线状IgA大疱性皮病的研究进展  

Research progress on linear IgA bullous dermatosis

作　　者：吴媚 李德良[1] 司徒欣欣[1] 李国丽 尹光文[2] WU Mei;LI Deliang;SITU Xinxin;LI Guoli;YIN Guangwen(Xinhui People's Hospital of Jiangmen,Jiangmen 529100,China;First Affiliated Hospital of Zhengzhou University,Zhengzhou 450052,China)

机构地区：[1]江门市新会区人民医院,广东江门529100 [2]郑州大学第一附属医院,河南郑州450052

出　　处：《皮肤性病诊疗学杂志》2023年第6期574-578,共5页Journal of Diagnosis and Therapy on Dermato-venereology

摘　　要：线状IgA大疱性皮病(LABD)是一种罕见的自身免疫性表皮下大疱性皮肤病,特征性皮损表现为红斑上覆“珍珠串”样环状水疱。其发病可能与免疫系统失调和遗传因素有关,发病机制涉及IgA抗体在皮肤基底膜区域的异常沉积和异常免疫反应的发生。LABD组织病理特征不典型,直接免疫荧光表现为皮肤组织中的IgA抗体线状沉积于基底膜带。分子生物学和免疫学的进展为提高该病诊断的精准性提供了新的可能。个体化治疗成为该病的治疗关键,轻度疾病者可采用局部激素和抗组胺药物,而严重者可能需要系统性免疫抑制剂,甚至生物制剂。本综述旨在总结LABD的病因、发病机制、诊断和治疗策略等方面的最新研究,并展望未来的研究方向。Linear IgA bullous dermatosis(LABD)is a rare autoimmune subepidermal blistering skin disease,manifested by erythematous plaques covered by circular blisters in a pattern of“string of pearls”.Immune dysregulation and genetic factors are implicated in the onset of LABD.The pathogenesis includes abnormal deposition of IgA antibodies in the basement membrane zone and abnormal immune response.Although the pathological characteristics are atypical,direct immunofluorescence shows deposition of linear IgA antibody at the basement membrane zone.Advances in molecular biology and immunology offer new possibilities for enhancing precise diagnosis.Personalized treatment has become pivotal,while topical steroids and antihistamines are suitable for mild cases.Severe cases may require systemic immunosuppressants or even biologics.This review aims to summarize the latest research advances in LABD,including epidemiological features,pathogenesis,diagnostic and therapeutic strategies,and to provide an outlook on future research directions.

关 键 词：线状IGA大疱性皮病 IGA抗体 免疫抑制剂 

分 类 号：R758.66[医药卫生—皮肤病学与性病学]