Diagnosis and Management of Stevens‐Johnson Syndrome and Toxic Epidermal Necrolysis in Pediatric Patients: A Systematic Review of Clinical Guidelines and Consensus Statements

Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are rare, life-threatening mucocutaneous reactions. Pediatric mortality is lower than in adults, but children face higher risks of recurrence and long-term sequelae. Despite disease severity, pediatric-specific guidelines remain scarce and often extrapolated from adult populations. We systematically reviewed and appraised national and international clinical practice guidelines and consensus statements on pediatric SJS/TEN, focusing on supportive care, pharmacologic interventions, mucosal management, and long-term follow-up. A systematic search of MEDLINE, Embase, PubMed, CENTRAL, Scopus, and CINAHL (January 2000-April 2025) identified national and international guidelines. For feasibility, we restricted full appraisal to documents published in English or French. Eligible documents provided explicit recommendations for patients < 18 years. Two reviewers independently extracted data and assessed quality using the AGREE II instrument. Of 144 records screened, 10 guidelines met inclusion criteria; 60% were published within the last 5 years, and 50% were consensus-based without systematic evidence synthesis. Most (90%) endorsed supportive measures such as wound care, nutritional support, and fluid resuscitation. Recommendations for pharmacologic therapy varied considerably: intravenous immunoglobulin (50%), systemic corticosteroids (40%), and cyclosporine (40%), with limited pediatric dosing guidance. Only one guideline mentioned biologics. Multidisciplinary care was recommended in 90%, yet long-term follow-up for sequelae appeared in only 50%. AGREE II appraisal showed high clarity of presentation but consistently low applicability; only the British Association of Dermatologists guideline achieved high quality across all domains. Current pediatric SJS/TEN guidelines display heterogeneity in scope and rigor. While supportive care is emphasized, pharmacologic and long-term management guidance remains inconsistent. Standardized, evidence-based, pediatric-specific guidelines are urgently needed.