de Feraudy, Sebastien

Elsensohn, Ashley N

Hosking, Anna-Marie

Kraus, Christina N

Shiu, Jessica

Smith, Janellen

English

eScholarship - University of California

UC IrvineUC Irvine Previously Published WorksTitleUlcerated nodules in a sporotrichoid distributionPermalinkhttps://escholarship.org/uc/item/0b89r935JournalJAAD Case Reports, 5(10)ISSN2352-5126AuthorsHosking, Anna-MarieKraus, Christina NElsensohn, Ashley Net al.Publication Date2019-10-01DOI10.1016/j.jdcr.2019.05.020Copyright InformationThis work is made available under the terms of a Creative Commons Attribution License, availalbe at https://creativecommons.org/licenses/by/4.0/ Peer reviewedeScholarship.org Powered by the California Digital LibraryUniversity of CaliforniaIMAGES IN DERMATOLOGYUlcerated nodules in a sporotrichoid distributionAnna-Marie Hosking, BS, Christina N. Kraus, MD, Ashley N. Elsensohn, MD, MPH, Jessica Shiu, MD, PhD,Sebastien de Feraudy, MD, PhD, and Janellen Smith, MDIrvine, CaliforniaFrom the Department of Dermatology, University of California,Irvine.Drs de Feraudy and Smith contributed equally to this work and areco-senior authors.Funding sources: None.Conflicts of interest: None disclosed.Correspondence to: Janellen Smith, MD, Professor, Department ofDermatology, UC Irvine Health, 118 Med Surg 1, Irvine, CA92697-2400. E-mail: Jesmith7@uci.edu.JAAD Case Reports 2019;5:925-7.2352-5126ª 2019 by the American Academy of Dermatology, Inc. Publishedby Elsevier, Inc. This is an open access article under the CCBY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).https://doi.org/10.1016/j.jdcr.2019.05.020925JAAD CASE REPORTSOCTOBER 2019926 Hosking et alA 42-year-old man presented with a 3-month history of persistent ulcerated nodules (Fig 1). Historywas relevantfor deployment to the Middle East, Africa, Turkey, and South East Asia. He also reported skinning squirrels andbutchering hogs on his farm in Missouri. Review of systems found intermittent fevers and night sweats. Priortreatment included multiple oral antibiotics without improvement. Examination found ulcerated, firm, pinknodules on the right forearm, elbow, and arm in sporotrichoid distribution without palpable lymphadenopathy.A punch biopsy was obtained and staining performed, including hematoxylin-eosin (Fig 2, A), CD30 (Fig 2, B),anaplastic lymphoma kinase (ALK-1) (Fig 2, C; control, Fig 2, D).Question 1: What is your diagnosis?A. Bacillus anthracisB. LeishmaniasisC. Lymphomatoid papulosis (LyP)D. Primary cutaneous anaplastic large cell lym-phoma (PC-ALCL)E. TularemiaAnswers:A. Bacillus anthracis e Incorrect. B anthracisis a spore-forming, toxin-producing bacteriafound in soil. Risk factors for cutaneous anthraxinclude living in Turkey (endemic area) andbutchering meat (mainly cows, also reported inpigs). However, ulcerations rapidly progress toeschars, and a CD301 infiltrate would not beobserved. Gram stain would find spore-formingrods.B. Leishmaniasis e Incorrect. Infection with Leish-mania results in a papule or nodule with centralulceration at the site of a sandfly bite. Although thepatient has risk factors for exposure to Old Worldleishmaniasis, geographically distributed from WestAfrica to Central Asia, histopathology would showLeishmania amastigotes within histiocytecytoplasm.1C. LyP e Incorrect. LyP presents as recurrentcrops of small papulonodules. Although LyP is onthe spectrum of CD301 lymphoproliferative disor-ders, lesions classically resolve and reoccur overweeks to months. Histopathology for type A LyPreveals a wedge-shaped dermal infiltrate of CD301lymphoid cells with numerous neutrophils andeosinophils.2D. PC-ALCL e Correct. PC-ALCL presents as asolitary tumor or several grouped papules/-nodules. Histopathology shows diffuse dermalinfiltration of CD301/ALK-1- atypical mononu-clear cells with hyperchromatic horseshoe-shaped nuclei, numerous mitoses, and apoptoticbodies.2E. Tularemia e Incorrect. Tularemia is caused bythe gram-negative coccobacillus Francisella tular-ensis. Although rabbits most commonly transmittularemia, squirrels can also be infected. The ulcer-oglandular form presents with a papule that pro-gresses to an ulcer with eschar formation.Histopathology shows suppurative granulomatousinflammation and would not show a CD301lymphoid infiltrate.Question 2: Which of the following immuno-histochemical markers would indicate that thediagnosis is likely systemic rather than primarycutaneous?A. Negative CD56B. Negative epithelial membrane antigen (EMA)C. Positive ALK-1D. Positive B-cell lymphoma 2 (Bcl-2)E. Positive cutaneous lymphocyte antigen (CLA)Answers:A. Negative CD56 e Incorrect. CD56 is expressedin 12% to 75% of PC-ALCL and is positive in systemicALCL and therefore does not help differentiatebetween these 2 entities.2B. Negative EMA e Incorrect. EMA is typicallynegative in PC-ALCL and positive in systemic ALCL.2C. Positive ALK-1 e Correct. Positive ALK-1would indicate that the ALCL is more likely sys-temic, as the t(2;5) gene rearrangement involvingthe ALK gene is an extremely rare event in PC-ALCLcompared with nearly 60% of all systemic ALCL.ALK-1 can help differentiate the 2 entities in inde-terminate cases.2D. Positive Bcl-2 e Incorrect. Bcl-2 does not helpdifferentiate between these 2 entities, as it is positivein 30% of PC-ALCL and is positive in systemicALCL.2E. Positive CLA e Incorrect. Most cases of PC-ALCL express CLA, whereas CLA is negative insystemic ALCL.2JAAD CASE REPORTSVOLUME 5, NUMBER 10Hosking et al 927Question 3:Which of the followingmedicationsis US Food and Drug Administration (FDA)-approved for the treatment of this condition?A. Bexarotene gelB. Brentuximab vedotin (BV)C. ImiquimodD. InterferonE. MethotrexateAnswers:A. Bexarotene gel e Incorrect. Bexarotene is FDAapproved for the topical treatment of cutaneouslesions in patients with cutaneous T-cell lymphoma,stage 1A and 1B, who have refractory or persistentdisease after other therapies or who have nottolerated other therapies. Data regarding the useof topical bexarotene gel are limited to case reportsand the control arm of the ALCANZA trial in whichthe response rate was low.3B. BV e Correct. BV is an anti-CD30 monoclonalantibody that is FDA approved for the treatment ofadult patients with PC-ALCL who have received atleast 1 prior systemic therapy.4 Approval was basedon a phase 3, randomized clinical trial (ALCANZA),which randomized patients to receive BV or eithermethotrexate or bexarotene. BV resulted in com-plete resolution of skin involvement in 10 of 16patients (63%) with PC-ALCL.3C. Imiquimod e Incorrect. The use of topicalimiquimod has been described in case reports andcase series; however, it is not FDA approved for PC-ALCL.5D. Interferon e Incorrect. Interferon is not FDAapproved for PC-ALCL, and because of its toxicity, itis generally reserved for patients who progress on orwho are intolerant to methotrexate or bexarotene.E. Methotrexate e Incorrect. Methotrexate is notFDA approved for PC-ALCL. However, low-dose(less than 25 mg/wk) is considered first-line therapyfor multifocal PC-ALCL when radiotherapy is notfeasible.2Patient Course: The patient underwent local radia-tion to the right arm, which he tolerated well, butnew lesions developed proximal to the radiated port.Thus, he was started on brentuximab for 4 cycles andexperienced complete remission.Abbreviations used:ALCL: anaplastic large cell lymphomaALK: anaplastic lymphoma kinaseBcl-2: B-cell lymphoma 2BV: brentuximab vedotinCLA: cutaneous lymphocyte antigenEMA: epithelial membrane antigenFDA: US Food and Drug AdministrationPC-ALCL: primary cutaneous anaplastic large celllymphomaREFERENCES1. Remadi L, Haouas N, Chaara D, et al. Clinical presentation ofcutaneous leishmaniasis caused by Leishmania major. DRM.2016;232(6):752-759.2. Sauder MB, O’Malley JT, LeBoeuf NR. CD301 Lymphoprolifer-ative disorders of the skin. Hematol Oncol Clin North Am.2017;31(2):317-334.3. Prince HM, Kim YH, Horwitz SM, et al. Brentuximab vedotin orphysician’s choice in CD30-positive cutaneous T-cell lym-phoma (ALCANZA): an international, open-label, randomised,phase 3, multicentre trial. Lancet. 2017;390(10094):555-566.4. FDA. Approved Drugs - FDA approves brentuximab vedotinfor the treatment of adult patients with primary cutaneousanaplastic large cell lymphoma. https://www.fda.gov/drugs/informationondrugs/approveddrugs/ucm584543.htm. AccessedDecember 30, 2018.5. Didona B, Benucci R, Amerio P, Canzona F, Rienzo O,Cavalieri R. Primary cutaneous CD301 T-cell lymphomaresponsive to topical imiquimod (Aldara). Br J Dermatol.2004;150(6):1198-1201.

Ulcerated nodules in a sporotrichoid distribution

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Ulcerated nodules in a sporotrichoid distribution.

Ulcerated nodules in a sporotrichoid distribution.

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