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儿童史蒂文斯-约翰逊综合征和中毒性表皮坏死松解症的闭塞性细支气管炎作为一种长期后遗症。

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

机构信息

Department of Dermatology, University Hospital Southampton NHS Foundation Trust, Southampton, Hampshire, UK.

Paediatric Respiratory Department, University Hospital Southampton NHS Foundation Trust, Southampton, Hampshire, UK.

出版信息

Clin Exp Dermatol. 2019 Dec;44(8):897-902. doi: 10.1111/ced.13969. Epub 2019 Jul 17.

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.

摘要

中毒性表皮坏死松解症 (TEN) 和史蒂文斯-约翰逊综合征 (SJS) 的特征为广泛的皮肤和黏膜水疱和坏死。儿童的诱因和长期后遗症可能与成人报道的不同。细支气管炎闭塞性 (BO) 是一种罕见的并发症,仅有 15 例先前报道,但可导致显著的长期发病率,在某些情况下需要进行肺移植。我们报告了 3 例非药物相关性 SJS(n=1)和 TEN(n=2)患儿发生 BO。2 例接受了静脉注射免疫球蛋白治疗(2-2.4g/kg),所有 3 例患儿均存活。我们从我们的病例中强调了 salient 学习要点和 BO 诊断中的潜在陷阱,包括迟发性,并回顾了文献。

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