中国麻风皮肤病杂志 ›› 2026, Vol. 42 ›› Issue (3): 203-205.doi: 10.12144/zgmfskin202603203

• 临床研究 • 上一篇    下一篇

度普利尤单抗治疗家族性良性慢性天疱疮一例并文献复习

何静1,黄晓慧1,朱梓墨1,窦侠1,2,3   

  1. 1北京大学深圳医院皮肤性病科，广东深圳, 518036;2深圳皮肤疾病转化医学重点实验室，广东深圳,518036;3深圳北京大学香港科技大学医学中心皮肤病研究所，广东深圳,518036
  • 出版日期:2026-03-15 发布日期:2026-03-10

Dupilumab for the treatment of Hailey-Hailey disease: a case report and literature review

HE Jing1, HUANG Xiaohui1, ZHU Zimo1, DOU Xia1,2,3   

  1. 1 Department of Dermatology, Peking University Shenzhen Hospital, Shenzhen 518036, China; 2 Shenzhen Key Laboratory for Translational Medicine of Dermatology, Shenzhen 518036, China; 3 Institute of Dermatology, Shenzhen Peking University-The Hong Kong University of Science and Technology Medical Center, Shenzhen 518036, China
  • Online:2026-03-15 Published:2026-03-10

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摘要： 患者，男，37岁。双侧腹股沟、阴囊、肛周红斑、糜烂伴痛8年，皮损组织病理符合家族性良性慢性天疱疮特点。其父有类似病史。患者病情反复发作，多种治疗方法疗效欠佳。根据既往度普利尤单抗治疗家族性良性慢性天疱疮的文献，给予患者度普利尤单抗治疗，随访 4 个月皮损控制理想，未发现不良反应。与既往报道多为广泛或中重度难治病例不同，本例皮损主要局限于腹股沟、阴囊及肛周高摩擦部位，病程长且反复发作。

关键词: 度普利尤单抗, 家族性良性慢性天疱疮

Abstract: A 37-year-old male patient presented with an 8-year history of erythema, erosion and pain on bilateral inguinal regions, scrotum and perianal area. Histopathological examination of the skin lesions was consistent with the characteristics of familial chronic benign pemphigus (Hailey-Hailey disease). His father had a similar medical history. The patient's condition recurred frequently and responded poorly to various treatments. Based on previous literature reporting the use of dupilumab in the treatment of familial chronic benign pemphigus, dupilumab was administered to the patient. After 4 months of follow-up, the skin lesions were well-controlled without any adverse reactions observed. Different from previously reported cases which were mostly extensive, moderate-to-severe and refractory, the lesions in this case were mainly confined to the high-friction areas including bilateral inguinal regions, scrotum and perianal area, with a long and recurrent course of disease.

Key words: dupilumab, Hailey-Hailey disease