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高嗜酸性粒细胞性皮炎:度普利尤单抗治疗成功病例

Hypereosinophilic Dermatitis: Successful Treatment with Dupilumab.

作者信息

Wu Chenyu, Zhang Jianzhong, Zhao Yan

机构信息

Department of Dermatology, People's Hospital of Peking University, Beijing, People's Republic of China.

出版信息

Biologics. 2023 Apr 20;17:57-60. doi: 10.2147/BTT.S400073. eCollection 2023.

Abstract

Hypereosinophilic dermatitis (HED) is a subtype of hypereosinophilic syndrome. HED is characterized by eosinophilic granulocytes increased in peripheral blood and bone marrow and infiltrated in skin. The clinical manifestations of HED are diffussed by erythema, papule and maculopapule with severe itching. The etiology of HED is unknown. At present, in addition to HED with FIP1L1-PDGFRA fusion gene positive, whose treatment is tyrosine kinase inhibitor, other types of HED first-line treatment are oral glucocorticoids, supplemented by antihistamines and immunosuppressants. Dupilumab is a human monoclonal antibody, which inhibits the IL-4 and IL-13 signaling by binding to the IL-4R-α and IL-13R-α-1 subunits of the receptor. We report a 76-year-old male patient with HED whose peripheral blood eosinophils decreased from 20.7% to 4.1% after 8 weeks of dupilumab, and his pruritus was completely relieved. Dupilumab was discontinued after 6 months of treatment. It is exciting that the patient has not experienced relapse for 17 months after the discontinuation. No adverse event was reported.

摘要

嗜酸性粒细胞增多性皮炎(HED)是嗜酸性粒细胞增多综合征的一种亚型。HED的特征是外周血和骨髓中嗜酸性粒细胞增多,并浸润至皮肤。HED的临床表现为弥漫性红斑、丘疹和斑丘疹,伴有严重瘙痒。HED的病因尚不清楚。目前,除了FIP1L1-PDGFRA融合基因阳性的HED,其治疗方法为酪氨酸激酶抑制剂外,其他类型的HED一线治疗是口服糖皮质激素,辅以抗组胺药和免疫抑制剂。度普利尤单抗是一种人单克隆抗体,它通过与受体的IL-4R-α和IL-13R-α-1亚基结合来抑制IL-4和IL-13信号传导。我们报告了一名76岁的HED男性患者,使用度普利尤单抗8周后,其外周血嗜酸性粒细胞从20.7%降至4.1%,瘙痒完全缓解。治疗6个月后停用度普利尤单抗。令人兴奋的是,停药后17个月患者未复发。未报告不良事件。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4afb/10124617/83d328e9959f/BTT-17-57-g0001.jpg

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