Zillikens Detlef
Department of Dermatology, University of Lübeck, Germany.
Clin Lab. 2008;54(11-12):491-503.
Autoimmune blistering skin diseases are characterized by autoantibodies to structural components of the skin. Diagnosis is based on the detection of autoantibodies in the skin and/or serum of the patients. Tissue-bound autoantibodies are found by direct immunofluorescence microscopy of tissue-bound autoantibodies and are still considered the gold standard for the diagnosis of autoimmune bullous diseases. Circulating antibodies are first characterized by indirect immunofluorescence microscopy on organ sections including monkey esophagus and 1M NaCl-split human skin. Specificities of these circulating autoantibodies are then analyzed by immunoblotting, ELISA, or immunoprecipitation. More recently, ELISA-based detection systems for some of these autoantibodies have become commercially available. Frequently, the levels of circulating autoantibodies as detected by ELISA correlate with disease activity and facilitate the evaluation for further need of treatment. In cases where the clinical picture strongly suggests an autoimmune bullous disease, diagnosis may be based only on serological tests. This review summarizes the diagnostic steps in the different autoimmune bullous skin diseases.
自身免疫性水疱性皮肤病的特征是针对皮肤结构成分产生自身抗体。诊断基于检测患者皮肤和/或血清中的自身抗体。通过对组织结合自身抗体进行直接免疫荧光显微镜检查来发现组织结合自身抗体，其仍被视为自身免疫性大疱性疾病诊断的金标准。循环抗体首先通过对包括猴食管和1M NaCl分离的人皮肤在内的器官切片进行间接免疫荧光显微镜检查来鉴定。然后通过免疫印迹、酶联免疫吸附测定（ELISA）或免疫沉淀分析这些循环自身抗体的特异性。最近，基于ELISA的某些自身抗体检测系统已商业化。通常，ELISA检测到的循环自身抗体水平与疾病活动相关，并有助于评估是否需要进一步治疗。在临床表现强烈提示自身免疫性大疱性疾病的情况下，诊断可能仅基于血清学检查。本综述总结了不同自身免疫性大疱性皮肤病的诊断步骤。
