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用度普利尤单抗治疗的瘙痒性大疱性表皮松解症。

Epidermolysis bullosa pruriginosa treated with dupilumab.

作者信息

Zhou Amanda G, Little Alicia J, Antaya Richard J

机构信息

Yale University School of Medicine, New Haven, CT, USA.

Department of Dermatology, Yale University School of Medicine, New Haven, CT, USA.

出版信息

Pediatr Dermatol. 2021 Mar;38(2):526-527. doi: 10.1111/pde.14493. Epub 2020 Dec 18.

Abstract

Epidermolysis bullosa pruriginosa (EBP) is a variant of dystrophic epidermolysis bullosa characterized by intense pruritus and prurigo nodularis-like lesions. While medical therapies for EBP exist, current treatments are not consistently effective, and symptoms often cause decreased quality of life. Here, we report two cases of EBP treated with dupilumab, which decreased symptoms of pruritus and improved skin findings. Both patients have been on dupilumab for over one year with sustained improvement and no adverse effects; although in one patient, increased dosing was required to attain optimal control of disease.

摘要

瘙痒性大疱性表皮松解症(EBP)是营养不良性大疱性表皮松解症的一种变体,其特征为剧烈瘙痒和结节性痒疹样皮损。虽然存在针对EBP的药物治疗方法,但目前的治疗并非始终有效,且症状常常导致生活质量下降。在此,我们报告两例用度普利尤单抗治疗的EBP病例,该治疗减轻了瘙痒症状并改善了皮肤表现。两名患者使用度普利尤单抗均已超过一年,病情持续改善且无不良反应;不过,其中一名患者需要增加剂量以实现疾病的最佳控制。

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