中国麻风皮肤病杂志 ›› 2026, Vol. 42 ›› Issue (3): 214-215.doi: 10.12144/zgmfskin202603214

• 病例报告 • 上一篇    下一篇

度普利尤单抗治疗痒疹样营养不良型大疱性表皮松解症一例

王莎,陈小强   

  1. 中部战区总医院皮肤科，湖北武汉，430070
  • 出版日期:2026-03-15 发布日期:2026-03-10

Dupilumab for the treatment of pruriginous dystrophic epidermolysis bullosa: a case report

WANG Sha, CHEN Xiaoqiang   

  1. Department of Dermatology, Central Theater Command General Hospital, Wuhan 430070, China
  • Online:2026-03-15 Published:2026-03-10

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摘要： 患者,男，68岁。四肢反复丘疹、水疱、结节伴瘙痒50余年。组织病理:角化过度，表皮下水疱形成，真皮及血管周围少量炎细胞浸润。免疫荧光: IgG、C3、IgM、IgA均阴性。诊断：痒疹样营养不良型大疱性表皮松解症(DEBP)。经抗组胺药、免疫抑制剂、糖皮质激素等对症治疗后皮疹无明显改善，瘙痒未缓解。度普利尤单抗为全人源化单克隆抗体，对特应性皮炎、大疱性类天疱疮和结节性痒疹的治疗有效，但对DEBP治疗研究资料相对缺乏。本案例采用度普利尤单抗治疗后，患者皮损部分变平，瘙痒较前明显缓解。

关键词: 营养不良型大疱性表皮松解症, 痒疹样, 度普利尤单抗

Abstract: A 68-year-old male presented with recurrent pruritic papules, vesicles, and nodules on the limbs for over 50 years. Histopathological examination revealed hyperkeratosis, subepidermal blister formation, and mild perivascular inflammatory infiltrate in the dermis. Immunofluorescence showed negative expression of IgG, C3, IgM, and IgA. The patient was diagnosed with pruriginous dystrophic epidermolysis bullosa pruriginosa (DEBP). Conventional therapies, including antihistamines, immunosuppressants, and corticosteroids, provided no significant improvement, with persistent pruritus. Dupilumab, a fully humanized monoclonal antibody, has demonstrated efficacy in atopic dermatitis, bullous pemphigoid, and prurigo nodularis, but clinical data on its use for DEBP remain limited. In this case, dupilumab treatment resulted in partial flattening of lesions, and marked alleviation of pruritus.

Key words: dystrophic epidermolysis bullosa, pruriginosa, dupilumab