Recent advances in the diagnosis and treatment of Stevens-Johnson syndrome/toxic epidermal necrolysis.

Abstract

Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are rare but serious mucocutaneous disorders primarily caused by medications. Despite their low incidence, both conditions remain associated with high mortality and significant long-term complications. Recent studies have advanced the understanding of their pathogenesis, including the roles of neutrophil extracellular traps and several cell death pathways. However, reliable biomarkers for early diagnosis are lacking, and no internationally standardized diagnostic criteria have been established. To address these gaps, new diagnostic frameworks have been proposed that incorporate conventional cutaneous and mucosal findings with mandatory histopathological confirmation. These are expected to enhance diagnostic precision and facilitate global comparability. The increasing incidence of SJS/TEN-like reactions associated with immune checkpoint inhibitors and molecular targeted therapies further highlights the importance of accurate histopathological differentiation. Prognostic tools are essential for therapeutic planning. Although the Severity-of-Illness Score for Toxic Epidermal Necrolysis (SCORTEN) score remains widely used, newer systems offer improved risk stratification and practical utility in diverse clinical settings. However, the optimal use of immunomodulatory therapies remains uncertain owing to limited consensus and evidence. Corticosteroids continue to serve as first-line treatment, but more selective agents are gaining attention for their potential efficacy and reduced toxicity. This review summarizes recent progress in SJS/TEN diagnosis, severity assessment, and management. It discusses the utility and limitations of novel scoring systems and emerging therapies while highlighting persistent challenges, including high mortality, regional practice variation, and delayed standardization. International collaboration and high-quality evidence generation remain essential for improving patient outcomes.