VIENNA — Corticosteroid therapy for Stevens-Johnson syndrome and toxic epidermal necrolysis received a big boost from two observational studies presented at the annual meeting of the European Society for Dermatological Research.
Juergen Schlingman, M.D., reported on 281 patients with Stevens-Johnson syndrome (SJS) or toxic epidermal necrosis (TEN) treated in French and German hospitals under the auspices of the European Registry of Severe Cutaneous Adverse Reactions (EuroSCAR) study group.
“This is the largest cohort of patients with SJS/TEN ever analyzed for treatment outcomes. It's the best data available, despite having the obvious limitations of an observational study,” said Dr. Schlingman of the University of Freiburg (Germany).
The results show that there is no benefit for high-dose intravenous immunoglobulin and that the use of corticosteroids—which has been a subject of controversy—may deserve a randomized therapeutic trial.
An overall death rate of 22.1% was seen in this series, which featured unbiased enrollment. Mortality was 18% in the 119 patients who received corticosteroids. In a multivariate analysis adjusted for age, disease severity, and other relevant variables, corticosteroid-treated patients were 60% less likely to die than were those who got only supportive care. In contrast, patients who got intravenous immunoglobulin (IVIg) alone had a 60% increased risk of mortality relative to those who received supportive care only. (See chart.)
Age proved a significant risk factor for mortality. Patients aged 40-70 years were 3.3-fold more likely to die than were those younger than age 40, and patients older than 70 were at 8.9-fold increased risk.
In recent years, a growing number of physicians have turned to high-dose IVIg based on several favorable published reports. But two recent patient series from Loyola University Chicago and the University of Toronto showed no benefit for IVIg (J. Burn Care Rehabil. 2004;25:81-8, 246-55). Dr. Schlingman said the most likely explanation for the reported favorable outcome with IVIg in some prior series is recruitment bias and exclusion of high-risk patients.
In a separate presentation at the meeting, S.H. Kardaun, M.D., reported on a series of 12 consecutive patients with SJS/TEN treated with dexamethasone pulse therapy, with highly favorable results.
The therapy consisted of one dose of dexamethasone IV 1.5 mg/kg given on each of 3 consecutive days starting as soon as the diagnosis was established. The mean time from the start of dexamethasone pulse therapy to lesion healing was 14.2 days. All cutaneous and mucosal lesions healed within 3 weeks except in two patients, both of whom had disseminated herpes simplex infections.
A single patient died of an underlying malignancy after his skin lesions were nearly healed. Predicted mortality based on the SCORTEN disease severity rating was four cases, said Dr. Kardaun of University Hospital Groningen (the Netherlands).
KEVIN FOLEY, RESEARCH/FORHAD S. HOSSAIN, DESIGN