Since identification and rapid withdrawal of the culprit drug positively correlate with the prognosis in SJS and TEN patients a priority should be to identify and immediately stop the potentially causal drug(s).
The current standard of care in SJS and TEN management is high-quality supportive care ideally (depending of the severity of disease symptoms and availability) within an intensive care or burn unit.
No specific therapy for SJS or TEN has yet shown efficacy in prospective, controlled clinical trials, mainly due to the low prevalence of SJS/TEN and its rapid life-threatening potential which make it extremely difficult to perform appropriately powered (large enough) randomized clinical trials. Although specific therapies with high evidence-based medicine standards of acceptance do not exist to date, conceptually interesting approaches based upon known elements of the pathogenesis of SJS/TEN and small case series have been described. Diverse treatments, such as systemic corticosteroids, cyclosporine, plasmapheresis, TNF-α antagonists and intravenous Immunoglobulins (IVIg) have shown promise and should be further investigated. The IRTEN prospective registry is aimed at helping clinicians and scientists better understand which therapy may best help patients.