中国麻风皮肤病杂志 ›› 2026, Vol. 42 ›› Issue (10): 722-724.doi: 10.12144/zgmfskin202610722

• 临床研究 • 上一篇    下一篇

乌帕替尼联合阿维A治疗家族性良性慢性天疱疮一例

浦天舒1,滕雅如1,武欣萌1,笪美红2,董正邦2,王飞2   

  1. 1东南大学医学院，江苏南京，210009；2东南大学附属中大医院，江苏南京，210009
  • 出版日期:2026-10-15 发布日期:2026-10-09

Upadacitinib combined with acitretin for the treatment of Hailey-Hailey disease: a case report

PU Tianshu1, TENG Yaru1, WU Xinmeng1, DA Meihong2, DONG Zhengbang2, WANG Fei2   

  1. 1 Southeast University, School of Medicine, Nanjing 210009, China; 2 Zhongda Hospital, Southeast University, Nanjing 210009, China
  • Online:2026-10-15 Published:2026-10-09

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摘要： 本文报道1例病程40余年、经常规米诺环素、阿维A治疗效果不佳的难治性家族性良性慢性天疱疮患者，予乌帕替尼联合阿维A治疗，4周后皮损明显缓解，2个月皮损基本消退。随访6个月无复发。文献复习显示，目前仅14例JAK抑制剂治疗家族性良性慢性天疱疮的个案报道，各类JAK抑制剂均可快速改善皮损，短期不良反应轻微，但仍缺乏大样本长期研究。

关键词: 家族性良性慢性天疱疮, Hailey-Hailey病, 乌帕替尼, 阿维A

Abstract: We report a patient with refractory Hailey-Hailey disease (HHD) who had a disease course of more than 40 years and failed conventional treatments including minocycline and acitretin. The patient received combination therapy of upadacitinib and acitretin, with remarkable relief of skin lesions at week 4 and nearly complete resolution of lesions at month 2. The patient was followed-up for 6 months with no recurrence. Literature review revealed that there are only 14 case reports regarding the application of JAK inhibitors in HHD at present. All types of JAK inhibitors can rapidly ameliorate skin lesions with mild short-term adverse reactions, while large-sample and long-term clinical studies are still lacking.

Key words: familial benign chronic pemphigus, Hailey-Hailey disease, upadacitinib, acitretin