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Science 16 October 1998:
Vol. 282. no. 5388, pp. 490 - 493
DOI: 10.1126/science.282.5388.490
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Reports

Inhibition of Toxic Epidermal Necrolysis by Blockade of CD95 with Human Intravenous Immunoglobulin

Isabelle Viard, Philippe Wehrli, * Roberto Bullani, * Pascal Schneider, Nils Holler, Denis Salomon, Thomas Hunziker, Jean-Hilaire Saurat, Jürg Tschopp, Lars E. French dagger

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 to 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.

I. Viard, P. Wehrli, R. Bullani, D. Salomon, J.-H. Saurat, L. E. French, Department of Dermatology, Geneva University Medical School, CH-1211 Geneva 4, Switzerland. P. Schneider, N. Holler, J. Tschopp, Institute of Biochemistry, University of Lausanne, BIL Research Center, Chemin des Boveresses 155, CH-1066 Epalinges, Switzerland. T. Hunziker, Department of Dermatology, University of Bern, Medical School, CH-3010 Bern, Switzerland.
*   These authors contributed equally to this work.

dagger    To whom correspondence should be addressed. E-mail: french{at}cmu.unige.ch

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