Department of Dermatology, The University of Texas Medical Branch, Galveston, TX, USA.
Burn Surgery, The University of Texas Medical Branch, Galveston, TX, USA.
Pediatr Dermatol. 2021 Jul;38(4):812-818. doi: 10.1111/pde.14598. Epub 2021 May 31.
Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis Syndrome (TEN) are rare immune-mediated diseases. Extensive research on adult triggers of SJS and TEN is available; however, research in children is more limited.
We sought to investigate and report the experience with pediatric SJS and TEN in our center, identifying associated medications.
A retrospective review from 1990 to 2015 at the Shriner's Burn Hospital in Galveston, Texas was performed to identify patients diagnosed with SJS, SJS/TEN overlap, and TEN. Data pertaining to demographic characteristics, medical history, physical exam, treatment, and outcomes were collected.
We identified SJS/TEN overlap or TEN in 51 patients. Antiepileptic drugs were the most common group of causative agents, closely followed by antibiotics. The most common causative agents were trimethoprim-sulfamethoxazole, phenytoin, and lamotrigine used concomitantly with valproic acid. Newer generation agents, with the definition of agents approved after 1990, were the cause in 13/51 (25.5%) cases. Newer generation agents included lamotrigine, clobazam, and zonisamide. Seven patients died, resulting in a 13.7% mortality rate. Renal failure, liver failure, sepsis, and gastrointestinal involvement each had a statistically significant association with mortality. SCORTEN was statistically significantly greater in patients who died compared to children who lived (3 vs 2).
This is a retrospective study.
Three drugs introduced into the market since 1990 have emerged as causes of SJS/TEN overlap and TEN: lamotrigine, clobazam, and zonisamide. These medications are being used more widely to treat seizures, as well as mood disorders. It is also important for clinicians to be aware of the extremely commonly used medications such as amoxicillin, tetracyclines, NSAIDs, and acetaminophen that can rarely cause SJS and TEN.
史蒂文斯-约翰逊综合征（SJS）和中毒性表皮坏死松解症（TEN）是罕见的免疫介导性疾病。大量研究已经探讨了成人 SJS 和 TEN 的发病诱因，然而，儿童相关研究较为有限。
我们旨在调查和报告我们中心儿童 SJS 和 TEN 的发病经验，明确相关的诱发药物。
对 1990 年至 2015 年在德克萨斯州加尔维斯顿 Shriners 烧伤医院的患者进行回顾性研究，以确定 SJS、SJS/TEN 重叠和 TEN 患者。收集患者的人口统计学特征、病史、体格检查、治疗和结局数据。
我们共发现 51 例 SJS/TEN 重叠或 TEN 患者。抗癫痫药物是最常见的致病药物，其次是抗生素。最常见的致病药物是与丙戊酸合用的甲氧苄啶-磺胺甲噁唑、苯妥英和拉莫三嗪。新型药物，定义为 1990 年后批准的药物，在 51 例患者中的 13 例（25.5%）中为病因。新型药物包括拉莫三嗪、氯巴占和佐米曲坦。7 例患者死亡，死亡率为 13.7%。肾衰竭、肝衰竭、脓毒症和胃肠道受累与死亡率均有统计学显著关联。死亡患者的 SCORTEN 评分明显高于存活患者（3 分 vs 2 分）。
这是一项回顾性研究。
自 1990 年以来，有 3 种药物已成为 SJS/TEN 重叠和 TEN 的致病原因：拉莫三嗪、氯巴占和佐米曲坦。这些药物被广泛用于治疗癫痫和情绪障碍。临床医生还应意识到，阿莫西林、四环素、非甾体抗炎药和对乙酰氨基酚等常用药物也可能罕见地引起 SJS 和 TEN。
