伴口腔损害的副肿瘤性天疱疮9例临床分析  

Clinical analysis of 9 cases of paraneoplastic pemphigus with oral damage

作　　者：金建秋[1] 王倩 刘佳 童萍 张玉幸[1] Jin Jianqiu;Wang Qian;Liu Jia;Tong Ping;Zhang Yuxing(Department of Stomatology,Beijing Hospital,National Center of Gerontology,Institute of Geriatric Medicine,Chinese Academy of Medical Sciences,Beijing 100730,China)

机构地区：[1]北京医院口腔科,国家老年医学中心,中国医学科学院老年医学研究院,北京100730

出　　处：《中华口腔医学杂志》2025年第3期248-253,共6页Chinese Journal of Stomatology

基　　金：北京中医药大学教育科学研究课题(XJY24119);国家重点研发计划(2023YFC3605600);北京卫生健康委员会住院医师规范化培训质量提高项目(住培2023039)。

摘　　要：目的总结伴口腔损害的副肿瘤性天疱疮的临床特点、治疗方法和预后,为该病的临床诊治提供参考。方法对2017年9月至2023年12月于北京医院内科病房和口腔科门诊治疗的9例伴口腔损害的副肿瘤性天疱疮患者的病例资料和临床照片进行回顾性分析,总结和分析9例患者的临床特征。结果9例伴口腔损害的副肿瘤性天疱疮患者均有口腔黏膜和皮肤病损,经全身检查均发现内脏肿瘤,其中4例为卡斯尔曼病,4例为胸腺瘤,1例为肺癌。所有患者明确诊断前均曾使用糖皮质激素治疗,效果不明显。6例患者原发肿瘤切除后,继续使用糖皮质激素静脉滴注或口服治疗6~12个月,辅以免疫球蛋白和免疫抑制剂进行治疗,口腔黏膜糜烂明显好转,皮肤的病损缓解。无法切除原发肿瘤的3例患者继续之前的糖皮质激素治疗方案,均在1年内死亡,但治疗过程中对患者进行口腔局部治疗,仍有助于缓解患者口腔黏膜疼痛,促进糜烂愈合,改善营养状况。结论口腔黏膜多处糜烂和多形性皮肤损害是副肿瘤性天疱疮常见的临床表现;卡斯尔曼病和胸腺瘤是常见的伴发肿瘤;早期诊断和及时手术切除肿瘤对全身治疗和预后非常重要,切除术后应用糖皮质激素和口腔局部治疗有效。ObjectiveSummarize the clinical characteristics,treatment methods,and prognosis of paraneoplastic pemphigus with oral lesions,and identify commonalities and features to provide better references for the clinical diagnosis and treatment of this disease.MethodsA retrospective analysis was conducted on the case data and clinical photos of 9 patients with paraneoplastic pemphigus accompanied by oral lesions treated in the Internal Medicine Ward and Department of Stomatology of Beijing Hospital from September 2017 to December 2023,and the clinical characteristics were summarized and analyzed.ResultsAll 9 patients had oral mucosal and skin lesions simultaneously,in whom visceral tumors were found upon systemic examinations,including 4 cases of Castleman′s disease,4 cases of thymoma,and 1 case of lung cancer.All patients had received glucocorticoid therapy before a clear diagnosis,but the effect was not significant.After removing the primary tumor,corticosteroid intravenous or oral therapies were usually continued,supplemented with immunoglobulin and immunosuppressants for treatment,which could achieve significant therapeutic effects.With primary tumor resection,6 patients continued to receive corticosteroid intravenous or oral treatment for 6 to 12 months,supplemented with immunoglobulin and immunosuppressants.Oral mucosal erosion improved significantly and skin lesions were relieved.Three patients who were unable to remove their primary tumor continued with the previous glucocorticoid treatment regimen and died within one year.However,local treatment of their oral cavity during the treatment process still helped alleviate oral mucosal pain,promote erosion healing,and improve nutritional status.ConclusionsMultiple erosions of the oral mucosa and polymorphic skin damages are common clinical manifestations of paraneoplastic pemphigus.Castleman′s disease and thymoma are common coexisting tumors.Early diagnosis and timely surgical resection of tumors are crucial for treatment and prognosis.The application of glucocorti

关 键 词：口腔黏膜 天疱疮 副肿瘤性天疱疮 卡斯尔曼病 胸腺瘤 糖皮质激素 

分 类 号：R739.5[医药卫生—肿瘤]