中国麻风皮肤病杂志 ›› 2018, Vol. 34 ›› Issue (7): 427-429.

• 经典病例研究 • 上一篇    下一篇

大疱性系统性红斑狼疮合并狼疮肾炎一例

陶 荣 耿 研 冉梦龙 王 昱 涂平 张卓莉   

  1. 北京大学第一医院，北京，100034
  • 出版日期:2018-07-15 发布日期:2018-12-14
  • 通讯作者: 张卓莉，E-mail：zhuoli.zhang@126.com；涂平，E-mail:tup0207@sina.com

Bullous systemic lupus erythematosus with lupus nephritis: a case report

TAO Rong,GENG Yan, RAN Menglong, WANG Yu,TU Ping,ZHANG Zhuoli   

  1. Peking University First Hospital，Bejing 100034,China
  • Online:2018-07-15 Published:2018-12-14
  • Contact: ZHANG Zhuoli，E-mail：zhuoli.zhang@126.com；TU Ping，E-mail:tup0207@sina.com

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摘要： 患者，女性，29岁。主因脱发2年，水肿伴疱疹1个月余就诊。血常规正常，尿常规RBC 6-10个/HP，24小时尿蛋白定量4.29 g,血白蛋白19.9 g/L。自身抗体ANA1:10000（S），抗ds-DNA(+),抗Sm（+），抗nRNP(+)。IgG 21.8g/L,补体C3：0.292 g/L,C4:0.037 g/L。确诊为系统性红斑狼疮伴狼疮肾炎，皮损组织病理检查：表皮下水疱形成，真皮乳头明显嗜中性细胞浸润，真皮中层血管周围灶状淋巴细胞浸润。免疫组化：皮肤直接免疫荧光法见基底膜带IgG阳性。诊断：大疱性系统性红斑狼。予甲强龙1 mg/Kg，联合环磷酰胺及硫酸羟氯喹治疗1周，患者狼疮性肾炎好转，但疱疹仍加重。予甲强龙冲击250mg×3天，加用盐酸米诺环素后好转。

关键词: 大疱性系统性红斑狼疮, 狼疮性肾炎

Abstract: A 29-year-old female presented with hair loss for two years, and edema with rash for more than one month. Blood routine examination is normal. Urine routine test showed that RBC was 6-10/HP; 24 hours urinary protein was 4.29g; quantitative blood albumin was 19.9 g/L. ?Antibody?detection?showed ANA1:10000 (S), anti-dsDNA(+), anti-Sm (+), anti-nRNP (+), IgG 21.8 g/L, C3:0.292 g/L, C4:0.037 g/L. Histopathological examination showed subepidermal blister, neutrophil cell infiltration in dermal papilla, lymphocytic infiltrates in the perivascular of middle level dermis. Direct immunohistochemistry revealed that IgG was positive in the basement membrane zone. The diagnosis of lupus?nephritis and bullous systemic lupus erythematosus was made. After the patient was treated with methylprednisolone, 1mg/kg, once a day combined with cyclophosphamide and hydroxychloroquine for 1 week, the lupus nephritis has improved, but herpes got worse. Then the patient was given methylprednisolone impulse therapy (250mg for 3 days) and minocycline hydrochloride. The lesions were improved.

Key words: bullous systemic lupus erythematosus, lupus nephritis