Systemic Therapy for Paediatric Stevens–Johnson Syndrome and Toxic Epidermal Necrolysis: A Systematic Review of Current Evidence and Limitations

Abstract

Aim

The role of systemic therapy in paediatric Stevens–Johnson Syndrome (SJS) and Toxic Epidermal Necrolysis (TEN) remains undefined. Management is primarily guided by observational data and variable practice patterns. This systematic review aims to integrate recent evidence and evaluate clinical outcomes associated with systemic therapies in paediatric SJS/TEN.

Methods

A systematic search of PubMed, Embase and Scopus was conducted from January 1990 to February 2025. Studies were eligible if they included patients (< 18 years) with SJS, SJS-TEN overlap or TEN managed with supportive care alone or systemic therapy (corticosteroids, intravenous immunoglobulin (IVIg), cyclosporin, TNF-α inhibitors or plasmapheresis). Studies without defined diagnoses or outcomes were excluded. Primary outcomes were mortality and length of hospital stay. Risk of bias was assessed using CARE guidelines for case reports and the Joanna-Briggs checklist for observational studies. Studies providing individual-level data were synthesised quantitatively, while aggregated data were described narratively.

Results

A total of 260 studies comprising 1608 paediatric patients met inclusion criteria. Most patients received systemic therapy, most commonly corticosteroids or IVIg. Systemic therapy was not associated with a shorter length of stay or reduced mortality rate compared to supportive care alone. Treatment with cyclosporin and TNF-α inhibitors trended towards lower mortality rates, and for cyclosporin, shorter length of stay. However, these findings did not reach statistical significance.

Conclusions

Evidence for systemic therapy remains limited by study heterogeneity and small sample sizes. Although emerging targeted therapies show early promise, firm conclusions regarding efficacy cannot be drawn. This highlights the need for further collaborative research.