肉芽肿性皮肤松弛症4例分析  

Analysis of Four Patients with Granulomatous Slack Skin

作　　者：曾跃平[1] 刘洁[1] 渠涛[1] 宿斌[1,2] 李峰[1] 

机构地区：[1]中国医学科学院北京协和医学院北京协和医院皮肤科,北京100730 [2]首都医科大学附属北京儿童医院皮肤科

出　　处：《中国皮肤性病学杂志》2010年第6期528-530,共3页The Chinese Journal of Dermatovenereology

摘　　要：目的分析肉芽肿性皮肤松弛症(GSS)的临床病理特点、诊断和治疗,提高对本病的认识。方法回顾性分析近5年北京协和医院诊治的4例GSS患者的临床和实验室检查资料。结果 4例患者平均发病年龄25.5岁,早期临床表现不特异,最后均出现GSS典型的皮损及组织病理表现;患者有不同程度外周血嗜酸性粒细胞(EOS)计数、血清β2微球蛋白(β2-MG)和血清血管紧张素转换酶(ACE)水平升高,血清白蛋白(Alb)水平降低;3例皮损TCR基因重排阳性,1例阴性;予多种方法治疗后取得一定疗效。结论 GSS的诊断应结合临床特点、组织病理学、免疫组化、弹力纤维染色和基因重排检测。本病治疗困难,其标准治疗方案还需进一步研究。Objective To study the elinicopathological, diagnostic and therapeutic features of granulomatous slack skin （GSS）. Methods The group included four patients with GSS in Peking Union Medical College Hospital in recent 5 years. Their clinical and laboratory data were analyzed retrospectively. Results The mean age of onset was 25.5 years. The initial skin manifestations of these patients were unremarkable. All of four patients finally presented with typical clinical and histological manifestations of GSS. Increased values of blood eosinophil count, serum β2 microglobulin, serum angiotensin-converting enzyme and down-regulated values of serum albumin could be detected in these patients. Clonal rearrangement of T-cell receptor gene could be detected in lesional skin of three patients. They were treated with varied modalities with some improvement. Conclusion Diagnosis of GSS should combine the characteristic skin lesions, histology and immunohisto- chemistry, clonal rearrangement of TCR gene detection. There is no effective therapy for GSS, and its standard therapy scheme needs further study.

关 键 词：肉芽肿性皮肤松弛症 皮肤T细胞淋巴瘤 

分 类 号：R739.5[医药卫生—肿瘤]