自身免疫性大疱性皮肤病的实验诊断

Laboratory diagnosis of autoimmune blistering skin diseases

自身免疫性大疱性皮肤病是一组器官特异性自身免疫性疾病，患者血清中有针对皮肤连接结构桥粒、半桥粒的自身抗体，破坏皮肤连接而形成以皮肤及黏膜水疱、大疱为主要特征的疾病。不同的自身抗体导致水疱发生的位置不同，可分为表皮内及表皮下大疱性皮肤病两大类，这两类疾病所采取的治疗方案不同、预后差别较大。根据临床表现及组织病理尚不足以明确自身免疫性大疱性皮肤病的诊断，结合直接免疫荧光、间接免疫荧光、ELISA、免疫印迹及免疫沉淀等免疫学检测技术能够更准确地诊断。同时，对血清的检测还可用于评估病情的活动度并用于指导治疗。

Autoimmune blistering skin diseases are a group of organ-specific autoimmune disorders that are characterized by autoantibodies against desmosome and hemidesmosome which are structural proteins of the epidermis or the dermal-epidermal junction and clinically by blisters and erosions on skin and/or mucous membranes. According to the skin level at which the blister occurs and the structural proteins that the autoantibodies target, autoimmune blistering diseases can be categorized into intraepithelial blister group and subepidermal blister group. The treatment options and prognosis are different among the various diseases. Since clinical criteria and histopathological characteristics are not sufficient for an accurate diagnosis of autoimmune blistering skin diseases, direct immunofluorescence microscopy, indirect immunofluorescence microscopy, ELISA, immunoblotting and immunoprecipitation are needed for exact diagnosis. The detection of serum autoantibodies have been shown to correlate with disease activity and thus may be helpful in deciding treatment options for the patients.