中华皮肤科杂志 ›› 2017, Vol. 50 ›› Issue (2): 95-98.

• 论著 • 上一篇    下一篇

肉芽肿性皮肤松弛症六例临床及病理研究

王光平1,2,王小坡2,陈浩3,张韡4,姜祎群3,曾学思3,徐秀莲3,孙建方3   

  1. 1. 天津市第一中心医院
    2. 中国医学科学院北京协和医学院皮肤病研究所
    3. 南京 中国医学科学院北京协和医学院皮肤病研究所
    4. 中国医学科学院皮肤病研究所
  • 收稿日期:2016-05-27 修回日期:2016-07-05 出版日期:2017-02-15 发布日期:2017-01-24
  • 通讯作者: 孙建方 E-mail:fangmin5758@aliyun.com
  • 基金资助:
    北京协和医学院创新基金;国家自然科学基金;江苏省自然科学基金;国家教育部博士点基金

Clinicopathological analysis of six patients with granulomatous slack skin

Guang-Ping WANG1,2, 3, 3,ZHANG Wei3,Yi-Qun JIANG3, 3,Xiu-Lian Xu   

  • Received:2016-05-27 Revised:2016-07-05 Online:2017-02-15 Published:2017-01-24
  • Supported by:
    Innovation Foundation of Peking Union Medical College;Natural?Science?Foundation?of?Jiangsu Province;Doctoral Fund of Ministry of Education of China

摘要: 目的 探讨6例肉芽肿性皮肤松弛症(GSS)的临床和病理特点。方法 收集6例GSS患者的临床和病理学资料。结果 6例均为男性,皮损为松弛斑块甚至垂悬状肿块,5例发生于皱褶部位(或为主),1例仅发生于右胸部。1例合并蕈样肉芽肿。组织病理均可见真皮内非干酪性肉芽肿,伴中等大小的淋巴样细胞、多核巨细胞浸润。1例见淋巴样细胞异形改变,余5例异形不明显。 1例伴有亲表皮现象。免疫组化均见CD4+ T细胞为主的单克隆增生。4例行弹性纤维染色见弹性纤维卷曲、断裂甚至消失,2例见多核巨细胞吞噬弹性纤维现象。2例行T细胞受体(TCR)基因重排,均为γ单克隆增生。2例患者采用浅层X线照射或电子束照射,皮损变小后手术切除,未再复发。其余4例患者采用干扰素α?2b肌内注射、0.02%氮芥外用等,症状有改善。结论 GSS进展缓慢,不推荐过度治疗。局限于皱褶部位的GSS推荐手术治疗。

Abstract: Wang Guangping, Wang Xiaopo, Chen Hao, Zhang Wei, Jiang Yiqun, Zeng Xuesi, Xu Xiulian, Sun Jianfang Department of Pathology, Institute of Dermatology, Chinese Academy of Medical Sciences and Peking Union Medical College, Nanjing 210042, China Corresponding author: Sun Jianfang, Email: fangmin5758@aliyun.com 【Abstract】 Objective To investigate clinicopathological features of granulomatous slack skin (GSS). Methods Clinical and pathological data were obtained from 6 cases of GSS, and analyzed retrospectively. Results All the 6 patients were males, and presented with slack plaques and even pendulous skin folds. These skin lesions mainly affected flexural areas in 5 patients, and only occurred in the right chest in 1 patient. One patient was diagnosed with GSS complicated by mycosis fungoides (MF). Histopathological examination showed non-caseating granulomas with infiltration of medium-sized lymphoid cells and multinucleated giant cells in the dermis in all the patients, atypical lymphoid cells in 1 patient, and epidermotropism in another patient. Immunohistochemical study revealed monoclonal CD4+ lympho-cytosis. Elastic fiber staining was performed in 4 patients, and showed crimp, fracture and disappearance of elastic fibers, and elastic fiber phagocytized by multinuclear giant cells in 2 patients. There were monoclonal rearrangements of T-cell receptor (TCR) γ gene in 2 patients. After radiotherapy with electron beam or superficial X-ray, lesions of 2 patients became smaller, and then were excised completely without recurrence. The other 4 patients received intramuscular injection of interferon α-2b and topical application of 0.02% mechlore-thamine solution, and their condition was improved. Conclusions GSS has a slowly progressive course, and overtreatment is not recommended. Surgical excision may be the first choice for GSS localized in flexural areas.