Toxic Epidermal Necrolysis Treated with Intravenous High-Dose Immunoglobulins: Our Experience

Background: Toxic epidermal necrolysis (TEN) is a rare severe acute exfoliative drug-induced skin disorder which has recently been ascribed to alterations in the control of keratinocyte apoptosis, mediated by an interaction between the cell surface death receptor Fas and its respective ligand. A therapeutic approach with intravenous immunoglobulins (IVIG) associated with pulse methylprednisolone, based on the inhibition of Fas-mediated keratinocyte death by naturally occurring Fas-blocking antibodies included in human immunoglobulin preparations, has produced good preliminary results. Objective: 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 dermo-epidermal detachment from 4 to 37% were treated. Results: Eight patients were healed and 1 died of septic shock and multiple organ failure. Interruption of further epidermal detachment occurred after an average of 4.8 days from the onset of IVIG therapy. Complete wound healing occurred after an average of 12 days. Concerning complications, 3 out of 8 surviving patients had acute respiratory failure requiring mechanical ventilation and 1 acute renal failure was treated with dialysis. Late sequelae were limited to dyschromia and nail dystrophies. No hypertrophic scars were observed. Conclusion: IVIG therapy represents a safe and valid approach for TEN.