中毒性表皮坏死松解症的研究进展

Toxic epidermal necrolysis

中毒性表皮坏死松解症是一种以表皮剥脱坏死为主要表现的药源性疾病，伴有表皮、黏膜以及肝肾功能的损害，死亡率高。其发病机制尚不完全清楚，遗传易感性、特定的T细胞抗原受体序列、药物特异性T细胞、细胞毒蛋白均在其中起着重要作用。治疗的主要方法包括一般处理、血浆置换、糖皮质激素、免疫球蛋白、肿瘤坏死因子a抑制剂等，尤其糖皮质激素和肿瘤坏死因子a抑制剂的疗效近年来得到较多肯定。

Toxic epidermal necrolysis （TEN） is a kind of drug-induced disease with epidermal necrosis as the main manifestation. It may affect epidermis and mucosa with hepatic and renal involvement, and often carries a high mortality rate. The pathogenesis of TEN remains unanswered, and is considered to be closely associated with genetic predisposition, specific T cell receptor sequence, drug-specific T cells and cytotoxic proteins. Treatment options mainly include general management, plasmapheresis, glueocorticoids, immunoglobulin, tumor necrosis factor （TNF）-alpha inhibitors, and so on. Recent studies have confirmed the effectiveness of glucocorticoids and tumor necrosis factor （TNF）-alpha inhibitors.