中国麻风皮肤病杂志 ›› 2022, Vol. 38 ›› Issue (10): 736-740.doi: 10.12144/zgmfskin202210736

• 综述 • 上一篇    下一篇

嗜毛囊性蕈样肉芽肿的最新分类与治疗进展

冯曦微,李凡,王琳   

  1. 四川大学华西医院皮肤科，成都，610041
  • 出版日期:2022-10-15 发布日期:2022-08-23

Update of classification and treatment of follicular mycosis fungoides

FENG Xiwei, LI Fan, WANG Lin   

  1. Department of Dermatology, West China Hospital, Sichuan University, Chengdu 610041, China
  • Online:2022-10-15 Published:2022-08-23

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摘要： 嗜毛囊性蕈样肉芽肿（folliculotropic mycosis fungoides，FMF）是蕈样肉芽肿（mycosis fungoides，MF）的一种独特变异型，临床少见，组织学改变以毛囊为中心的非典型淋巴细胞浸润为特征。过去认为FMF预后普遍较经典型MF差，但近年研究发现FMF是一组异质性疾病，根据预后不同至少可以分为两类，一类为早期病变，以斑片、毛囊性丘疹、痤疮样皮疹为主要表现，预后良好；另一类为晚期病变，以结节、肿瘤为主要表现，或者出现皮肤外其他器官受累，预后差。故FMF治疗方式的选择应同经典型MF一样根据分类分期而定。本综述重点阐述了FMF的最新分类，预后因素及其治疗选择。

关键词: 嗜毛囊性蕈样肉芽肿, 蕈样肉芽肿, 皮肤T细胞淋巴瘤

Abstract: Folliculotropic mycosis fungoides (FMF) is a rare variant of mycosis fungoides, and is characterized by histological characters of perifollicular infiltration and folliculotropism of atypical lymphocytes. It have previously been reported that FMF had a worse prognosis than classic MF.  However, recent studies have shown that FMF can be divided into two different subtypes. Patients with patches, follicular papules and acneiform lesions are defined as early-stage FMF with favourable prognosis. The patients with infiltrated plaques, nodules, tumors, and/or extracutaneous involvement are defined as late-stage FMF with poor prognosis. Management of FMF patients should follow a stage-adapted therapeutic approach like classic MF. In this paper, the classification, prognostic factors and therapeutic options are reviewed.