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JAMA Dermatology Clinicopathological Challenge
February 19, 2020

Rapidly Evolving Necrotic Plaques and Nodules in a Middle-aged Woman

Author Affiliations
  • 1Department of Dermatology, University of Minnesota, Minneapolis
JAMA Dermatol. 2020;156(4):455-456. doi:10.1001/jamadermatol.2019.4992

A woman in her 50s presented to a tertiary dermatology referral center with scaly erythematous patches and plaques covering 10% of her total body surface area without clinically appreciable lymphadenopathy. Histopathological testing demonstrated an epidermotropic infiltrate of hyperchromatic, convoluted, atypical CD3+, CD4, and CD8+ lymphocytes with haloed nuclei with basilar tagging and papillary dermal fibrosis. Studies of T-cell gene rearrangements identified a clonal population. Blood-flow cytometry did not reveal aberrant T-cell immunophenotypes. She was treated initially with topical and intralesional corticosteroids and narrow band UV B phototherapy. Oral bexarotene was subsequently added for recalcitrant disease. Within 2 months, she developed isolated, painful tumors. A second biopsy was performed, which was notable for an epidermotropic infiltrate of CD3+, CD4, and CD8 weak lymphocytes, which were positive for TIA-1, granzymes, and B factor (Bf) 1 and negative for CD30 and CD56. The results of flow cytometry and imaging were unremarkable. After an initial favorable response with chlorambucil, she developed expanding ulcerated tumors complicated by a polymicrobial superinfection. Chlorambucil was discontinued in favor of antimicrobial treatment. She then received liposomal doxorubicin, although skin involvement continued to progress rapidly, with ulcerating tumors making up as much as 80% of her total body surface area. She was hospitalized for worsening disease and pain (Figure, A). Repeated histopathologic analysis was done (Figure, B, C, and D).

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