Rodot S, Botcazou V, Lacour J P, Dor J F, Bodokh I, Joly P, Ortonne J P
Service de dermatologie, hôpital Pasteur, Nice, France.
Rev Med Interne. 1995;16(12):938-43. doi: 10.1016/0248-8663(96)80818-6.
In 1990, Anhalt et al described a newly autoimmune bullous disease: paraneoplastic pemphigus, in five patients. It was characterized by a distinct set of circulating autoantibodies from those in the sera of patients with pemphigus vulgaris and superficial pemphigus. We report a 71 year-old man with chronic lymphocytic leukemia of 4 years duration who developed a severe mucocutaneous eruption with clinical and immunofluorescence findings of pemphigus vulgaris evolving into an oral bullous lichen planus presentation. Evaluation of his serum confirmed the presence of autoantibodies specific for paraneoplastic pemphigus by indirect immunofluorescence on rat-bladder and immunoprecipitation. Subsequently, additional cases have been reported in the literature. All occurred in patients with various neoplastic conditions. These patients present with polymorphous skin lesions and severe erosive oral disease. Histologic examination shows interface dermatitis and keratinocyte necrosis in addition to acantolysis. Direct immunofluorescence may reveal deposition of immunoglobulin and/or complement at the basement membrane as well as deposition on epithelial cell surfaces. Circulating IgG anti-cell-surface antibodies are detectable with both stratified and stratified epithelia as substrates. These antibodies immunoprecipitate a complex of four desmosomal proteins, including desmoplakin I (250 kDa), the bullous pemphigoid antigen (230 kDa), desmoplakin II (210 kDa) and a 190 kDa antigen.
1990年，安哈尔特等人描述了一种新的自身免疫性大疱性疾病：副肿瘤性天疱疮，涉及5例患者。其特征是一组与寻常型天疱疮和浅表性天疱疮患者血清中不同的循环自身抗体。我们报告了一名71岁男性，患有4年慢性淋巴细胞白血病，出现严重的黏膜皮肤疹，临床和免疫荧光检查结果显示寻常型天疱疮演变为口腔大疱性扁平苔藓表现。对其血清的评估通过大鼠膀胱间接免疫荧光和免疫沉淀证实了副肿瘤性天疱疮特异性自身抗体的存在。随后，文献中又报道了其他病例。所有病例均发生在患有各种肿瘤性疾病的患者中。这些患者表现为多形性皮肤病变和严重的糜烂性口腔疾病。组织学检查除棘层松解外，还显示界面性皮炎和角质形成细胞坏死。直接免疫荧光可能显示免疫球蛋白和/或补体在基底膜以及上皮细胞表面的沉积。以分层和非分层上皮为底物均可检测到循环IgG抗细胞表面抗体。这些抗体免疫沉淀出四种桥粒蛋白的复合物，包括桥粒斑蛋白I（250 kDa）、大疱性类天疱疮抗原（230 kDa）、桥粒斑蛋白II（210 kDa）和一种190 kDa抗原。
