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JAMA Dermatology Clinicopathological Challenge
August 7, 2019

Multiple Atrophic Papules and Plaques on the Trunk and Extremities of a Young Man

Author Affiliations
  • 1Department of Dermatology, Howard University Hospital, Washington, DC
  • 2Dermatology Branch, National Institute of Arthritis and Musculoskeletal and Skin Diseases, National Institutes of Health, Bethesda, Maryland
  • 3Division of Dermatology, Children’s National Health System, Washington, DC
JAMA Dermatol. 2019;155(9):1075-1076. doi:10.1001/jamadermatol.2019.2239

A young man in his late teens presented with numerous atrophic papules and plaques on his trunk and extremities that developed gradually over several years. Although most were asymptomatic, the patient was concerned about the ongoing appearance of new lesions and the associated pain that some lesions were causing. His medical history was significant for adenosine deaminase–deficient severe combined immunodeficiency (ADA-SCID), which had been managed since childhood with twice weekly intramuscular injections of pegademase bovine enzyme replacement, thrice weekly trimethoprim-sulfamethoxazole for Pneumocystis prophylaxis, and monthly infusions of intravenous immunoglobulin. Physical examination revealed 9 smooth-surfaced, skin-colored to hyperpigmented, 0.5- to 1.5-cm atrophic papules and plaques that exhibited the buttonhole sign on palpation (Figure, A). The multicentric lesions were located on the left knee, left thigh, bilateral chest, and back. Punch biopsies of lesions on his trunk and extremities were sent for histopathological examination with hematoxylin-eosin (H&E) staining (Figure, B and C), immunohistochemical (IHC) studies, and molecular evaluation with reverse transcription polymerase chain reaction.

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