中华皮肤科杂志 ›› 2017, Vol. 50 ›› Issue (3): 182-185.

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大疱性系统性红斑狼疮六例临床病理分析

王莲1,李萌萌1,徐飞2,闫薇2,李薇3   

  1. 1. 四川大学华西医院
    2. 四川大学华西医院皮肤性病科
    3. 四川大学华西医院皮肤科
  • 收稿日期:2016-07-28 修回日期:2016-09-11 发布日期:2017-03-01
  • 通讯作者: 李薇 E-mail:liwei@mcwcums.com

Clinicopathological analysis of six cases of bullous systemic lupus erythematosus

  • Received:2016-07-28 Revised:2016-09-11 Published:2017-03-01

摘要: 目的 总结大疱性系统性红斑狼疮(BSLE)的临床特点、治疗及预后。方法 回顾性分析2009年5月至2015年9月诊治的6例BSLE患者临床资料。结果 男2例,女4例,平均发病年龄34岁。6例均表现为红斑或正常皮肤上出现张力性水疱、大疱,3例水疱、大疱排列呈环状。6例患者SLE疾病活动指数评分均大于4。皮损组织病理示表皮下水疱或裂隙;直接免疫荧光均表现为基底膜带线状IgG、IgM、IgA沉积,其中基底膜带线状C3沉积4例,线状C1q沉积2例。6例均予糖皮质激素、羟氯喹治疗;2例加用环磷酰胺。随访6例均发生程度不一的不良反应。结论 BSLE多见于中青年,皮损组织病理示表皮下水疱或裂隙,直接免疫荧光见基底膜带线状IgG、IgM、IgA或C3沉积。

Abstract: Wang Lian, Li Mengmeng, Xu Fei, Yan Wei, Li Wei Department of Dermatology and Venereology, West China Hospital, Sichuan University, Chengdu 610041, China Corresponding author: Li Wei, Email: liwei@mcwcums.com 【Abstract】 Objective To summarize the clinical features, treatment and prognosis of bullous systemic lupus erythematosus (BSLE). Methods Clinical data were collected from 6 cases of BSLE between May 2009 and September 2015, and were analyzed retrospectively. Results Among the 6 patients, 2 were male and 4 were female. The mean age of onset was 34 years. All the 6 patients presented with tense blisters and bullae arising on an erythematous base or normal skin, which were arranged in an annular pattern in 3 patients. The SLE Disease Activity Index (SLEDAI) score was > 4 in all the 6 patients. Histopathological examination showed subepidermal blisters or fissures in all the patients. Direct immunofluorescence (DIF) revealed continuous linear deposition of immunoglobulin G (IgG), IgM and IgA in all the patients, linear deposition of C3 in 4 patients, and linear deposition of C1q in 2 patients at the basement membrane zone (BMZ). All the 6 patients were treated with oral glucocorticoids and hydroxychloroquine, and 2 patients were additionally treated with cyclophosphamide. During the follow?up period, adverse reactions to different extents were observed in the 6 patients. Conclusions BSLE mainly occurs in young and middle?aged people. Histopathologically, subepidermal blisters or fissures can be observed with linear deposition of IgG, IgM, IgA, or C3 along the basement membrane zone on DIF.

引用本文

王莲 李萌萌 徐飞 闫薇 李薇. 大疱性系统性红斑狼疮六例临床病理分析[J]. 中华皮肤科杂志, 2017,50(3):182-185. doi: