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两名重症中毒性表皮坏死松解症患者在接受皮质类固醇治疗失败后进行血浆置换取得了有益的治疗效果。

Beneficial therapeutic effect of plasmapheresis after unsuccessful treatment with corticosteroids in two patients with severe toxic epidermal necrolysis.

作者信息

Szczeklik Wojciech, Nowak Ilona, Seczynska Bozena, Sega Aurelia, Krolikowski Wieslaw, Musial Jacek

机构信息

Department of Medicine, Division of Critical Care, Jagiellonian University, Krakow, Poland.

出版信息

Ther Apher Dial. 2010 Jun;14(3):354-7. doi: 10.1111/j.1744-9987.2009.00800.x.

Abstract

Toxic epidermal necrolysis (TEN) is a rare, life-threatening disease with a high mortality rate. It is linked to drug toxicity and characterized by epidermal necrolysis with mucositis and conjunctivitis. Treatment is not established due to the unknown pathogenesis and lack of randomized clinical trials. It is mostly based on withdrawal of the culprit drug and symptom-related approach. The role of corticosteroids and plasmapheresis in the disease treatment remains controversial. We present two patients with severe TEN (both with >80% body skin surface involvement) treated unsuccessfully with corticosteroids followed by plasmapheresis. Plasmapheresis led to prompt improvement, with extensive reepithealization of the skin, and eventually total recovery of both patients. In severe TEN unresponsive to corticosteroids, treatment with plasmapheresis should be considered.

摘要

中毒性表皮坏死松解症(TEN)是一种罕见的、危及生命的疾病,死亡率很高。它与药物毒性有关,其特征为伴有黏膜炎和结膜炎的表皮坏死松解。由于发病机制不明且缺乏随机临床试验,目前尚无既定的治疗方法。治疗大多基于停用致病药物及对症治疗。皮质类固醇和血浆置换在该病治疗中的作用仍存在争议。我们报告了两名重度TEN患者(均有超过80%的体表面积受累),先用皮质类固醇治疗无效,随后进行血浆置换。血浆置换使病情迅速改善,皮肤广泛重新上皮化,两名患者最终均完全康复。对于对皮质类固醇无反应的重度TEN患者,应考虑采用血浆置换治疗。

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