Bronchiolitis obliterans as a long‐term sequela of Stevens–Johnson syndrome and toxic epidermal necrolysis in children

Abstract

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

British Association of Dermatologists' guidelines for the management of Stevens–Johnson syndrome/toxic epidermal necrolysis in children and young people, 2018

“…This can occur at any stage of the illness, including after discharge, and respiratory function should be expertly monitored. 60,61 Psychological complications are now well recognized in adults and include post-traumatic stress disorder and fear of taking medication. [62][63][64] In children, further work on psychosocial implications is needed to assess both short-and longer-term impact and the potential impact of long-term cosmetic consequences.…”

“…The respiratory complication bronchiolitis obliterans can be severe in children. This can occur at any stage of the illness, including after discharge, and respiratory function should be expertly monitored . Psychological complications are now well recognized in adults and include post‐traumatic stress disorder and fear of taking medication .…”

Rare case of Stevens-Johnson syndrome with bronchiolitis obliterans as a chronic complication

“…The time to onset of respiratory symptoms following initial presentation with SJS/TEN varies from case to case and ranges from 5 days to 5 months 13. Therefore, some patients may develop BO much later than the onset of SJS.…”

Pancytopenia in a Patient with Stevens-Johnson Syndrome: A Case Report with Literature Review

“…All mucous membranes have a tendency of being involved, with buccal, ocular, and genital mucosas more commonly while respiratory and gastrointestinal mucosas in rare instances. The most common early complications include superficial skin infections, sepsis, and pneumonia while long-term sequelae are categorized into skin (pruritis, hypertrophic scars), ocular (dry watery eyes, decreased visual acuity) and organ-specific (vaginal/labial adhesions, bronchiolitis obliterans) (Table 1) [2, 7, 10].…”