Analysis of intravenous immunoglobulin for the treatment of toxic epidermal necrolysis using SCORTEN - The University of Miami experience

Background: Toxic epidermal necrolysis (TEN) is a rare, life-threatening condition caused by certain medications. Keratinocytes affected by TEN have been found to undergo apoptosis mediated by Fas-FasL interactions. Treatment with intravenous immunoglobulin (IVIG) has been proposed to inhibit this interaction. Objective: To demonstrate the effectiveness of IVIG therapy in reducing mortality in patients with TEN. Design: A retrospective analysis of 16 consecutive patients with TEN who were treated with IVIG. The SCORTEN system, a validated predictor of TEN mortality, was used to analyze the data of these patients. Using SCORTEN, we compared the predicted mortality of our patient population with observed mortality. Main Outcome Measures: For each patient, causes of TEN and other medical problems were documented prior to IVIG therapy, as were the 7 independent SCORTEN risk factors. Results: One patient died. Based on the SCORTEN system, 5.81 patients were expected to die. These mortality rates were compared using the standardized mortality ratio (SMR) analysis ([Sigma. observed deaths/Sigma expected deaths] x 100) to determine the efficacy of this treatment, which showed that patients with TEN treated with IVIG were 83% less likely to die than those not treated with IVIG (SMR = 0.17; 95% confidence interval, 0.0-0.96). Setting: Dermatology inpatient unit at a university-affiliated hospital. Intervention: All 16 patients received IVIG treatment daily for 4 days. Fifteen patients received 1 g/kg per day and I patient received 0.4 g/kg per day. Conclusion: Based on comparison of our observed mortality rate with the SCORTEN-predicted mortality rate, treatment with IVIG significantly decreased mortality in patients with TEN.