Effect of high-dose intravenous immunoglobulin therapy in Stevens-Johnson syndrome: a retrospective,multicenter study

BACKGROUND:Stevens-Johnson syndrome (SJS) is a severe cutaneous drug reaction associated with considerable morbidity, possible transition to toxic epidermal necrolysis (TEN) and death in certain cases. OBJECTIVE: To determine whether treatment with high-dose IVIG in SJS patients may improve outcome. METHODS: Data from 12 patients (collected between January 1997 and November 2000 from 7 university dermatology centers in Europe and North America) diagnosed with SJS according to a recent consensus definition was analyzed retrospectively. All patients had progressive ongoing epidermal detachment at the time of treatment initiation. Patients with overlap syndromes and TEN were excluded. Tolerance, survival at 45 days after onset and total healing time were assessed. RESULTS: Twelve SJS patients (mean age 44 years) were treated with IVIG at a mean dose of 0.6g/kg/day for an average of 4 days. An objective response to IVIG infusion was observed in all patients within a mean of 2 days, and the overall survival rate was 100%. Total skin healing occurred, on average, within 8.3 days. Time to total healing was shorter in a group of patients with fewer severe underlying diseases who had received IVIG infusion rapidly after the onset of skin lesions. CONCLUSION: High-dose IVIG may be effective in blocking the progression of SJS and reducing the time to complete skin healing.