Inhibition of toxic epidermal necrolysis by blockade of CD95 with human intravenous immunoglobulin

I Viard, P Wehrli, R Bullani, P Schneider, N Holler… - Science, 1998 - science.org
I Viard, P Wehrli, R Bullani, P Schneider, N Holler, D Salomon, T Hunziker, JH Saurat…
Science, 1998science.org
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 …
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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