1例伴发恶性胸腺瘤的副肿瘤性天疱疮临床及免疫学研究

目的报告我国首例伴发恶性胸腺瘤的副肿瘤性天疱疮(PNP)临床及免疫学特征。方法对患者进行系统体检、医学影像学、组织病理学和免疫学检查,使用重组蛋白免疫印迹法对由胸腺瘤分泌抗体所针对的自身抗原进行了研究。结果患者确诊为PNP,伴发恶性胸腺瘤,患者血清中含有抗重组人包斑蛋白(envoplakin)和周斑蛋白(periplakin)L亚区GST融合蛋白的抗体。结论PNP具有独特的临床表现、组织病理以及免疫学特点,患者血清中的自身抗体针对多种抗原,包斑蛋白和周斑蛋白的L亚区很可能是PNP患者血清抗体结合的主要位点。

Clinical and Immunological Study of One Case Paraneoplastic Pemphigus Complicated with Thymoma

Objective To characterize the clinical profiles and immunological findings in the first case of paraneoplastic pemphigus (PNP) complicated with thymoma in China.Methods Systemic physical examination,medical imaging,histopathological and immunological tests were performed.Immunoblotting was performed by using recombinant glutathione S transferase fusion (GST fusion) proteins to investigate the antigen epitope of PNP.Results The results of the examination confirmed the diagnosis of PNP complicated with thymoma.Antibodies against the recombinant GST fusion proteins of the L subdomain of envoplakin and periplakin were detected in the serum.Conclusion PNP is a clinically, histologically, and immunologically distinct autoimmune bullous disease.PNP patients developed characteristic autoantibodies against multiple antigens.We could speculate that PNP autoantibodies react mainly with the L subdomain of envoplakin and periplakin.