Hoetzenecker Wolfram, Mehra Tarun, Saulite Ieva, Glatz Martin, Schmid-Grendelmeier Peter, Guenova Emmanuella, Cozzio Antonio, French Lars E
Department of Dermatology, University Hospital of Zürich, Zürich, Switzerland.
Medical Directorate, University Hospital of Zürich, Zürich, Switzerland.
F1000Res. 2016 May 20;5. doi: 10.12688/f1000research.7574.1. eCollection 2016.
Toxic epidermal necrolysis (TEN) is a rare, life-threatening drug-induced skin disease with a mortality rate of approximately 30%. The clinical hallmark of TEN is a marked skin detachment caused by extensive keratinocyte cell death associated with mucosal involvement. The exact pathogenic mechanism of TEN is still uncertain. Recent advances in this field have led to the identification of several factors that might contribute to the induction of excessive apoptosis of keratinocytes. In addition, specific human leukocyte antigen types seem to be associated with certain drugs and the development of TEN. As well-controlled studies are lacking, patients are treated with various immunomodulators (e.g. intravenous immunoglobulin) in addition to the best supportive care.
中毒性表皮坏死松解症(TEN)是一种罕见的、危及生命的药物性皮肤病,死亡率约为30%。TEN的临床特征是由广泛的角质形成细胞死亡伴黏膜受累引起的明显皮肤剥脱。TEN的确切致病机制仍不确定。该领域的最新进展已导致确定了几种可能促成角质形成细胞过度凋亡诱导的因素。此外,特定的人类白细胞抗原类型似乎与某些药物及TEN的发生有关。由于缺乏严格对照的研究,除了最佳支持治疗外,还使用各种免疫调节剂(如静脉注射免疫球蛋白)对患者进行治疗。