中华皮肤科杂志 ›› 2025, Vol. 58 ›› Issue (4): 289-296.doi: 10.35541/cjd.20240569

• 指南与共识·急危重皮肤病 • 上一篇    下一篇

【开放获取】   副肿瘤性天疱疮诊疗中国专家共识(2025版)

中华医学会皮肤性病学分会    中国医师协会皮肤科医师分会    中国医疗保健国际交流促进会皮肤医学分会    中国罕见病联盟皮肤罕见病专业委员会    国家皮肤与免疫疾病临床医学研究中心   

  1. 中华医学会皮肤性病学分会    中国医师协会皮肤科医师分会    中国医疗保健国际交流促进会皮肤医学分会    中国罕见病联盟皮肤罕见病专业委员会    国家皮肤与免疫疾病临床医学研究中心
  • 收稿日期:2024-10-23 修回日期:2025-02-10 发布日期:2025-04-03
  • 通讯作者: 晋红中;王刚 E-mail:jinhongzhong@263.net; wanggangxjyy@163.com
  • 基金资助:
    中央高水平医院临床科研业务费(2022-PUMCH-B-092);国家重点研发计划(2022YFC3601800);国家临床重点专科建设项目;北京市自然科学基金(7242109);北京市临床重点专科建设项目

Expert consensus on the diagnosis and treatment of paraneoplastic pemphigus in China (2025 version)

Chinese Society of Dermatology; China Dermatologist Association; Dermatology Branch of China International Exchange and Promotive Association for Medical and Health Care; Rare Skin Diseases Committee, China Alliance for Rare Diseases; National Clinical Research Center for Dermatologic and Immunologic Diseases   

  1. Chinese Society of Dermatology; China Dermatologist Association; Dermatology Branch of China International Exchange and Promotive Association for Medical and Health Care; Rare Skin Diseases Committee, China Alliance for Rare Diseases; National Clinical Research Center for Dermatologic and Immunologic Diseases
  • Received:2024-10-23 Revised:2025-02-10 Published:2025-04-03
  • Contact: Jin Hongzhong; Wang Gang E-mail:jinhongzhong@263.net; wanggangxjyy@163.com
  • Supported by:
    National High Level Hospital Clinical Research Funding (2022-PUMCH-B-092); National Key R&D Program of China (2022YFC3601800); National Key Clinical Specialty Project of China; Beijing Natural Science Foundation (7242109); Key Clinical Specialty Project of Beijing

摘要: 【摘要】 天疱疮是一种自身免疫性大疱性疾病,副肿瘤性天疱疮(PNP)是天疱疮的特殊亚型。PNP临床特点主要包括严重而顽固的疼痛性黏膜糜烂和溃疡、多形性皮损、合并肿瘤,常伴有其他系统损害(如闭塞性细支气管炎),多数患者对治疗反应差,严重者危及生命。PNP的诊疗涉及皮肤科、口腔科、肿瘤科、血液科、呼吸科、外科、麻醉科、全科医学科和重症医学科等多个科室。相关学术组织召集相关领域专家制订本共识,基于国内PNP的诊疗现状拟定了PNP诊断的中国标准,并针对皮肤黏膜损害、肿瘤、闭塞性细支气管炎以及并发症等方面提出了治疗建议,为PNP的诊断和治疗提供指导。

关键词: 天疱疮, 副肿瘤性天疱疮, 肿瘤, 闭塞性细支气管炎, 罕见病, 诊断, 治疗, 专家共识

Abstract: 【Abstract】 Pemphigus is an autoimmune bullous disease, and paraneoplastic pemphigus (PNP) is a unique subtype of pemphigus. Clinical characteristics of PNP mainly include severe and refractory painful erosions and ulcers of the mucosa, polymorphous skin lesions, and comorbid neoplasms, often accompanied by other systemic involvement (such as bronchiolitis obliterans). Most patients respond poorly to treatment, and in severe cases, it is life-threatening. The diagnosis and treatment of PNP involves dermatology, stomatology, oncology, hematology, respiratory medicine, surgery, anesthesiology, general internal medicine, intensive care unit and other departments. Relevant academic organizations enrolled experts in related fields together to formulate this consensus, to provide guidance for the diagnosis and treatment of PNP. Chinese standards for PNP diagnosis are proposed based on the status of diagnosis and treatment of PNP in China. Treatment suggestions are put forward from the aspects of skin and mucosal involvement, neoplasms, bronchiolitis obliterans, and complications.

Key words: Pemphigus, Paraneoplastic pemphigus, Neoplasms, Bronchiolitis obliterans, Rare disease, Diagnosis, Treatment, Expert consensus

引用本文

中华医学会皮肤性病学分会 中国医师协会皮肤科医师分会 中国医疗保健国际交流促进会皮肤医学分会 中国罕见病联盟皮肤罕见病专业委员会 国家皮肤与免疫疾病临床医学研究中心. 【开放获取】   副肿瘤性天疱疮诊疗中国专家共识(2025版)[J]. 中华皮肤科杂志, 2025,58(4):289-296. doi:10.35541/cjd.20240569

Chinese Society of Dermatology, China Dermatologist Association, Dermatology Branch of China International Exchange and Promotive Association for Medical and Health Care, Rare Skin Diseases Committee, China Alliance for Rare Diseases, National Clinical Research Center for Dermatologic and Immunologic Diseases. Expert consensus on the diagnosis and treatment of paraneoplastic pemphigus in China (2025 version)[J]. Chinese Journal of Dermatology, 2025, 58(4): 289-296.doi:10.35541/cjd.20240569