Thivolet J
Department of Dermatology, Hôpital E.-Herriot, Lyon, France.
Dermatology. 1994;189 Suppl 2:26-9. doi: 10.1159/000246985.
Pemphigus remains the archetype of the autoimmune bullous diseases with circulating and skin antibodies in patients as well as in animal models. Pemphigus antigens are desmosomal-associated glycoproteins of 130 kD in pemphigus vulgaris (desmoglein 3) and of 160 kD (desmoglein 1) in pemphigus foliaceus (fogo selvagem in Brazil, pemphigus seborrheicus in other countries). Pemphigus may be associated to drugs and/or immune system related tumors (e.g. thymoma). Two new forms of pemphigus have been described: IgA pemphigus and paraneoplastic pemphigus. Targets of the autoantibodies are various desmosomal and cellular adhesion molecules. Even if each main pemphigus variant corresponds to specific antibodies, association of various antibodies may be found, leading to the concept of dysimmunoreactivity.
天疱疮仍是自身免疫性大疱性疾病的原型，在患者以及动物模型中均存在循环抗体和皮肤抗体。寻常型天疱疮的天疱疮抗原是一种130kD的桥粒相关糖蛋白（桥粒芯糖蛋白3），落叶型天疱疮（巴西的丛林大疱疮、其他国家的脂溢性天疱疮）的天疱疮抗原是一种160kD的桥粒相关糖蛋白（桥粒芯糖蛋白1）。天疱疮可能与药物和/或免疫系统相关肿瘤（如胸腺瘤）有关。已描述了两种新的天疱疮形式：IgA天疱疮和副肿瘤性天疱疮。自身抗体的靶标是各种桥粒和细胞粘附分子。即使每种主要的天疱疮变体对应于特定抗体，但也可能发现多种抗体的关联，从而产生了免疫反应异常的概念。
