Toxic epidermal necrolysis treated with intravenous high-dose immunoglobulins: Our experience

Background. Toxic epidermal necrolysis (TEN) is a rare severe acute exfolia tive drug-induced skin disorder which has recently been ascribed to alterat ions in the control of keratinocyte apoptosis, mediated by an interaction b etween the cell surface death receptor Fas and its respective ligand. A the rapeutic approach with intravenous immunoglobulins (IVIG) associated with p ulse methylprednisolone, based on the inhibition of Fas-mediated keratinocy te death by naturally occurring Fas-blocking antibodies included in human i mmunoglobulin preparations, has produced good preliminary results. Objectiv e: To analyse the efficacy of IVIG in the treatment of TEN. Patients: Nine patients with erythematous body surface area ranging from 38 to 85% and der moepidermal detachment from 4 to 37% were treated. Results: Eight patients were healed and 1 died of septic shock and multiple organ failure. Interrup tion of further epidermal detachment occurred after an average of 4.8 days from the onset of IVIG therapy. Complete wound healing occurred after an av erage of 12 days. Concerning complications, 3 out of 8 surviving patients h ad acute respiratory failure requiring mechanical ventilation and 1 acute r enal failure was treated with dialysis. Late sequelae were limited to dysch romia and nail dystrophies. No hypertrophic scars were observed. Conclusion : IVIG therapy represents a safe and valid approach for TEN. Copyright (C) 2001 S. Karger AG, Basel.