Unilesional Follicular Mycosis Fungoides: Report of 6 Cases and Review of the Literature

摘要

Unilesional follicular mycosis fungoides (MF) is a rare of cutaneous T-cell lymphoma characterized by selective of the hair follicles by neoplastic T lymphocytes esenting initially as a solitary lesion occupying less than 5% of he body surface; there are 22 previous reported cases. We describe 6 with this rare variant of MF, all males (age range 6-64 years; age: 28 years) presenting with a solitary lesion primarily on the ace and scalp except 1 patient who presented with a truncal lesion, the patients had the lesions for at least a few months. The lesions vere associated with follicular prominence and hair loss. In each, the biopsies showed an atypical folliculotropic lymphocytic infiltrate by follicular mucinosis in certain cases. The infiltrate the lower isthmic part of the follicle, and in 1 case, there vas an alopecia areata-like neoplastic lymphomatoid bulbitis. There vas no evidence of large cell transformation nor was there a significant degree of infiltration of the interfollicular dermis or epidermis, Higher magnification disclosed marked cerebriform atypia amidst the lymphocytes. Phenotypic studies demonstrated a high CD4 to CD8 ratio in excess of 5 with a significant loss of CD7. One patient developed additional similar lesions mvolving the thigh and buttock lafter a period of at least 3-4 years of untreated unilesional MF. lUnilesional follicular MF is a potentially curable form of MF with la young male predilection. Early diagnosis and treatment interven-tion likely define a cornerstone for ensuring the best patient outcome and preventing clinical evolution to either tumor stage MF and or multilesional MF.