以皮肤水疱和狼疮肾炎为首发症状的大疱性系统性红斑狼疮1例  

A Case of Bullous Systemic Lupus Erythematosus Presented Firstly with Skin Blisters and Lupus Nephritis

作　　者：莫玉兰 吴玮 黎兆军 李定 陈蕾 蔡艳霞 周顺婷 谢锦莹 MO Yulan;WU Wei;LI Zhaojun;LI Ding;CHEN Lei;CAI Yanxia;ZHOU Shunting;XIE Jinying(Department of Dermatology,the Affiliated Hospital of Guangdong Medical University,Zhanjiang 524000,China)

机构地区：[1]广东医科大学附属医院皮肤科,广东湛江524000

出　　处：《中国皮肤性病学杂志》2021年第7期780-784,共5页The Chinese Journal of Dermatovenereology

基　　金：广东医科大学附属医院博士科研启动经费(BK201604)。

摘　　要：患者女,53岁,颈项和躯干红斑、水疱25 d,泛发全身4 d。查体:全身对称分布散在红斑,上有紧张性水疱,呈环形排列,尼氏征阴性,小腿轻度凹陷性水肿。实验室检查:尿蛋白定量3178 mg/24 h,血清白蛋白18.1 g/L,总胆固醇8.11 mmol/L,抗核抗体(ANA)(+),抗双链DNA(dsDNA)抗体(2+),抗Sm抗体(+),抗小核核糖核蛋抗体(snRNP)(+)。皮损组织病理示:表皮下水疱,疱内和真皮浅层见大量中性粒细胞浸润。直接免疫荧光见基底膜IgG、IgA、C3线状沉积。盐裂直接免疫荧光:IgA、IgG和C3表皮侧和真皮侧线状沉积。肾脏病理提示狼疮性肾炎(V型)。最后确诊为:①狼疮性肾炎(V型);②大疱性系统性红斑狼疮。A 53-year-old female presented with erythematosus blisters on her neck and trunk for 25 days and spread all over the body for four days.Physical examination revealed scattered annular erythematous lesions with blisters,negative Nikolsky's sign and mild edema on her legs.Laboratory investigations showed proteinuria(3178 mg/24 h),hypoproteinemia(18.1 g/L)and increased levels of total cholesterol(8.11 mmol/L).Antinuclear antibodies(ANA),anti-dsDNA antibodies,anti-Sm antibodies,and antismall nuclear ribonucleoproteins(snRNP)antibodies were positive.Histopathological examination showed a subepidermal blister with prominent neutrophil infiltration in the papillary dermis.Direct immunofluorescence showed linear deposits of IgG,IgA,and C3 at the basement membrane zone,the dermis and the epidermis.Renal biopsy showed lupus nephritis(type V).Eventually,lupus nephritis(type V)and bullous systemic lupus erythematosus were diagnosed.

关 键 词：环形水疱 狼疮性肾炎 大疱性系统性红斑狼疮 

分 类 号：R593.24[医药卫生—内科学]