Generalized syringotropic mycosis fungoides responsive to extracorporeal photopheresis

Abstract

Funding sources: none. Conflicts of interest: none declared. Dear Editor, Syringotropic mycosis fungoides (STMF), a rare variant of mycosis fungoides (MF) and a subtype of follicular MF based on current classification,1 classically presents with punctate erythematous papules or plaques on the trunk or limbs, often with alopecia or anhidrosis, and typically follows an indolent clinical course. Because of the perieccrine localization of the dermal infiltrates, STMF is often less responsive to skin‐targeted therapies than classic MF, and given the rarity of the disease there is no accepted treatment approach, especially for those with generalized disease. A 72‐year‐old white man presented with a 10‐year history of generalized severe pruritus, scored as 10/10 on the numerical rating scale (NRS),2 with erythematous papules and plaques on his trunk and extremities, associated with generalized patchy hair loss. He reported no improvement with topical corticosteroids or emollients. Clinical examination revealed erythematous plaques, folliculotropic papules with perifollicular erythema (Fig. 1a) and large, open comedones on his upper mid‐back, flanks and thighs, amounting to a body surface area involvement of 15%. There was focal alopecia of the frontal and temporal scalp, without loss of follicular orifices, and diffuse thinning of the eyebrows and body hair. There was no evidence of organomegaly or lymphadenopathy on clinical examination. Routine laboratory testing, lactic dehydrogenase and flow‐cytometric analysis of circulating T‐cell subsets were normal.