儿童大疱性系统性红斑狼疮1例

Bullous Systemic Lupus Erythematosus in Children:A Case Report

患者女,11岁,周身红斑、水疱2周,伴发热1周。实验室检查:抗核抗体、抗双链DNA、抗Sm抗体阳性,免疫蛋白升高,补体下降,胸部CT提示双肺炎症及心包积液。右上臂内侧皮肤组织病理:以中性粒细胞为主的表皮下水疱性疾病;直接免疫荧光:表皮基底膜带C3线状沉积。诊断:儿童大疱性系统性红斑狼疮。予以糖皮质激素治疗联合硫酸羟氯喹治疗,2周后皮疹控制稳定。

An 11-year-old female was admitted to hospital with erythema and blisters for 2 weeks and fever for 1 week.Laboratory examination revealed positive anti-nuclear antibodies,double-stranded DNA and anti-Sm antibodies,increased immune proteins,and decreased complement.Chest CT showed double pneumonia and pericardial effusion.Histopathology of the inner skin of the right upper arm showed subepidermal vesicular disease dominated by neutrophils.Direct immunofluorescence showed C3 linear deposition in the epidermal basement membrane.The diagnosis of bullous systemic lupus erythematosus in children was confirmed.Glucocorticoid therapy combined with hydroxychloroquine sulfate for symptom control in 2 weeks.