Otten J V, Hashimoto T, Hertl M, Payne A S, Sitaru C
Department of Dermatology, University of Freiburg, Hauptstrasse 7, D-79104 Freiburg, Germany.
Curr Mol Med. 2014 Jan;14(1):69-95. doi: 10.2174/15665240113136660079.
Blister formation in skin and mucous membranes results from a loss of cell-cell or cell-matrix adhesion and is a common outcome of pathological events in a variety of conditions, including autoimmune and genetic diseases, viral and bacterial infections, or injury by physical and chemical factors. Autoantibodies against structural components maintaining cell-cell and cell-matrix adhesion induce tissue damage in autoimmune blistering diseases. Detection of these autoantibodies either tissue-bound or circulating in serum is essential to diagnose the autoimmune nature of disease. Various immunofluorescence methods as well as molecular immunoassays, including enzyme-linked immunosorbent assay and immunoblotting, belong to the modern diagnostic algorithms for these disorders. There is still a considerable need to increase awareness of the rare autoimmune blistering diseases, which often show a severe, chronic-relapsing course, among physicians and the public. This review article describes the immunopathological features of autoimmune bullous diseases and the molecular immunoassays currently available for their diagnosis and monitoring.
皮肤和黏膜中水疱的形成是由于细胞间或细胞与基质黏附丧失所致,是多种疾病(包括自身免疫性疾病和遗传性疾病、病毒和细菌感染或物理和化学因素损伤)病理事件的常见结果。针对维持细胞间和细胞与基质黏附的结构成分的自身抗体,在自身免疫性水疱病中会导致组织损伤。检测这些组织结合或血清中循环的自身抗体对于诊断疾病的自身免疫性质至关重要。各种免疫荧光方法以及分子免疫测定法,包括酶联免疫吸附测定和免疫印迹,都属于这些疾病的现代诊断算法。医生和公众对罕见的自身免疫性水疱病的认识仍有很大提升空间,这类疾病通常呈现严重的慢性复发性病程。这篇综述文章描述了自身免疫性大疱病的免疫病理特征以及目前可用于其诊断和监测的分子免疫测定法。