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

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.