Inhibition of toxic epidermal necrolysis by blockade of CD95 with human intravenous immunoglobulin

被引:806
作者
Viard, I
Wehrli, P
Bullani, R
Schneider, P
Holler, N
Salomon, D
Hunziker, T
Saurat, JH
Tschopp, J
French, LE [1 ]
机构
[1] Univ Geneva, Sch Med, Dept Dermatol, CH-1211 Geneva 4, Switzerland
[2] Univ Lausanne, BIL Res Ctr, Inst Biochem, CH-1066 Epalinges, Switzerland
[3] Univ Bern, Sch Med, Dept Dermatol, CH-3010 Bern, Switzerland
关键词
D O I
10.1126/science.282.5388.490
中图分类号
O [数理科学和化学]; P [天文学、地球科学]; Q [生物科学]; N [自然科学总论];
学科分类号
07 ; 0710 ; 09 ;
摘要
Toxic epidermal necrolysis (TEN, Lyell's syndrome) is a severe adverse drug reaction in which keratinocytes die and Large sections of epidermis separate from the dermis. Keratinocytes normally express the death receptor Fas (CD95); those from TEN patients were found ro express lytically active Fas ligand (FasL). Antibodies present in pooled human intravenous immunoglobulins (IVIG) blocked Fas-mediated keratinocyte death in vitro. In a pilot study, 10 consecutive individuals with clinically and histologically confirmed TEN were treated with IVIG; disease progression was rapidly reversed and the outcome was favorable in all cases. Thus, Fas-FasL interactions are directly involved in the epidermal necrolysis of TEN, and IVIG may be an effective treatment.
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页码:490 / 493
页数:4
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