Folliculotropic mycosis fungoides: clinical and epidemiological evaluation in a single center in Brazil

BackgroundFolliculotropic mycosis fungoides (FMF) is a rare variant of mycosis fungoides with clinical peculiarities, refractoriness to conventional treatments, and worse prognosis when compared to classic mycosis fungoides. ObjectiveTo evaluate the clinical and epidemiological characteristics of FMF patients in a single center in Brazil. MethodsData were retrospectively collected from patients with FMF who attended the Cutaneous Lymphoma Clinic, University of SAo Paulo Medical School, between 1987 and 2013. ResultsThirty-three patients were included (median age 46 years old at diagnosis; 20 male; 27 white). The median disease duration before diagnosis was 3 years. Regarding stage at diagnosis, 61% had advanced stage disease (IIb). Follicular papules were reported in 66% and alopecia in 59% of the cases. The most involved regions were limbs, followed by trunk and head. Pruritus was present in 81% of the patients. The median time of patients' follow-up was 38 months. At the last follow-up visit, 67% of the patients were alive with active disease, 27% deceased, and 6% were in complete remission. Four patients had large cell transformation. At the time of diagnosis, 25% of the patients showed eosinophilia. LimitationsRetrospective study with partial unavailable data. ConclusionsThe characteristics of our patients with FMF correlated with aspects previously described in the literature, which were at a more advanced stage at diagnosis and had a less favorable outcome. Pruritus is a very common complaint. Large cell transformation should be monitored as it is implicated in poor prognosis.