Follicular mycosis fungoides

We describe a patient with follicular mycosis fungoides (MF), a rare follic ulotropic variant of cutaneous T-cell lymphoma (CTCL). Follicular involveme nt in CTCL usually presents clinically as alopecia mucinosa associated hist ologically with follicular mucinosis. Follicular MF differs from alopecia m ucinosa/follicular mucinosis associated with MF with regard to its clinical presentation, histology and, presumably, prognosis. Our patient presented with the characteristic findings of follicular MF i.e, infiltrated plaques showing numerous enlarged, comedo-like follicular infundibula; histology wa s dominated by exclusive folliculotropism of atypical lymphocytes sometimes forming follicular Pautrier's microabscesses, and by lack of epidermotropi sm and follicular mucinosis. Despite photochemotherapy and treatment with o ral retinoids and interferon alpha, the patient's follicular MF rapidly dev eloped into a progressive CTCL with large tumorous lesions, but responded t o electron beam therapy. The course of our patient's disease confirms the n otion that follicular MF may be associated with a worse prognosis than clas sical ME However, electron beam irradiation induced remission of follicular MF that was maintained by a combination therapy consisting of extracorpore al photopheresis and interferon alfa.