Publication Date:
1998-10-17
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.〈br /〉〈span class="detail_caption"〉Notes: 〈/span〉Viard, I -- Wehrli, P -- Bullani, R -- Schneider, P -- Holler, N -- Salomon, D -- Hunziker, T -- Saurat, J H -- Tschopp, J -- French, L E -- New York, N.Y. -- Science. 1998 Oct 16;282(5388):490-3.〈br /〉〈span class="detail_caption"〉Author address: 〈/span〉Department of Dermatology, Geneva University Medical School, CH-1211 Geneva 4, Switzerland.〈br /〉〈span class="detail_caption"〉Record origin:〈/span〉 〈a href="http://www.ncbi.nlm.nih.gov/pubmed/9774279" target="_blank"〉PubMed〈/a〉
Keywords:
Adolescent
;
Adult
;
Aged
;
Aged, 80 and over
;
Antibodies, Blocking/immunology/therapeutic use
;
Antigens, CD95/immunology/*physiology
;
*Apoptosis
;
Child
;
Dermis/pathology
;
Disease Progression
;
Epidermis/pathology
;
Fas Ligand Protein
;
Female
;
Humans
;
Immunoglobulins, Intravenous/*therapeutic use
;
Jurkat Cells
;
Keratinocytes/metabolism/*pathology
;
Male
;
Membrane Glycoproteins/metabolism
;
Middle Aged
;
Pilot Projects
;
Stevens-Johnson Syndrome/pathology/*therapy
Print ISSN:
0036-8075
Electronic ISSN:
1095-9203
Topics:
Biology
,
Chemistry and Pharmacology
,
Computer Science
,
Medicine
,
Natural Sciences in General
,
Physics
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