误诊为线状IgA大疱性皮病的大疱性系统性红斑狼疮1例  

A Case of Bulloussystemic Lupus Erythematosus Misdiagnosed as Linear Ig A Bullous Dermatosis

作　　者：王小云 尹光文[1] 张华[1] 

机构地区：[1]郑州大学第一附属医院皮肤科,河南郑州450052

出　　处：《中国皮肤性病学杂志》2018年第2期200-202,共3页The Chinese Journal of Dermatovenereology

摘　　要：患者男,20岁,面部、胸前皮肤出现孤立或半环状排列的水疱7天,曾诊断为线状IgA大疱性皮病(linear IgA bullous dermatosis,LABD),后经实验室检查患者外周血白细胞2.50×10~9/L,血小板65×109/L,ANA 1∶640,抗dsDNA抗体阳性,尿蛋白(3+)。组织病理示:表皮下水疱形成,真皮浅层及血管周可见淋巴细胞、中性粒细胞浸润,免疫荧光(direct immunofluorescence,DIF)基底膜带有IgG,IgA和C_3均呈线状沉积,确诊为大疱性系统性红斑狼疮(bullous systemic lupus erythematosus,BSLE)。予甲泼尼龙、羟氯喹等治疗7天,皮损全部消退,随访未见新发水疱。A 20-year-old man presented with isolated or semi-circular formed blisters on his face and anterior thorax for 7 days.He had been diagnosed as IgA bullousdermatosis.Laboratory analysis revealed white blood test（WBC）2.50109/L,platelet 65109/L,ANA 1：640,ds-DNA（＋）,urineprotein（3＋）.The histopathological examination showed subepidermal bullae,with prominent infiltration of neutrophil and lymphocytes in the upper dermis and perivascular locations.DIF revealed linear depostion of IgA,IgG and C3in the basement zone membrane.A diagnosis of bullous systemic lupus eyrthematosuts was finally established.The skin rashes resolved dramatically with the treatment of methylprednisolone and hydroxychloroquine for one week,and no new blisters were followed.

关 键 词：大疱性 红斑狼疮 系统性 大疱性皮病 线状IgA 

分 类 号：R593.241[医药卫生—内科学]