Stevens-Johnson综合征和中毒性表皮坏死松解症发病机制的研究进展

Recent progress in research of pathogeneses of Stevens-Johnson syndrome and toxic epidermal ;necrolysis

Stevens-Johnson综合征（Stevens-Johnson syndrome，SJS）和中毒性表皮坏死松解症（toxic epidermal necrolysis，TEN）是由药物引起的皮肤和黏膜的不良反应，易导致皮肤和眼部的慢性并发症，发病率低但病死率高。目前，对SJS和TEN发病机制的研究已深入到基因学、药理学和免疫学领域。遗传易感性在SJS和TEN的发病中发挥重要作用，多个HLA等位基因与其发病相关。已证实药物代谢过程及参与该过敏反应的免疫细胞（如调节性T细胞）和细胞因子（如Fas/FasL、颗粒溶素、颗粒酶B/穿孔素等）在其发病机制中意义重大。本文主要从遗传易感性、药物代谢过程和免疫细胞及细胞因子等方面，总结并讨论SJS和TEN的发病机制。（中华眼科杂志，2016，52：708-713）

Stevens-Johnson syndrome （SJS） and toxic epidermal necrolysis （TEN） are life-threatening cutaneous adverse drug reactions that induce widespread epidermal necrosis. Ocular and cutaneous diseases are common chronic sequelae of SJS and TEN. Several concepts have been proposed to explain the pathogenesis of severe cutaneous adverse drug reactions. Recent advances in genetic, pharmacogenomics and immunologic studies have provided evidences of genetic predispositions, drug metabolism and cytokines related to SJS and TEN. With regard to keratinocyte death, several cell death mediators, such as Fas/FasL, granulysin and TNF, have been proposed to play an important role in the pathogeneses of SJS and TEN. A subset of T lymphocytes, including regulatory T cells, may also play a role. This review summarizes the pathogeneses of SJS and TEN mainly from the aspects of genetic susceptibilities, drug metabolism, and immune cells and cytokines.