Department of Dermatology and Venereology, University Hospital Center Zagreb, School of Medicine, University of Zagreb, Salata 4, 10000 Zagreb, Croatia.
Clin Dermatol. 2011 Jul-Aug;29(4):355-9. doi: 10.1016/j.clindermatol.2011.01.005.
In the not so distant past, the word pemphigus or pemphix was common for describing various diseases characterized by blistering as well as various disorders that do not originate from a blistering pathology. Patients with these conditions were grouped in "other" skin diseases. Step by step, during the past, we were introduced to these severe conditions. First, we learned from sporadic case reports, then new differentiations were reported according to histology, later immunopathology was developed, and now there are discoveries of new molecules. Immense progress with new approaches to therapy has been achieved, but much improvement is still needed. The modern definition of pemphigus undoubtedly represents a group of rare, intraepidermal autoimmune bullous diseases characterized by intraepidermal blisters and circulating autoantibodies desmogleins against the keratinocytes cell surface.
在不久的过去,“天疱疮”或“类天疱疮”这两个词是用来描述各种以水疱为特征的疾病以及各种并非源于水疱性病理的紊乱。这些病症的患者被归入“其他”皮肤病。在过去,我们一步一步地认识到了这些严重的病症。首先,我们从零星的病例报告中了解到这些病症,然后根据组织学进行了新的区分,后来发展了免疫病理学,现在又有了新分子的发现。通过新的治疗方法取得了巨大的进展,但仍有很大的改进空间。天疱疮的现代定义无疑代表了一组罕见的、表皮内自身免疫性大疱性疾病,其特征是表皮内水疱和循环自身抗体桥粒芯糖蛋白针对角朊细胞细胞表面。