中华皮肤科杂志 ›› 2009, Vol. 42 ›› Issue (4): 230-233.

• 论著 • 上一篇    下一篇

亲毛囊性蕈样肉芽肿四例分析

陈昆1,姜祎群1,李诚让1,弓娟琴1,顾恒1,常宝珠1   

  1. 1.中国医学科学院北京协和医学院皮肤病研究所
  • 收稿日期:2008-05-19 修回日期:2008-07-11 出版日期:2009-04-15 发布日期:2009-04-14
  • 通讯作者: 常宝珠 email:cbz1950@yahoo.com.cn

Analysis of four cases of folliculotropic mycosis fungoides

  • Received:2008-05-19 Revised:2008-07-11 Online:2009-04-15 Published:2009-04-14

摘要:

报道4例亲毛囊性蕈样肉芽肿。男3例,女1例,年龄32 ~ 52岁。3例以头面部及颈部受累为主,密集多发性暗红色不规则浸润性斑块、结节、肿块、毛囊性丘疹及痤疮样皮损,躯干、四肢表现为斑片、轻度浸润的斑块、毛囊性丘疹及痤疮样皮损。1例头面部未受累,全身密集毛囊性丘疹。组织病理显示真皮内、血管周围及毛囊周围大量淋巴样细胞为主的团块状浸润,部分细胞异形,浸润细胞移入毛囊上皮是他们共同而显著的特征,而亲表皮现象不明显。其中2例毛囊内充满黏蛋白,阿新蓝染色阳性。免疫组化显示浸润细胞主要为CD4阳性T细胞。对常规用于经典蕈样肉芽肿的治疗反应差,病情难以完全缓解,即使部分缓解后也极易复发。

关键词: 毛囊性蕈样肉芽肿;临床;组织病理;治疗

Abstract:

Here four cases of folliculotropic mycosis fungoides are reported. Of these patients, one was a female and three were males with the age varying from 32 to 52 years. Three patients presented with multiple, densely distributed, irregularly shaped, dark red infiltrated plaques, nodules, tumors, follicular papules and acneiform lesions preferentially distributed on the head and neck, as well as patches and mildly infiltrated plaques, follicular papules and acneiform lesions on the trunk and extremities. One patient presented with follicular papules all over the body with the exception of head and face. Characteristic findings of histopathology included massive lymphoid cell infiltration, heteromorphism of some cells and migration of infiltrated cells into follicular epithelium. No typical epidermotropism was noticed. Two cases showed muciprotein deposition in follicular epithelium, which was positive for alcian blue staining. The infiltrates were predominated by CD4+ T lymphocytes. Folliculotropic mycosis fungoides is a refractory disease with poor response to conventional therapy of classical mycosis fungoides. Relapse is common in patients with partial remission.