Zillikens D, Bröcker E B
Universitäts-Hautklinik, Würzburg.
Hautarzt. 1994 Dec;45(12):827-33. doi: 10.1007/s001050050181.
Paraneoplastic pemphigus (PP) is a newly reported autoimmune disease, which is always associated with an underlying neoplasia. It is characterized by painful mucosal erosions and ulcerations, and by polymorphous skin lesions reminiscent of erythema multiforme, pemphigus vulgaris and lichen planus pemphigoides. These patients have autoantibodies that bind to a distinct complex of epidermal proteins, including desmoplakin I (250 kD), major bullous pemphigoid antigen (230 kD), desmoplakin II (210 kD) and a 190-kD and a 170-kD protein, neither of which has yet been further characterized. Histological findings include acantholysis, epidermal cell necrosis, vacuolar interface changes and, sometimes, lichenoid infiltrates of the upper dermis. Direct immunofluorescence shows an intercellular deposition of immunoreactants in the epidermis and granular deposits at the dermal-epidermal junction. Indirect immunofluorescence with rodent bladder as substrate shows an intercellular pattern. This method is the most convenient and cost-effective method of screening for PP, since antigens of both pemphigus vulgaris and pemphigus foliaceus are not expressed in this tissue.
副肿瘤性天疱疮（PP）是一种新报道的自身免疫性疾病，常与潜在的肿瘤相关。其特征为疼痛性黏膜糜烂和溃疡，以及类似多形红斑、寻常型天疱疮和类天疱疮样扁平苔藓的多形性皮肤损害。这些患者具有与一种独特的表皮蛋白复合物结合的自身抗体，该复合物包括桥粒斑蛋白I（250kD）、大疱性类天疱疮主要抗原（230kD）、桥粒斑蛋白II（210kD）以及一种190kD和一种170kD的蛋白，这两种蛋白尚未进一步明确其特征。组织学表现包括棘层松解、表皮细胞坏死、空泡状界面改变，有时还包括真皮上部的苔藓样浸润。直接免疫荧光显示表皮中有免疫反应物的细胞间沉积以及真皮-表皮交界处的颗粒状沉积。以鼠膀胱为底物的间接免疫荧光显示细胞间模式。这种方法是筛查PP最便捷且经济有效的方法，因为寻常型天疱疮和落叶型天疱疮的抗原在该组织中均不表达。
