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Ulcerated Plaques on the Scalp and Dorsal Hands of an Older Man

Educational Objective
Based on this clinical scenario and the accompanying image, understand how to arrive at a correct diagnosis.
1 Credit CME

A man in his 60s with a history of hypertension and hyperlipidemia presented to dermatology clinic with a 4-month history of fatigue, night sweats, and weight loss and 2 months of enlarging lesions on his scalp and hands. He reported no new medications or recent infection. His temperature was 37.5 °C (99.5 °F); heart rate, 102/min; blood pressure, 98/72 mm Hg; respiratory rate, 12/min; and oxygen saturation, 94% on room air. His skin examination revealed 3 tender, nonfluctuant 10-cm scalp plaques with overlying hemorrhagic crust and necrosis on an erythematous indurated base (Figure, left panel). Similar-appearing papules and plaques were present on the dorsal surfaces of his hands. The remainder of his physical examination was normal.

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Sweet syndrome with myelodysplastic syndrome (MDS)

B. Perform a bone marrow biopsy with flow cytometry and cytogenetics

The key to the diagnosis is the combination of cutaneous lesions characteristic of Sweet syndrome with constitutional symptoms (fatigue, night sweats, and weight loss), pancytopenia, and acute DVT, which may be associated with hematologic malignancy. Choice A is incorrect because the clinical suspicion for infection was low, with nonfluctuant skin lesions and no organisms identified on biopsy using special stains. Incision and drainage (choice C) may result in worsening of the lesions in response to trauma (pathergy). Although oral dapsone (choice D) may be used as a steroid-sparing agent, it is not the recommended first-line treatment for Sweet syndrome.

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Article Information

Corresponding Author: Amrita Goyal, MD, Department of Dermatology, University of Minnesota, 516 Delaware St SE, Minneapolis, MN 55455 (goyal046@umn.edu).

Published Online: April 28, 2022. doi:10.1001/jama.2022.6315

Conflict of Interest Disclosures: Dr O’Leary reported receiving fellowship support (T32 grant) from the National Institutes of Health (NIH). Dr Rosenbach reported receiving consulting fees from Merck, Janssen, Eli Lilly, AbbVie, and Novartis and grants from Processa. No other disclosures were reported.

Disclaimer: The content is solely the responsibility of the authors and does not necessarily represent the official views of the NIH.

Additional Contributions: We thank Mark A. Klein, MD, and Adina M. Cioc, MD (Minneapolis Veterans Affairs Medical Center), for clinical care of the patient and Laurel L. Wessman, MD (Caris Health, Wilmar, Minnesota), for providing the supplementary clinical photograph. These individuals received no compensation for their contributions. We also thank the patient’s family for providing permission to share the patient’s information.

References
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2.
Joshi  TP , Friske  SK , Hsiou  DA , Duvic  M .  New practical aspects of Sweet syndrome.   Am J Clin Dermatol. Published online February 14, 2022. doi:10.1007/s40257-022-00673-4PubMedGoogle ScholarCrossref
3.
Nelson  CA , Noe  MH , McMahon  CM ,  et al.  Sweet syndrome in patients with and without malignancy.   J Am Acad Dermatol. 2018;78(2):303-309.PubMedGoogle ScholarCrossref
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Heath  MS , Ortega-Loayza  AG .  Insights into the pathogenesis of Sweet’s syndrome.   Front Immunol. 2019;10:414. doi:10.3389/fimmu.2019.00414PubMedGoogle ScholarCrossref
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Taşkın  B , Vural  S , Altuğ  E ,  et al.  Coronavirus 19 presenting with atypical Sweet’s syndrome.   J Eur Acad Dermatol Venereol. 2020;34(10):e534-e535.PubMedGoogle ScholarCrossref
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Chirasuthat  P , Triyangkulsri  K , Rutnin  S ,  et al.  Cutaneous nontuberculous mycobacterial infection in Thailand.   Medicine (Baltimore). 2020;99(10):e19355.PubMedGoogle Scholar
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Sleiman  J , Hitawala  AA , Cohen  B ,  et al.  Systematic review: Sweet syndrome associated with inflammatory bowel disease.   J Crohns Colitis. 2021;15(11):1864-1876. doi:10.1093/ecco-jcc/jjab079PubMedGoogle ScholarCrossref
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Rochet  NM , Chavan  RN , Cappel  MA , Wada  DA , Gibson  LE .  Sweet syndrome: clinical presentation, associations, and response to treatment in 77 patients.   J Am Acad Dermatol. 2013;69(4):557-564.PubMedGoogle ScholarCrossref
9.
Merlant  M , Lepelletier  C , Battistella  M ,  et al.  Acute myeloid leukemia and myelodysplastic syndrome-associated Sweet syndrome.   J Am Acad Dermatol. 2021;84(3):838-840.PubMedGoogle ScholarCrossref
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Webber  L , Cummins  M , Mann  R , Shaw  L , Ghinai  R , Mahon  C .  Panniculitis in a 3-year-old child with Fanconi anemia–associated bone marrow hypoplasia heralds transformation to acute myeloid leukemia.   Pediatr Dermatol. 2019;36(5):725-727.PubMedGoogle ScholarCrossref
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