Toxic epidermal necrolysis (TEN) and Stevens-Johnson syndrome (SJS): experience with high-dose intravenous immunoglobulins and topical conservative approach. A retrospective analysis.

Toxic epidermal necrolysis (TEN) and Stevens-Johnson syndrome (SJS) are rare, drug-induced, severe acute exfoliative skin and mucosal disorders. Several treatments previously proposed have produced contradictory results in small series; in 1998 the use of intravenous immunoglobulins (IVIG) was introduced with excellent clinical findings. Our experience (1999-2005) using IVIG in the therapy of TEN/SJS, together with a local conservative approach, is reported and related to our previous treatments (1993-1998). The SCORTEN and the standardized mortality ratio (SMR) was used to evaluate the efficacy of our therapeutic modalities. Eight patients were treated before IVIG era and 23 patients have been treated with IVIG. There was no significant difference in SCORTEN between the two groups. Concerning the local approach, a conservative wound management in IVIG series replaced an extensive epidermal debridment and coverage with artificial skin substitutes of the pre-IVIG series. Overall mortality in patients treated before IVIG was 75% (6/8), in the IVIG group it decreased to 26% (6/23) with a cessation of further epidermal detachment after an average of 5 days (3-10 days) from the onset of the therapy. The SMR showed a trend to lower actual mortality (not significative) with IVIG treatment than the predicted mortality (SMR=0.728; 95% CI: 0.327-1.620).