Inhibition of Toxic Epidermal Necrolysis by Blockade of CD95 with Human Intravenous Immunoglobulin
- 16 October 1998
- journal article
- clinical trial
- Published by American Association for the Advancement of Science (AAAS) in Science
- Vol. 282 (5388) , 490-493
- https://doi.org/10.1126/science.282.5388.490
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.Keywords
This publication has 26 references indexed in Scilit:
- Early Murine Cytomegalovirus (MCMV) Infection Induces Liver Natural Killer (NK) Cell Inflammation and Protection Through Macrophage Inflammatory Protein 1α (MIP-1α)–dependent PathwaysThe Journal of Experimental Medicine, 1998
- Sunlight-induced basal cell carcinoma tumor cells and ultraviolet-B-irradiated psoriatic plaques express Fas ligand (CD95L).Journal of Clinical Investigation, 1998
- Kinetics of Response in Lymphoid Tissues to Antiretroviral Therapy of HIV-1 InfectionScience, 1997
- Melanoma Cell Expression of Fas(Apo-1/CD95) Ligand: Implications for Tumor Immune EscapeScience, 1996
- Epidemiology of erythema exsudativum multiforme majus, Stevens-Johnson syndrome, and toxic epidermal necrolysis in Germany (1990–1992): Structure and results of a population-based registryJournal of Clinical Epidemiology, 1996
- Fas and Fas ligand in embryos and adult mice: ligand expression in several immune-privileged tissues and coexpression in adult tissues characterized by apoptotic cell turnover.The Journal of cell biology, 1996
- Apoptosis as a mechanism of keratinocyte death in toxic epidermal necrolysisBritish Journal of Dermatology, 1996
- Cytotoxic T cells deficient in both functional fas ligand and perforin show residual cytolytic activity yet lose their capacity to induce lethal acute graft-versus-host disease.The Journal of Experimental Medicine, 1996
- Severe Adverse Cutaneous Reactions to DrugsNew England Journal of Medicine, 1994
- Expression of Fas Antigen on Keratinocytes In Vivo and Induction of Apoptosis in Cultured KeratinocytesJournal of Investigative Dermatology, 1994