Systematic review of treatments for Stevens-Johnson syndrome and toxic epidermal necrolysis using the SCORTEN score as a tool for evaluating mortality.

Epidermal necrolysis, whether involving limited, that is, Stevens-Johnson syndrome (SJS), or large, that is, toxic epidermal necrolysis (TEN), areas of the skin surface, is associated with high mortality. A specific prognosis score (SCORTEN) accurately predicts mortality. Management of SJS and TEN mainly relies on supportive care but several treatments have been proposed, particularly corticosteroids and intravenous immunoglobulins (IVIG), to block the progression of the disease. The aim of this study was a pooled analysis of published series addressing the question of treatment efficacy by comparing the mortality observed to the mortality predicted by the SCORTEN score. A literature search was carried out through PubMed from January 2001 to December 2009. It found 47 original series including at least 10 patients. Among these, 13 containing a clear description of treatment(s) and an evaluation of SCORTEN score were analyzed allowing the calculation of a mortality ratio (MR) for each series and a pooled MR with 95% confidence interval (CI) for each treatment. The analyzed series comprised a total of 439 patients. Supportive care was used only in 199 patients with a pooled MR of 0.89 (CI 0.67-1.16, p = 0.43), corticosteroids were administered to 78 patients with a pooled MR of 0.92 (CI 0.53-1.48, p = 0.84), and IVIG in 162 with a pooled MR of 0.82 (CI 0.58-1.12, p = 0.23). In conclusion, even though this analysis had some limitations, it strongly suggested that neither corticosteroids nor IVIG provide any important reduction in the risk of dying from SJS and TEN.