Mycosis Fungoides of the Palms and Soles

dok_zlo — 05.12.2024

A 38-year-old man presented to the dermatology clinic with a 15-year history of a waxing and waning rash on his palms and soles, which had spread to his forearms over the past year. A skin biopsy that had been performed 5 years before presentation had shown nonspecific inflammation. The rash had not abated with treatments for possible palmoplantar pustulosis. Physical examination was notable for diffuse erythema of the palms and proximal forearms with overlying scabs and scaling (Panel A). A nodule on the volar aspect of the right forearm was also detected on palpation (Panel A, arrow). The soles showed patchy erythema and desquamation (Panel B). Palpable axillary and inguinal lymphadenopathy was also present. Skin biopsies of the palms, soles, and forearm were performed. Histopathological analysis of a tissue sample stained with hematoxylin and eosin showed a dense lymphocytic infiltration of the superficial and middle dermis with atypical lymphocytes migrating among epidermal keratinocytes — a finding known as epidermotropism (Panel C). The lymphocytes also formed intraepidermal aggregates known as Pautrier’s microabscesses (Panel C, arrows). Immunophenotyping identified the atypical lymphocytes as being positive for CD4 and negative for CD8, with down-regulation of CD7. Clonal T-cell receptor (TCR) gene rearrangement was also identified in the biopsy specimens. No abnormal cells or clonal TCR gene rearrangements were detected in the peripheral blood. A diagnosis of mycosis fungoides of the palms and soles was made. Treatment with subcutaneous interferon and topical glucocorticoids was given. After 6 months of treatment, the rash and the nodule had abated.
Chen Li, M.D. , and Wen-Hui Wang, M.D.Author
Published November 30, 2024
N Engl J Med 2024;391:2147
DOI: 10.1056/NEJMicm2401673
VOL. 391 NO. 22

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