文献简介

出版社:Elsevier

作  者:Daisuke Tsuruta, MD, PhD, Norito Ishii, MD, PhD

编  号:10.1016/j.clindermatol.2011.01.014

关键字:IgA pemphigus

年  份:2011   点击量:770

文献摘要

AbstractPemphigus is a life-threatening autoimmune blistering disease. Pemphigus is divided into 4 major types; pemphigus vulgaris, pemphigus foliaceus, paraneoplastic pemphigus, and IgA pemphigus. Among them, IgA pemphigus is characterized by tissue-bound and circulating IgA antibodies targeting desmosomal or nondesmosomal cell surface components in the epidermis. Histopathologically, slight epidermal acantholysis and extensive neutrophilic infiltration in either the upper part or all layers of the epidermis were observed. IgA pemphigus is subdivided into intraepidermal neutrophilic IgA dermatosis type (IEN-type), whose target antigen is still unknown (probably nondesmosomal cell surface protein), and subcorneal pustular dermatosis-type (SPD-type), whose target antigen is desmocollin 1 (Dsc1). We summarize reported cases of IgA pemphigus and describe current knowledge including epidemiology, clinical manifestations, pathology, laboratory tests, pathophysiology, associated diseases, prognosis and treatment, and future perspectives of IgA pemphigus.

摘要

天疱疮是一种危及生命的自身免疫性起泡病。天疱疮分为4大类型:寻常型天疱疮,天疱疮,副肿瘤性天疱疮和IgA型天疱疮。他们中,IgA型天疱疮的特征是,针对表皮中表面成分的桥粒细胞或非桥粒细胞的组织结合和循环IgA抗体。组织病理检查,观察到在表皮层无论是上半部分或所有图层有轻微表皮细胞棘层和广泛的中性粒细胞浸润。。IgA天疱疮被细分为表皮内嗜中性粒细胞的IgA皮肤病型(IEN-type),其靶抗原仍是未知(可能为非桥粒细胞表面蛋白),和角层下脓疱性皮病型(SPD-型),其靶抗原是桥粒胶蛋白1Dsc1)。我们总结报道IgA型天疱疮病例,并描述目前的知识,包括流行病学,临床表现,病理,实验室检查,病理生理学,相关的疾病,预后和治疗,和IgA型天疱疮的未来前景。