Abstract

Background: Toxic epidermal necrolysis (TEN) is an acute adverse drug reaction, that has an unpredictable progression and a 30% mortality. The incidence of TEN in the general population is approximately 0.4 to 1.2 cases/million/year. It is characterized pathologically by keratinocyte apoptosis which leads to epidermal detachment. Keratinocyte apoptosis is triggered by activation of the Fas-FasL, pathway and could be prevented by the use of intravenous immunoglobulin (IVIG). Aim: To report the experience with the use of IVIG in TEN. Material and methods: Retrospective study of 15 patients with a diagnosis of Stevens-Johnson/TEN overlap (SJS/TEN) or TEN, that received a total dose of 2.3 ± 0.6 mg/kg of IVIG over a period of 3 to 4 days. The infusion was initiated during the first 24 hours after diagnosis and was associated with standard care for burn victims. Steroids were avoided if the patient was not in chronic steroidal therapy. Results: All patients responded to IVIG in a lapse of 46.4 ± 14.2 hours from the beginning of infusion. Eighty percent of patients survived, but one developed acute renal failure due to IVIG, and another became blind due to corneal opacities, a complication of TEN. Those who survived were discharged after a lapse of 19.8 ± 6.6 days from the beginning of the disease. Conclusions: Despite the lack of blind, multicentric and randomized trials, we agree with some international studies that IVIG is beneficial as a treatment for SSJ/NET and TEN.