Case Report: Stevens-Johnson syndrome/toxic epidermal necrolysis overlap with ocular and urogenital involvement in an extremely preterm infant following broad-spectrum antibiotic exposure.

Stevens-Johnson syndrome/toxic epidermal necrolysis (SJS/TEN) is a rare, life-threatening epidermolytic reaction characterized by widespread keratinocyte necrosis, epidermal detachment, and mucosal involvement. In neonates, particularly extremely premature infants, diagnosis is challenging because several mimickers are more common in the neonatal intensive care unit (NICU), and evidence guiding systemic therapy is limited. We report a male infant born at 23 + 5 weeks' gestation (birth weight 620 g) who developed progressive mucocutaneous erosions and flaccid bullae at a corrected gestational age of 35 + 3 weeks (day of life [DOL] 83, weight 1800 g). The eruption began periorally and spread to the trunk, extremities, and genitals, with ocular and urethral involvement. Epidermal detachment was estimated at 25-30% body surface area (BSA), consistent with SJS/TEN overlap. Skin biopsy demonstrated widespread full-thickness keratinocyte necrosis with sub-epidermal blistering and sparse lymphocytic inflammation; direct immunofluorescence was negative. Following protocolized supportive care and adjunctive intravenous immunoglobulin (IVIG), cutaneous progression halted with re-epithelialization. The infant later died from septic shock with multi-organ failure related to extreme prematurity and recurrent necrotizing enterocolitis (NEC). This case highlights the diagnostic complexity of epidermolytic syndromes in extremely premature infants and supports early suspicion, prompt withdrawal of potential triggers, and protocolized multidisciplinary supportive care.