Clinicopathological analysis of six cases of bullous systemic lupus erythematosus / 中华皮肤科杂志

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 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.