大疱性系统性红斑狼疮六例临床病理分析

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.

Clinicopathological analysis of six cases of bullous systemic lupus erythematosus

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 Clq 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.