jeffrey P. North, MD, and
Education Siegrid S. Yu, MD
See related article on page 457
443 CME examination

444 Answers to CME examination (Identification No. JA0318),

March 2018 issue of the Journal of the American Academy of
Dermatology

445 Merkel cell carcinoma: An update and review

Current and future therapy
Tiffany L. Tello, MD, Kathleen Coggshall, MD,
Sue S. Yom, MD, PhD, MAS, and Siegrid S. Yu, MD
See related article on page 457
455 CME examination

456 Answers to CME examination (Identification No. JB0318),

March 2018 issue of the Journal of the American Academy of
Dermatology

Original Articles 457 Merkel cell carcinoma: Current US incidence and projected
increases based on changing demographics
Bottom line: The incidence of MCC in 2025 is forecast to be [3000 new
cases/year. Given this increasing impact and newly available therapies,
more MCC-focused education is needed.
Kelly G. Paulson, MD, PhD, Song Youn Park, MD, Natalie A. Vandeven, PhD,
Kristina Lachance, MS, Hannah Thomas, BS, Aude G. Chapuis, MD,
Kelly L. Harms, MD, PhD, John A. Thompson, MD, Shailender Bhatia, MD,
Andreas Stang, MD, MPH, and Paul Nghiem, MD, PhD
See related articles on pages 433 and 445

Continued on page A14

Copyright ª 2018 by the American Academy of Dermatology, Inc. Journal of the American Academy of Dermatology (ISSN: 0190-9622), is published monthly by
Elsevier Inc., 230 Park Avenue, Suite 800, New York, NY 10169-0901, USA. Periodicals postage paid at New York, NY and additional mailing offices.
POSTMASTER: Send address changes to Elsevier, Journal Returns, 1799 Highway 50 East, Linn, MO 65051, USA.

J AM ACAD DERMATOL MARCH 2018 A9

Contents continued

464 Cancer risk in patients with Behçet disease: A nationwide

population-based dynamic cohort study from Korea
Bottom line: Physicians should carefully monitor patients with Behçet
disease for the potential development of malignancy.
Sae Jung Na, MD, Min Ji Kang, MD, Dong Soo Yu, MD, PhD,
Kyung-Do Han, PhD, Ji Hyun Lee, MD, PhD, Yong Gyu Park, PhD, and
Young Bok Lee, MD, PhD

471 Prevalence and clinicopathologic characteristics of multiple

myeloma with cutaneous involvement: A case series from Korea
Bottom line: Cutaneous involvement in MM was associated with reduced
overall survival.
Yu Ri Woo, MD, Jong Sic Kim, MD, Ji Hong Lim, MD, Sewon Hwang, MD,
Miri Kim, MD, PhD, Jung Min Bae, MD, PhD, Young Min Park, MD, PhD,
Chang-Ki Min, MD, PhD, Dong-Wook Kim, MD, PhD, and
Hyun Jeong Park, MD, PhD
See related letter on page 621

479 Clinicopathologic features of 28 cases of nail matrix nevi (NMNs)

in Asians: Comparison between children and adults
Bottom line: In pediatric cases of even-width, sharply demarcated
longitudinal melanonychia, the presence of Hutchinson's sign with
longitudinal brushy pigmentation may favor a diagnosis of nevus.
Jae Ho Lee, MD, Youngkyoung Lim, MD, Ji-Hye Park, MD,
Jong Hee Lee, MD, PhD, Kee-Taek Jang, MD, PhD, Eun Ji Kwon, MD, and
Dong-Youn Lee, MD, PhD

490 Epidemiology of malignant cutaneous granular cell tumors: A US

population-based cohort analysis using the Surveillance,
Epidemiology, and End Results (SEER) database
Bottom line: These results may facilitate identifying and managing
high-risk patients and prioritizing surgical treatment.
Fatima N. Mirza, MPH, Charles T. Tuggle, MD, MHS,
Cheryl K. Zogg, MSPH, MHS, Humza N. Mirza, and Deepak Narayan, MD

498 The spectrum of manifestations in desmoplakin gene (DSP)

spectrin repeat 6 domain mutations: Immunophenotyping and
response to ustekinumab
Bottom line: Response to treatment with ustekinumab on the basis of this
discovery supports the value of “personalized” targeted immunotherapy.
Amy S. Paller, MSc, MD, Tali Czarnowicki, MD, MSc, Yael Renert-Yuval, MD,
Kristen Holland, MD, Thy Huynh, MD, Muriel Sadlier, MB, BCh,
Maeve A. McAleer, MB, BCh, Gary Tran, MD, Gabrielle C. Geddes, MD,
Alan D. Irvine, MD, DSc, and Emma Guttman-Yassky, MD, PhD

506 Cause-specific mortality in adults with atopic dermatitis

Bottom line: Health care practitioners and patients should be aware of risk
factors associated with these disorders in adults with atopic dermatitis.
Jacob P. Thyssen, MD, PhD, Lone Skov, MD, PhD, and
Alexander Egeberg, MD, PhD

Continued on page A18

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511 Pityrosporum folliculitis: A retrospective review of 110 cases

Bottom line: Pityrosporum folliculitis responds to treatment with topical
or oral azole antifungal agents.
Brea Prindaville, MD, Leah Belazarian, MD, Nikki A. Levin, MD, PhD, and
Karen Wiss, MD

515 Subclinical sensitization with diphenylcyclopropenone is

sufficient for the treatment of alopecia areata: Retrospective
analysis of 159 cases
Bottom line: Sensitization to induce an eczematous reaction may not be
required for successful contact immunotherapy.
Sung Jay Choe, MD, Solam Lee, MD, Long Quan Pi, PhD, Dong In Keum, MD,
Chung Hyeok Lee, MD, Beom Jun Kim, MD, and Won-Soo Lee, MD, PhD
See related letter on page 599

522 Development and validation of the Frontal Fibrosing Alopecia

Severity Score
Bottom line: The Frontal Fibrosing Alopecia Severity Score can be used in
clinical practice and for research purposes.
David Saceda-Corralo, MD, Óscar Mu~ noz Moreno-Arrones, MD,
Pablo Fonda-Pascual, MD, Cristina Pindado-Ortega, MD,
Diego Buendı́a-Casta~no, MD, Adrián Alegre-Sánchez, MD,
Gonzalo Segurado-Miravalles, MD, Ana Rita Rodrigues-Barata, MD,
Pedro Jaén-Olasolo, MD, PhD, and Sergio Va~no-Galván, MD, PhD

Dermatopathology 530 Unrelated immunodeficiency states may impact outcomes and

immune checkpoint molecule expression in patients with
mycosis fungoides: A clinicopathologic case-control study
Bottom line: Immunodeficient patients with mycosis fungoides may be
good candidates for immune checkpoint inhibitor therapy.
Shay Warren, BS, Meenal Kheterpal, MD, Patricia L. Myskowski, MD,
Alison Moskowitz, MD, Steven M. Horwitz, MD, and Melissa P. Pulitzer, MD

From the Academy 540 Guidelines of care for the management of basal cell carcinoma
Work Group: Christopher Bichakjian, MD, (Co-Chair),
April Armstrong, MD, MPH, Christian Baum, MD,
Jeremy S. Bordeaux, MD, MPH, Marc Brown, MD, Klaus J. Busam, MD,
Daniel B. Eisen, MD, Vivek Iyengar, MD, Clifford Lober, MD, JD,
David J. Margolis, MD, PhD, Jane Messina, MD, Alexander Miller, MD,
Stanley Miller, MD, Eliot Mostow, MD, MPH, Christen Mowad, MD,
Kishwer Nehal, MD, Kristi Schmitt-Burr, Aleksandar Sekulic, MD, PhD,
Paul Storrs, MD, Joyce Teng, MD, PhD, Siegrid Yu, MD, Conway Huang, MD,
Kevin Boyer, MPH, Wendy Smith Begolka, MBS,
and Murad Alam, MD, (Co-Chair)
Invited Reviewers: John Y. S. Kim, MD, Jeffrey H. Kozlow, MD, MS,
Bharat Mittal, MD, Jeffrey Moyer, MD, Thomas Olencki, DO, and
Phillip Rodgers, MD

Continued on page A22

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560 Guidelines of care for the management of cutaneous squamous

cell carcinoma
Work Group: Murad Alam, MD, (Co-Chair), April Armstrong, MD, MPH,
Christian Baum, MD, Jeremy S. Bordeaux, MD, MPH, Marc Brown, MD,
Klaus J. Busam, MD, Daniel B. Eisen, MD, Vivek Iyengar, MD,
Clifford Lober, MD, JD, David J. Margolis, MD, PhD, Jane Messina, MD,
Alexander Miller, MD, Stanley Miller, MD, Eliot Mostow, MD, MPH,
Christen Mowad, MD, Kishwer Nehal, MD, Kristi Schmitt-Burr,
Aleksandar Sekulic, MD, PhD, Paul Storrs, MD, Joyce Teng, MD, PhD,
Siegrid Yu, MD, Conway Huang, MD, Kevin Boyer, MPH,
Wendy Smith Begolka, MBS, and Christopher Bichakjian, MD, (Co-Chair)
Invited Reviewers: John Y. S. Kim, MD, Jeffrey H. Kozlow, MD, MS,
Bharat Mittal, MD, Jeffrey Moyer, MD, Thomas Olenecki, DO, and
Phillip Rodgers, MD

Review 579 Langerhans cell histiocytosis: A neoplastic disorder driven by

Ras-ERK pathway mutations
Bottom line: Patients with BRAFV600E-positive LCH might benefit from
B-Raf inhibition; however, additional studies are needed to evaluate the
safety and efficacy of such treatment.
Gary Tran, MD, Thy N. Huynh, MD, and Amy S. Paller, MS, MD

Research Letters 591 Referral patterns to an osteoporosis clinic for dermatology

patients undergoing prolonged corticosteroid therapy
Christen Mowad, MD, Stacy F. Cohen, MD, Nicole Flandry Fussell, MD,
R. Erin Martin, MD, and Cassondra Andreychik Ellison, MD

592 Detection and characterization of IgG, IgE, and IgA

autoantibodies in patients with bullous pemphigoid associated
with dipeptidyl peptidase-4 inhibitors
Luca Fania, MD, PhD, Adele Salemme, BS, Alessia Provini, MD,
Gianluca Pagnanelli, MD, Maria Chiara Collina, MD,
Damiano Abeni, MD, MPH, Biagio Didona, MD, Giovanni Di Zenzo, PhD, and
Cinzia Mazzanti, MD

595 An open-label exploratory study evaluating the efficacy and

safety of ingenol mebutate gel 0.05% for the treatment of verruca
vulgaris
Neal D. Bhatia, MD

596 Frontal fibrosing alopecia and cutaneous comorbidities:

A potential relationship with rosacea
Cristina Pindado-Ortega, MD, David Saceda-Corralo, MD,
Diego Buendı́a-Casta~ no, MD, Pablo Fernández-González, MD,
Óscar M. Monero-Arrones, MD, Pablo Fonda-Pascual, MD,
Ana R. Rodrigues-Barata, MD, Pedro Jaén-Olasolo, MD, PhD, and
Sergio Va~
nó-Galván, MD, PhD

597 Successful treatment of moderate-to-severe alopecia areata

improves health-related quality of life
Lucy Y. Liu, BA, Brittany G. Craiglow, MD, and Brett A. King, MD, PhD

Continued on page A24

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599 Home-based contact immunotherapy with

diphenylcyclopropenone for alopecia areata is as effective and
safe as clinic-based treatment in patients with stable disease:
A retrospective study of 40 patients
Solam Lee, MD, and Won-Soo Lee, MD, PhD
See related article on page 515

601 Treatment for facial alopecia areata: A systematic review with

evidence-based analysis
Aizuri Murad, MRCS, MRCP, and Wilma Bergfeld, MD, FAAD

605 Comparison of cyclic and continuous 308-nm excimer laser

treatments for vitiligo: A randomized controlled noninferiority
trial
Jae Min Sung, MD, Jung Min Bae, MD, PhD, and Hee Young Kang, MD, PhD

607 The microbial flora of taxane therapyeassociated nail disease in

cancer patients
Cesar A. Virgen, MD, PhD, Viswanath R. Belum, MD, Mini Kamboj, MD,
Shari B. Goldfarb, MD, Victoria S. Blinder, MD, Ayca Gucalp, MD, and
Mario E. Lacouture, MD
See related letter on page e71

610 Satisfaction with care and likelihood to recommend ratings

attributed to dermatology trainees
Laura E. McDermott, MD, Margaretta Midura, BA,
Vassiliki Papagermanos, BA, Joslyn Kirby, MD, MS, Med,
Karolyn A. Wanat, MD, Leah T. Belazarian, MD,
Cory Dunnick, MD, Jessica S. Mounessa, BA, Stephanie A. Savory, MD,
Nidhi Avashia-Khemka, MD, Andrew Strunk, MA, and Amit Garg, MD

612 A cross-sectional study of YouTube videos about atopic

dermatitis
Benjamin Freemyer, BS, Brandy Drozd, BS, and Andrea Suarez, MD, PhD

613 Weight change and risk of onychomycosis: A nationwide cohort

study in Korea
Seong Jun Ha, BSc, Kyung Do Han, PhD, Yumee Song, MD, and
Ji Hyun Lee, MD, PhD

615 Hormonal and reproductive factors and incidence of basal cell

carcinoma and squamous cell carcinoma in a large, prospective
cohort
Catherine M. Olsen, PhD, Nirmala Pandeya, PhD, Bridie S. Thompson, PhD,
Jean Claude Dusingize, MBBS, Padmini Subramaniam, MBBS,
Christina M. Nagle, PhD, Adele C. Green, MBBS, PhD, Rachel E. Neale, PhD,
Penelope M. Webb, PhD, and David C. Whiteman, MBBS, PhD, for the QSkin
Study

618 Forehead horizontal primary closure: A retrospective analysis of

25 cases assessing for long-term eyebrow asymmetry
Logan M. Skelley, MD, Daniel S. Winchester, MD, and Randall K. Roenigk, MD

Continued on page A26

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620 Machine learning and melanoma: The future of screening

Tyler Safran, DEC, Alex Viezel-Mathieu, MDCM, Jason Corban, MDCM,
Ari Kanevsky, Stephanie Thibaudeau, MD, FRCSC, and
Jonathan Kanevsky, MDCM
See related commentary on page 643

621 Risk of melanoma in patients with multiple myeloma: A

Surveillance, Epidemiology, and End Results population-based
study
Timothy W. Chang, MD, Amy L. Weaver, MS, Jerry D. Brewer, MD,
Robert A. Kyle, MD, and Christian L. Baum, MD
See related article on page 471

623 A comparison of apremilast monotherapy and combination

therapy for plaque psoriasis in clinical practice: A Canadian
multicenter retrospective study
Arvin Ighani, BMSc, Jorge R. Georgakopoulos, BSc, Scott Walsh, MD, FRCPC,
Neil H. Shear, MD, FRCPC, and Jensen Yeung, MD, FRCPC

Dermatoethics 627 Maintenance of Certification: A grandfatherly ethical analysis

Consultations Benjamin K. Stoff, MD, MAB, Lionel Bercovitch, MD, and
Jane M. Grant-Kels, MD
See related letter on page e69

631 To fee or not to fee? The ethical issues of concierge

medicine/dermatology
Matthew Belanger, MS IV, and Jane M. Grant-Kels, MD

634 The ethics of medical marijuana in dermatology

Walter Liszewski, MD, Benjamin K. Stoff, MD, MAB, and Ronda S. Farah, MD

Dermatology Grand 637 Recurrent scarring papulovesicular lesions on sun-exposed skin

Rounds at the NIH in a 22-year-old man
Melissa A. Levoska, BS, Jeffrey I. Cohen, MD, Irini Manoli, MD, PhD,
Chyi-Chia Richard Lee, MD, PhD, Steven S. T. Ching, MD,
Jessica Shand, MD, MHS, Deborah Tamura, RN, MS,
Kenneth H. Kraemer, MD, and John J. DiGiovanna, MD

Commentary 643 Crowdsourcing dermatology: DataDerm, big data analytics, and

machine learning technology
Andrew J. Park, BA, Justin M. Ko, MD, MBA, and Robert A. Swerlick, MD
See related letter on page 620

Continued on page A30

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JAAD ONLINE Available at http://www.jaad.org

Dermoscopy Case e57 Crown vessels and follicular white dot: New dermoscopic
of the Month findings in a case of solitary reticulohistiocytoma
Biswanath Behera, MD, Laxmisha Chandrashekar, MD, DNB,
Debasis Gochhait, MD, DNB, Divya Gupta, MD, and
Devinder Mohan Thappa, MD

Therapeutic Pearl e61 The Frisbee maneuver: A novel method to abort acute attacks of
the Raynaud phenomenon
Paul Curtiss, BA, Gabriela Cobos, MD, Kristen Lo Sicco, MD, and
Andrew G. Franks, Jr, MD

Surgical Pearls e63 A quick template for skin grafts

Amir Varedi, MPH, and Glen M. Bowen, MD

e65 Fully digital Mohs map for micrographic surgery

Jonathan Kantor, MD, MSCE, MA

e67 The eyelid grid

Derek Beaulieu, BS, and Rajiv I. Nijhawan, MD

Notes & Comments e69 Response to: “Maintenance of Certification: A grandfatherly

ethical analysis”
Thomas D. Horn, MD, MBA, and Randall K. Roenigk, MD
See related article on page 627

e71 Reply to: “The microbial flora of taxane therapyeassociated nail

disease in cancer patients”
Dirk M. Elston, MD
See related letter on page 607

e73 In response to Lebwohl et al, “Psychiatric adverse events during

treatment with brodalumab: Analysis of psoriasis clinical trials”
Evan A. Rieder, MD

e75 Reply to: “Psychiatric adverse events during treatment with

brodalumab: Analysis of psoriasis clinical trials”
Mark Lebwohl, MD

In Memoriam e77 Clarence Carnot Evans, Jr, MD: A statesman for dermatology
Seymour Rand, MD, John J. DiGiovanna, MD, Howard I. Maibach, MD,
Rebat Halder, MD, Jonathan K. Wilkin, MD, Maria L. Chanco Turner, MD,
Lynn A. Drake, MD, and John J. Voorhees, MD

Iotaderma e79 Iotaderma #289

Robert I. Rudolph, MD, FACP

Continued on page A32

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A40 JAAD Case Reports article list
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A32 MARCH 2018 J AM ACAD DERMATOL