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

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...

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Published in:Science (American Association for the Advancement of Science) 1998-10, Vol.282 (5388), p.490-493
Main Authors: Viard, Isabelle, Wehrli, Philippe, Bullani, Roberto, Schneider, Pascal, Holler, Nils, Salomon, Denis, Hunziker, Thomas, Saurat, Jean-Hilaire, Tschopp, Jürg, French, Lars E.
Format: Article
Language:English
Subjects:
Adolescent
Adult
Aged
Aged, 80 and over
Albumins
Antibodies
Antibodies, Blocking - immunology
Antibodies, Blocking - therapeutic use
Apoptosis
Biological and medical sciences
Care and treatment
Cell death
Child
Control Groups
Dermis - pathology
Disease Progression
Drug Use
Epidermis - pathology
Fas Ligand Protein
fas Receptor - immunology
fas Receptor - physiology
Female
Health aspects
Humans
Immune system
Immunoglobulins
Immunoglobulins, Intravenous - therapeutic use
Immunology
Inhibition
Jurkat Cells
Keratinocytes
Keratinocytes - metabolism
Keratinocytes - pathology
Ligands
Male
Medical research
Medical sciences
Membrane Glycoproteins - metabolism
Middle Aged
Patients
Pharmacology
Pharmacology. Drug treatments
Pilot Projects
Skin
Skin, nail, hair, dermoskeleton
Stevens-Johnson Syndrome - pathology
Stevens-Johnson Syndrome - therapy
T lymphocytes
Toxic epidermal necrolysis
Wigs
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cited_by cdi_FETCH-LOGICAL-c509t-e35312cad85f1cf63e6c7ffbb46845951a09a90642d0deb217f3ba0f4b78c6ad3
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container_end_page 493
container_issue 5388
container_start_page 490
container_title Science (American Association for the Advancement of Science)
container_volume 282
creator Viard, Isabelle
Wehrli, Philippe
Bullani, Roberto
Schneider, Pascal
Holler, Nils
Salomon, Denis
Hunziker, Thomas
Saurat, Jean-Hilaire
Tschopp, Jürg
French, Lars E.
description 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.
doi_str_mv 10.1126/science.282.5388.490
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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.</abstract><cop>Washington, DC</cop><pub>American Society for the Advancement of Science</pub><pmid>9774279</pmid><doi>10.1126/science.282.5388.490</doi><tpages>4</tpages></addata></record>
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identifier ISSN: 0036-8075
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source American Association for the Advancement of Science; JSTOR Archival Journals and Primary Sources Collection; Social Science Premium Collection; Alma/SFX Local Collection; Education Collection
subjects Adolescent
Adult
Aged
Aged, 80 and over
Albumins
Antibodies
Antibodies, Blocking - immunology
Antibodies, Blocking - therapeutic use
Apoptosis
Biological and medical sciences
Care and treatment
Cell death
Child
Control Groups
Dermis - pathology
Disease Progression
Drug Use
Epidermis - pathology
Fas Ligand Protein
fas Receptor - immunology
fas Receptor - physiology
Female
Health aspects
Humans
Immune system
Immunoglobulins
Immunoglobulins, Intravenous - therapeutic use
Immunology
Inhibition
Jurkat Cells
Keratinocytes
Keratinocytes - metabolism
Keratinocytes - pathology
Ligands
Male
Medical research
Medical sciences
Membrane Glycoproteins - metabolism
Middle Aged
Patients
Pharmacology
Pharmacology. Drug treatments
Pilot Projects
Skin
Skin, nail, hair, dermoskeleton
Stevens-Johnson Syndrome - pathology
Stevens-Johnson Syndrome - therapy
T lymphocytes
Toxic epidermal necrolysis
Wigs
title Inhibition of Toxic Epidermal Necrolysis by Blockade of CD95 with Human Intravenous Immunoglobulin
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