中毒性表皮坏死松解症的诊疗进展

Advancement in the diagnosis and management of toxic epi- dermal necrolysis

中毒性表皮坏死松解症（TEN）常由药物不良反应引起，是重症大疱性药疹的一种。虽然TEN发病率低，但任何年龄均可发病，妇女和老年人更多见，总体病死率为25%～35%[1]。TEN急性期发展迅速，皮肤黏膜损害广泛，累及面积大于30%TBSA，并伴有全身中毒症状及内脏损害，如救治不及时患者可因出血、脓毒症及MOF等并发症死亡，是临床比较棘手的难题。TEN发展过程中，广泛的表皮黏膜剥脱、水电解质平衡紊乱、高代谢状态、免疫功能失调、脓毒症和器官衰竭等表现与烧伤极为相似。本文主要从TEN的病因和发病机制、诊断及鉴别诊断和治疗等方面作一综述。

Toxic epidermal necrolysis （TEN） is a po- tentially life-threatening condition usually attributed to severe ad- verse drug reactions. The evolvement of TEN, including exten- sive epidermal sloughing, fluid and electrolyte imbalances, hy- permetabolism, immune dysfunction, sepsis and organs failure, are very similar to that of extensive burn. There is no unified therapeutic regimen for TEN due to its unclear pathogenesis. This article reviews the recent progress in regard to TEN in etiology, pathogenesis, diagnosis, differential diagnosis, treatment, new standard diagnostic approach, primary care, and supportive treatment.