Stevens-Johnson综合征/中毒性表皮坏死松解综合征的发病机制研究进展

Stevens-Johnson综合征/中毒性表皮坏死松解综合征(SJS/TEN)是一类少见的,由药物引起的以广泛皮肤松解坏死为特征的重症皮肤不良反应,临床主要表现为皮肤黏膜疼痛、红斑、水疱、表皮剥脱等。虽然本病发病率很低,但死亡率高。大量研究表明其主要是由遗传、药理、免疫等多方面共同作用的结果,但发病机制目前尚未完全明确,目前,已发现多种人类白细胞抗原(HLA)风险等位基因与特定药物引起的SJS/TEN相关。近年来,除了更为精准的认识到HLA在抗原的识别、提呈过程中的作用,免疫细胞(T细胞、NKT细胞等)及细胞因子(穿孔素/颗粒酶B、颗粒溶素、Fas-FasL、TNF-α、IL-15等)在发病机制中的作用也得到了更为广泛的研究,本文主要对近年来SJS/TEN发病机制方面的研究进展进行综述。

Research Updates of the Pathogenesis Mechanisms of Stevens-Johnson Syndrome/toxic Epidermal Necrolysis Syndrome

Stevens-Johnson syndrome/toxic epidermal necrolysis syndrome ( SJS/TEN ) is a rare, mostly (ling-induced severe cutaneous adverse reaction which is characterized by widespread epidermal necrosis. The main clinical manifestations are mucocutaneous pain,erythema,blisters,epidermal exfoliation ,etc. In severe cases, multiple organs are involved. Although the incidence is low, the mortality is high. Numerous studies have shown that it is mainly the result of a combination of genetics, pharmacology, and immunity. However, the exact pathogenesis is still unclear. In recent years, in addition to a more accurate recognition of the role of HLA in antigen recognition and presentation , the role of immune cells ( T cells, NKT cells, etc ), and cytokines (Perforin/granzyme B, Granuiysin, Fas-FasL, TNF-α, IL-15, etc.) in the pathogenesis of SJS/TEN have also been studied more extensively. Phis article mainly reviews recent advances in the pathogenesis of SJS/TEN.