线状IgA大疱性皮病研究进展

Research advance in linear IgA bullous dermatosis

线状IgA大疱性皮病是一种以基底膜带存在连续性IgA抗体沉积为特点的罕见的自身免疫性大疱病,可能与遗传、药物、炎症性疾病、肿瘤等有关。线状IgA大疱性皮病的诊断依据临床表现、常规病理和免疫荧光。该病首选的治疗方案是口服氨苯砜,近年来也有生物制剂治疗该病的报道。本文从流行病学、病因、发病机制、临床表现、实验室检查、诊断及治疗等方面对线状IgA大疱性皮病进行总结。

Linear IgA bullous dermatosis(LABD)is an autoimmune bullous diseases characterized by continuous deposition of IgA autoantibody in the basement membrane zone(BMZ).It may be related to genetics,drugs,inflammatory diseases and tumor.The diagnosis of LABD should be confirmed by clinical manifestation,histopathological and immunofluorescence examination.Dapsone is the first line therapy for LABD.Biological agents have also been reported to treat the disease in recent years.This review summarizes epidemiological features,pathogenesis,clinical manifestations,laboratory examination,diagnosis and treatment of LABD.