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

Int J Dermatol. 2016 May;55(5):e256-61. doi: 10.1111/ijd.13149. Epub 2015 Dec 29.

Abstract

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 (≥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.

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.

MeSH terms

  • Adolescent
  • Adult
  • Aged
  • Aged, 80 and over
  • Alopecia / etiology
  • Brazil / epidemiology
  • Eosinophilia / etiology
  • Female
  • Follow-Up Studies
  • Hair Follicle*
  • Humans
  • Male
  • Middle Aged
  • Mycosis Fungoides / epidemiology*
  • Mycosis Fungoides / pathology*
  • Mycosis Fungoides / therapy
  • Neoplasm Staging
  • Pruritus / etiology
  • Retrospective Studies
  • Skin Neoplasms / epidemiology*
  • Skin Neoplasms / pathology*
  • Skin Neoplasms / therapy
  • Survival Rate
  • Young Adult