Evaluation of clinical features and treatment modality of pediatric patients with Steven Johnson syndrome/toxic epidermal necrolysis: a single-center experience.

Abstract

Background/aim: Steven Johnson Syndrome (SJS) and Toxic Epidermal Necrolysis (TEN) are rare but life-threatening severe cutaneous drug hypersensitivity reactions. The aim of our study was to evaluate the clinical features of pediatric patients diagnosed with SJS, TEN or SJS/TEN overlap and to examine treatment modalities.

Materials and methods: Patients aged 0-18 years who were followed up with SJS, TEN or SJS/TEN overlap at Ankara Bilkent City Hospital between August 2019 and January 2024 were retrospectively analyzed.

Results: Twelve patients who met the inclusion criteria were included in the study. Five of the patients had SJS, four had SJS/TEN overlap, and three had TEN. Eight of the patients were female, and the median age at presentation was 10.5 (IQR:6-16) years. Ten of the patients had a history of drug use. Eight patients had antibiotics, two had proton pump inhibitors, two had allopurinol, and two had antiepileptic (lamotrigine and valproic acid) use. All 12 patients received IVIG and systemic steroid therapy. Three TEN and four SJS/TEN overlap patients received cyclosporine. Two TEN patients underwent plasmapheresis. The most common long-term sequelae were dermatological sequelae.

Conclusion: SJS/TEN patients should be monitored with a multidisciplinary approach, and if necessary, in the burn intensive care unit. Primary treatment is supportive care. Early initiation of cyclosporine may have a positive effect on the prognosis in patients with SJS/TEN overlap and TEN.