白细胞介素36受体拮抗剂缺陷与皮肤无菌性脓疱病研究进展  

Research progress of deficiency of interleukin-36 receptor antagonist and cutaneous sterile pustulosis

作　　者：王雅辰 张振华[1] 徐子刚[1] Wang Yachen;Zhang Zhenhua;Xu Zigang(Department of Dermatology,Beijing Children′s Hospital,Capital Medical University,National Center for Children′s Health,Beijing 100045,China)

机构地区：[1]国家医学儿童中心,首都医科大学附属北京儿童医院皮肤科,北京100045

出　　处：《中华实用儿科临床杂志》2021年第20期1598-1600,共3页Chinese Journal of Applied Clinical Pediatrics

摘　　要：皮肤无菌性脓疱病系非感染性、非毛囊性脓疱病,发病率低且治疗困难。白细胞介素(IL)36受体拮抗剂缺陷(DITRA)是由IL36RN突变所致的IL-36受体拮抗剂(IL-36Ra)活性下降的自身炎症性疾病,IL-36Ra功能或结构缺陷会导致角质形成细胞、巨噬细胞、树突状细胞分泌炎症因子和促炎因子增加,上调的IL-36受体激动剂可诱导17型辅助性T淋巴细胞分泌IL-17,而IL-17是多种无菌性脓疱病的发病基础。研究DITRA与皮肤无菌性脓疱病的关系对于靶向治疗有重要意义。Cutaneous sterile pustulosis is categorized as a non-infectious and non-follicular impetigo,with a low prevalence and difficulty in the treatment.Deficiency of interleukin(IL)-36 receptor antagonist(DITRA)is an auto inflammatory disease featured by the decreased interleukin-36 receptor antagonist(IL-36Ra)activity caused by IL36RN mutation.Functional or structural defects of IL-36Ra increase the secretion of inflammatory and pro-inflammatory factors by keratinocytes,macrophages and dendritic cells.Upregulation of IL-36 receptor agonists induce type 17 helper T lymphocytes to secrete IL-17,which is essential for the onset of multiple subtypes of aseptic pustulosis.Research on the relationship between DITRA and cutaneous sterile pustulosis is important for developing targeted therapies.

关 键 词：脓疱病 无菌性 皮肤 IL36RN基因 

分 类 号：R758.6[医药卫生—皮肤病学与性病学]