中华皮肤科杂志 ›› 2026, Vol. 59 ›› Issue (5): 387-395.doi: 10.35541/cjd.20250587

• 指南与共识 •    下一篇

抗p200类天疱疮诊断与治疗专家共识(2025版)

中国“一带一路”皮肤病学专科联盟水疱病专病联盟    中华医学会皮肤性病学分会天疱疮/大疱性类天疱疮研究协作组   

  1. 中国“一带一路”皮肤病学专科联盟水疱病专病联盟    中华医学会皮肤性病学分会天疱疮/大疱性类天疱疮研究协作组
  • 收稿日期:2025-10-21 修回日期:2026-03-09 发布日期:2026-04-30
  • 通讯作者: 冯素英;潘萌;张桂英 E-mail:fengsy@pumcderm.cams.cn; panmeng@medmail. com.cn; lindazgy@csu.edu.cn
  • 基金资助:
    江苏省卫生健康委科研项目(ZD2021035);中国医学科学院临床与转化专项项目(2023-I2M-C&T-B-112);国家重点研发计划(2022YFC3601800);中国医学科学院医学与健康科技创新工程项目(2021-I2M-1-059)

Expert consensus on the diagnosis and treatment of anti-p200 pemphigoid(2025 edition)

Blistering Diseases Research Group, China “Belt and Road” Dermatology Alliance; Collaborative Group on Pemphigus/Bullous Pemphigoid Research, Chinese Society of Dermatology   

  1. Blistering Diseases Research Group, China “Belt and Road” Dermatology Alliance; Collaborative Group on Pemphigus/Bullous Pemphigoid Research, Chinese Society of Dermatology
  • Received:2025-10-21 Revised:2026-03-09 Published:2026-04-30
  • Contact: Feng Suying; Pan Meng; Zhang Guiying E-mail:fengsy@pumcderm.cams.cn; panmeng@medmail. com.cn; lindazgy@csu.edu.cn
  • Supported by:
    Scientific Research Project of Jiangsu Provincial Health Commission(ZD2021035); Clinical and Translational Medicine Research Project of Chinese Academy of Medical Sciences (2023-I2M-C&T-B-112); National Key Research and Development Program of China (2022YFC3601800); CAMS Innovation Fund for Medical Sciences (2021-I2M-1-059)

摘要: 【摘要】 抗p200类天疱疮是一种少见的自身免疫性表皮下水疱病,临床表现具有多样性,易与其他自身免疫性表皮下水疱病混淆。其典型免疫学特征为:盐裂皮肤间接免疫荧光显示,患者血清IgG抗体结合于盐裂皮肤的真皮侧,且循环抗体可识别真皮提取物中相对分子质量200 000的蛋白。本共识涵盖抗p200类天疱疮的临床特征、诊断标准、病情评估指标、治疗策略、预后、随访及饮食指导。诊断需结合临床表现、组织病理、直接免疫荧光及血清学检查(如盐裂皮肤间接免疫荧光、免疫印迹等)。治疗应根据病情严重程度进行分级管理,强调个体化用药方案,并重视对药物不良反应的动态监测。

关键词: 类天疱疮, 大疱性, 治疗学, 诊断, 诊疗准则, 抗p200类天疱疮, 专家共识, 推荐意见

Abstract: 【Abstract】 Anti?p200 pemphigoid is a rare autoimmune subepidermal blistering disease characterized by diverse clinical manifestations, which can easily be confused with other autoimmune subepidermal blistering disorders. Its typical immunopathological feature is the binding of serum IgG antibodies to the dermal side of salt?split skin by indirect immunofluorescence, with circulating antibodies capable of recognizing a protein with a relative molecular mass of 200 000 in dermal extracts. This consensus covers the clinical features of, diagnostic criteria for, disease activity assessment of, therapeutic strategies for, prognosis and follow?up of anti?p200 pemphigoid, as well as dietary recommendations. Its diagnosis requires comprehensive evaluation integrating clinical manifestations, histopathological features, direct immunofluorescence and serological assays (such as indirect immunofluorescence on salt-split skin and immunoblotting). In terms of treatment, a stepwise strategy based on disease severity is recommended, with emphasis on individualized regimens and vigilant monitoring of adverse drug reactions.

Key words: Pemphigoid, bullous, Therapeutics, Diagnosis, Practice guidelines, Anti-p200 pemphigoid, Expert consensus, Recommendation

引用本文

中国“一带一路”皮肤病学专科联盟水疱病专病联盟 中华医学会皮肤性病学分会天疱疮/大疱性类天疱疮研究协作组. 抗p200类天疱疮诊断与治疗专家共识(2025版)[J]. 中华皮肤科杂志, 2026,59(5):387-395. doi:10.35541/cjd.20250587

Blistering Diseases Research Group, China “Belt and Road” Dermatology Alliance, Collaborative Group on Pemphigus/Bullous Pemphigoid Research, Chinese Society of Dermatology. Expert consensus on the diagnosis and treatment of anti-p200 pemphigoid(2025 edition)[J]. Chinese Journal of Dermatology, 2026, 59(5): 387-395.doi:10.35541/cjd.20250587