Department of Dermatology, Kurume University School of Medicine, and Kurume University Institute of Cutaneous Cell Biology, 67 Asahimachi, Kurume, Fukuoka 830-0011, Japan.
Clin Dermatol. 2011 Jul-Aug;29(4):437-42. doi: 10.1016/j.clindermatol.2011.01.014.
Pemphigus 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.
天疱疮是一种危及生命的自身免疫性水疱病。天疱疮分为 4 种主要类型；寻常型天疱疮、落叶型天疱疮、副肿瘤性天疱疮和 IgA 天疱疮。其中，IgA 天疱疮的特征是组织结合和循环 IgA 抗体针对表皮中的桥粒或非桥粒细胞表面成分。组织病理学观察到轻微的表皮棘层松解和广泛的中性粒细胞浸润在上部或表皮的所有层中。IgA 天疱疮分为棘层内中性粒细胞 IgA 皮肤病型（IEN 型），其靶抗原仍不清楚（可能是非桥粒细胞表面蛋白）和角层下脓疱性皮病型（SPD 型），其靶抗原是桥粒芯糖蛋白 1（Dsc1）。我们总结了报道的 IgA 天疱疮病例，并描述了包括流行病学、临床表现、病理学、实验室检查、病理生理学、相关疾病、预后和治疗以及 IgA 天疱疮的未来展望在内的当前知识。
