肉芽肿性皮肤松弛症六例临床及病理研究

Clinicopathological analysis of six patients with granulomatous slack skin

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1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。

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^＋ lymphocytosis. Elastic fiber staining was performed in 4 patients, and showed crimp, fracture and disappearance of elastic fibers, and elastic fiber phagoeytized by multinuclear giant cells in 2 patients. There were monoclonal rearrangements of T-cell receptor （TCR） γgene 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.