仅表现口腔粘膜糜烂的副肿瘤性天疱疮临床及免疫学研究

Clinical and immunological characteristics of paraneoplastic pemphigus manifested only with erosion of oral mucous membrane

目的 : 报告 1例仅表现为口腔粘膜糜烂的副肿瘤性天疱疮患者的临床及免疫学特征。方法 :对患者进行系统及皮肤科、口腔科临床学、医学影像学、组织病理学和免疫学检查。结果 : 通过上述检查 ,结果符合副肿瘤天疱疮 ,并发现患者合并胸部castleman’s病 ,患者存在抗 2 10kDa(Envoplakin)及 190kDa(Periplakin)的抗体。结论 : 临床上遇有仅表现口腔粘膜顽固性糜烂溃疡的患者 ,要考虑到PNP的可能。

Objective: To characterize the clinical profiles, medical imaging and imunological tests in a patient with paraneoplastic pemphigus (PNP) manifested only with oral erosion.Methods: Systemic physical examination including skin and mucous membrane, medical imaging and immunological tests were performed for a patient with paraneoplastic pemphigus manifested only with oral erosion. Results: The results of the systemic examination confirmed the diagnosis of paraneoplastic pemphigus and a Castleman's tumor was found in chest cavity. 210 kda (Envoplakin) and 190 kda (Periplakin) antibodies were detected in the serum. Conclusion : PNP should be considered clinically when a patient only presents with reluctant oral mucous membrane erotion or ulceration. Detection of potential tumor and surgical intervention are the most effective method in the treatment of PNP.