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Folliculotropic mycosis fungoides: clinical and epidemiological evaluation in a single center in Brazil
Author(s) -
Deonizio Janyana M. D.,
Ascef Rogers Diego,
Sanches José Antonio
Publication year - 2016
Publication title -
international journal of dermatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.677
H-Index - 93
eISSN - 1365-4632
pISSN - 0011-9059
DOI - 10.1111/ijd.13149
Subject(s) - mycosis fungoides , medicine , epidemiology , stage (stratigraphy) , dermatology , retrospective cohort study , single center , cutaneous lymphoma , peripheral t cell lymphoma , malignancy , eosinophilia , disease , lymphoma , surgery , paleontology , immune system , t cell , immunology , biology
Background Folliculotropic 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. Objective To evaluate the clinical and epidemiological characteristics of FMF patients in a single center in Brazil. Methods Data were retrospectively collected from patients with FMF who attended the Cutaneous Lymphoma Clinic, University of São Paulo Medical School, between 1987 and 2013. Results Thirty‐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 (≥ II b). 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. Limitations Retrospective study with partial unavailable data. Conclusions The 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.