Abstract

Background: Folliculotropic mycosis fungoides (FMF) is a rare variant of cutaneous T-cell lymphoma, characterizedby the infiltration of atypical lymphocytes into hair follicles (folliculotropism). Design: A single centre retrospective cohort analysis. Objective: To provide additional data regarding clinicopathological features, response to treatment, prognosis and disease course of FMF. Methods: We searched cutaneous lymphoma database for patients with FMF. FMF was defined by both clinical and histological features, including immunohistochemical studies and molecular genetic analysis of T-cell receptor genes. Results: Ten patients (8 male, 2 female) with a median age at diagnosis of 45 years were included. The median duration of the presence of skin lesions before diagnosis was 11 years. The most common sites of involvement were head and neck (80%), trunk and extremities (80-90%). Clinical features included patches and plaques, often associated with follicular prominence and alopecia, or comedo-like lesions, nodules and cysts. Fifty percent complained of pruritus. Histological findings included folliculotropism and/or follicular mucinosis (present in 80%). Initial therapy consisted of psoralen plus UV-A (PUVA) treatment in 50% of our patients. Only 2 out of 10 patients achieved complete remission. At the conclusion of this study 3 patients had died of lymphoma progression. Conclusions: Our findings support the current classification of folliculotropic mycosis fungoides as a distinct entity in the WHO-EORTC classification of cutaneous lymphomas, with distinct clinicopathological features, survival and therapeutic responses.

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