Toxic epidermal necrolysis (TEN) and Stevens-Johnson syndrome (SJS) are characterized by widespread skin and mucosal blistering and necrosis. The triggers and long-term sequelae in children may differ from those reported for adults. Bronchiolitis obliterans (BO) is an uncommon complication, with only 15 previously reported cases, but can lead to significant long-term morbidity, requiring lung transplantation in some cases. We report three children with nondrug-related SJS (n = 1) and TEN (n = 2) who developed BO. Two were treated with intravenous immunoglobulin therapy (2-2.4 g/kg) and all three survived. We highlight salient learning points from our cases and potential pitfalls in diagnosis of BO, including delayed onset, and we also review the literature.Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are rare, potentially life-threatening conditions, characterized by widespread skin and mucosal necrosis. SJS is defined as < 10% body surface area (BSA) detachment, SJS-TEN overlap as 10-30% BSA detachment and TEN as > 30% detachment. 1 The reported incidence is lower in children than adults: 5.3 vs. 9.3, 0.8 vs. 1.9 and 0.4 vs. 1.6 cases per million per year for SJS, SJS-TEN and TEN, respectively, in the USA. 2,3 Medications are usually culpable, although infections may be implicated, particularly in children. 4 Mortality in childhood is reportedly 0% (SJS), 4% (SJS-TEN) and 16% (TEN), 2 with deaths most frequently due to sepsis and organ failure. 2 However, similarly to adults, increased mortality is noted with higher degrees of epidermal detachment: 60-70% BSA involvement was associated with 37.5% mortality in one study. 5 Intravenous immunoglobulin (IVIG), ciclosporin and/or systemic corticosteroids have been used despite the relative lack of evidence, as summarized in recent guidelines. 6 Bronchiolitis obliterans (BO) is a rarely reported complication, but when present, is associated with significant long-term morbidity. We report three children with nondrug-related SJS/TEN who developed BO. ReportPatient 1 was a previously well 5-year-old boy who presented with a 2-day history of an erythematous maculopapular rash in association with fever and coryza. Overall, the skin changes involved 95% BSA, with 40% epidermal detachment. There were additional bilateral conjunctival defects with confluent superficial punctate epithelial erosions of the cornea. He had severe oral and upper airway ulceration with sloughing. Subsequent development of stridor necessitated intubation.Histological examination of a skin biopsy confirmed TEN, with negative direct immunofluorescence. Chest radiography was unremarkable. Bronchoscopy showed ulceration at the base of insertion of the endotracheal

Background/Purpose: To examine the efficacy, tolerability and safety of phototherapy in children, in whom there is currently a paucity of data. Materials and Methods: Retrospective review of children under 18 years who received narrowband UVB (NB-UVB), broadband UVB (BB-UVB) phototherapy or psoralen with UVA (PUVA) photochemotherapy between 2003 and 2017 at a tertiary Paediatric dermatology centre in Southampton, UK. Results: 100 children aged 6-17 years were included. The majority of children had psoriasis (74), atopic dermatitis (10) or vitiligo (8), with others having rarer dermatoses. Grade 2 erythema or above occurred in 46% of all included children and 42% (36/86) of those receiving NB-UVB; however, grade 3 and 4 reactions were infrequent and only 3 children stopped treatment due to burning. NB-UVB was particularly effective in those with psoriasis; 55/65 (85%) significantly improved, and 72% had not relapsed after 2 years. However, its effectiveness in atopic dermatitis was less convincing; in a small group of children, 6/10 (60%) significantly improved, but 66% relapsed within 3 months. Conclusions: Our analysis demonstrates that NB-UVB is effective in children with psoriasis and vitiligo, with potential to achieve extended periods of remission in psoriasis. Its usefulness in atopic dermatitis is less clear. The long-term safety of NB-UVB in children is still unknown, but it appears to be a well-tolerated treatment and should be considered in children for a variety of inflammatory dermatoses before progressing to immunosuppressive therapies.

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