Interstitial mycosis fungoides: A rare presentation of mycosis fungoides with overlapping granulomatous and folliculotropic features.

Background: Interstitial mycosis fungoides (IMF) is a rare subtype of mycosis fungoides (MF) characterized by atypical lymphocytes infiltrating the reticular dermis between collagen bundles with limited epidermotropism and variable granulomatous features. Methods: Retrospective single institution review of 31 cases of IMF including clinical characteristics, disease course and pathological features. Results: Our cohort was predominately male (19; 61%, M:F 1.6:1) with a mean age at diagnosis of 43 years (range 11–85), mean signs/symptoms duration of 7 years prior to diagnosis, and 6 years mean follow‐up duration. Clinically, patients often exhibited symmetric ill‐defined patches/plaques involving intertriginous regions with tan‐yellow hyperpigmentation and follicular‐based papules, wrinkling, and alopecia. Lymphadenopathy was noted in seven patients. Fifteen (52%) patients were in near or complete clinical remission at the latest follow‐up. T‐cell receptor gene rearrangement was positive in 23/24 (96%) cases. Histopathologically, atypical cells were small–medium, CD4+ (29; 94%) or rarely CD4+/CD8+ (1; 3%) lymphocytes infiltrating the reticular dermis with thickened collagen bundles (27; 87%), multinucleated giant cells (12; 39%), and often tracing along adnexa with subtle folliculotropism (12/20; 60%). Conclusions: Our study demonstrates IMF is an indolent subtype of MF with distinct features, including frequent granulomatous and subtle follicular involvement resulting in alopecia. [ABSTRACT FROM AUTHOR]

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