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

Viard, Isabelle, Wehrli, Philippe, Bullani, Roberto, Schneider, Pascal, Holler, Nils, Salomon, Denis, Hunziker, Thomas, Saurat, Jean-Hilaire, Tschopp, Jürg, French, Lars E.·1998·Science

Abstract

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.

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Crossref · 10.1126/science.282.5388.490

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Metadata

Authors
Viard, I., Wehrli, P., Bullani, R., Schneider, P., Holler, N., Salomon, D., Hunziker, T., Saurat, J.-H., Tschopp, J., & French, L. E.
Venue
Science
Published
1998-10-16
Volume
282
Issue
5388
Pages
490-493
ISSN
  • 0036-8075 (ISSN-L)
  • 1095-9203
OpenAlex
W2048486696
Cited by
1,005
References
0
FWCI
30.52
Type
journal article
Language
en
Open access
closed

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Contains information from the Semantic Scholar Academic Graph Datasets, made available under the ODC Attribution License (ODC-BY 1.0).

Semantic Scholar’s data is described in: Kinney, R., et al. (2023). The Semantic Scholar Open Data Platform. arXiv:2301.10140.