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静脉注射人免疫球蛋白治疗药物性中毒性表皮坏死松解症(莱尔综合征)

Treatment of drug-induced toxic epidermal necrolysis (Lyell's syndrome) with intravenous human immunoglobulins.

作者信息

Paquet P, Jacob E, Damas P, Piérard G E

机构信息

Department of Dermatopathology, CHU Sart Tilman, 4000, Liège, Belgium.

出版信息

Burns. 2001 Sep;27(6):652-5. doi: 10.1016/s0305-4179(01)00005-5.

DOI:10.1016/s0305-4179(01)00005-5
PMID:11525863
Abstract

Toxic epidermal necrolysis (TEN) is a rare drug-induced life-threatening disease. Currently, the disease is only treated by supportive and antiseptic measures. Quite recently intravenous immunoglobulins (IG) were shown to be a promising TEN treatment. The rationale for their use is based on the fact that keratinocyte apoptosis in TEN involves the CD95 (APO-1/Fas) cell surface receptor-ligand system. We successfully treated a TEN patient with high dose of intravenous IG. The clinical recovery appeared exceptionally rapid. Immunohistochemistry showed that the IG action probably developed on the CD95 receptor-ligand system at the keratinocytes surface.

摘要

中毒性表皮坏死松解症(TEN)是一种罕见的、由药物引起的危及生命的疾病。目前,该疾病仅通过支持性和防腐措施进行治疗。最近,静脉注射免疫球蛋白(IG)被证明是一种有前景的TEN治疗方法。使用它们的理论依据基于这样一个事实,即TEN中的角质形成细胞凋亡涉及CD95(APO-1/Fas)细胞表面受体-配体系统。我们用高剂量静脉注射IG成功治疗了一名TEN患者。临床恢复异常迅速。免疫组织化学显示,IG的作用可能是在角质形成细胞表面的CD95受体-配体系统上发挥的。

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