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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Bibliographic Details
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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Summary: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.
ISSN:0036-8075
1095-9203
DOI:10.1126/science.282.5388.490