Abstract
ABSTRACTIntroduction: Folliculotropic mycosis fungoides (FMF) is a cutaneous T cell lymphoma that is recognized as a distinct variant of mycosis fungoides (MF) due to its unique clinicopathologic features and its more aggressive disease course as compared to classic MF. Recent studies distinguished an indolent (early stage FMF) and a more aggressive (advanced stage FMF) subgroup, that require a different therapeutic approach.Areas covered: This review provides an overview of the evolving concepts and recent developments in FMF and summarizes various treatment options for early and advanced stages of FMF including skin-directed therapies, immunomodulating agents, HDAC inhibitors, targeted anti-CD30 therapy and the role of chemotherapy and haematological stem cell transplantation.Expert opinion: Recently defined prognostic subgroups show that early stage FMF patients have an indolent disease course and may benefit very well from skin-directed therapies, also used in early stage classic MF. Advanced skin limited FMF patients benefit most from radiotherapy based treatment modalities. Systemic chemotherapy is reserved for patients with extracutaneous dissemination or progressive disease that cannot be controlled with skin-directed or immunomodulating therapies.
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