Raap U, Völker B, Petering H, Hashimoto T, Zillikens D, Kapp A, Werfel T
Klinik und Poliklinik für Dermatologie und Venerologie der Medizinischen Hochschule Hannover.
Hautarzt. 2005 Jan;56(1):66-70. doi: 10.1007/s00105-004-0822-4.
IgA pemphigus is a rare autoimmune intraepidermal blistering disease characterized by in vivo bound, and circulating IgA autoantibodies that target cell surface components (cadherins) of epidermal keratinocytes. The average age of onset is approximately 50 years. Histological hallmarks of IgA pemphigus are epidermal acantholysis, subcorneal or intraepidermal pustulosis, and neutrophilic infiltration. The main clinical characteristics are erythematous skin lesions with vesiculopustules, erosions, crusts and desquamation, favoring the trunk, groins, auxiliaries, and proximal extremities. The drug of choice for treating IgA pemphigus is diaminodiphenylsulfone. In this case study, we describe a patient who suffered from IgA pemphigus and responded well to diaminodiphenylsulfone.
IgA天疱疮是一种罕见的自身免疫性表皮内水疱性疾病，其特征是体内结合及循环的IgA自身抗体靶向表皮角质形成细胞的细胞表面成分（钙黏蛋白）。平均发病年龄约为50岁。IgA天疱疮的组织学特征为表皮棘层松解、角层下或表皮内脓疱形成以及中性粒细胞浸润。主要临床特征为伴有水疱脓疱、糜烂、结痂和脱屑的红斑性皮肤损害，好发于躯干、腹股沟、腋窝及四肢近端。治疗IgA天疱疮的首选药物是二氨基二苯砜。在本病例研究中，我们描述了一名患有IgA天疱疮且对二氨基二苯砜反应良好的患者。
