PA27 Long-term complications of toxic epidermal necrolysis in children

Abstract The mortality of toxic epidermal necrolysis (TEN) and Steven–Johnsons syndrome (SJS) appears to be lower in children than in adults; however, children may develop significant long-term sequelae. Here we describe a child with multiorgan complications requiring long-term care from a range of specialists. Our patient, now age 13 years, was diagnosed with TEN at age 7 years. He presented with approximately 60% body surface area involvement thought to be secondary to an infectious trigger. He was transferred to a burns unit, where he was treated with intensive wound care management, full supportive care and intravenous immunoglobulin, and underwent skin debridement on two occasions. Over the following 6 years, he has developed a myriad of long-term complications. Most notably he has had chronic ocular involvement with persistent conjunctival inflammation, keratoconjunctivitis sicca and corneal opacification requiring cataract surgery. He has experienced persistent xerostomia along with dental growth abnormalities including arrested root development of the majority of his permanent teeth. Immediately following the acute episode, he experienced onychomadesis of all nails; these subsequently grew back with pterygium formation and longitudinal ridging. He has postinflammatory hypopigmentation of the skin and hypertrophic scars. He has had chronic fatigue requiring long school absences. In addition, he has developed significant otological complications; he was diagnosed with left external ear canal stenosis and conductive hearing loss at the age of 11 years. He is being considered for a bone-anchored hearing aid. Two years following the episode of TEN he was diagnosed with Hashimoto thyroiditis. This case illustrates the breadth of sequelae that can be seen in the longer term following TEN/SJS in children, including the rarer otological and possible autoimmune complications (Hotaling JM, Hotaling AJ. Otologic complications of Stevens–Johnson syndrome and toxic epidermal necrolysis. Int J Pediatr Otorhinolaryngol 2014; 78: 1408–9). Although there is no established relationship between SJS/TEN and the development of autoimmune disease, a review of the literature reveals case reports of later-onset SLE, Sjögren syndrome and Hashimoto thyroiditis after TEN/SJS (Couissi I, Douhi Z, Mejjati K et al. Autoimmune diseases a late complication of toxic epidermal necrolysis: a case report. J Dermatol Res 2023; 4: 1–4). As recommended in the 2019 British Association of Dermatologists guidelines for SJS and TEN in children, parents and children should be counselled on possible long-term sequelae and require long-term follow-up with an appropriate specialist able to coordinate multidisciplinary care.