Department of Pediatrics, Divisions of Emergency Medicine, Hospital for Sick Children, Toronto, Ontario, Canada.
Pediatrics. 2011 Oct;128(4):723-8. doi: 10.1542/peds.2010-3322. Epub 2011 Sep 2.
To report clinical course, etiology, management, and long-term outcomes of children suffering from Stevens-Johnson syndrome (SJS) or toxic epidermal necrolysis (TEN).
We conducted a study of all pediatric patients with SJS or TEN admitted between 2000 and 2007 to the Hospital for Sick Children and Children's Hospital Boston, and particular attention was paid to clinical manifestations, etiology, mortality, and long-term outcomes.
We identified 55 cases of SJS (n = 47), TEN (n = 5), or SJS/TEN overlap syndrome (n = 3). Drugs were identified as the most likely etiologic agent in 29 children (53%); antiepileptic drugs were the most common agents (n = 16), followed by sulfonamide antibiotics (n = 7) and chemotherapy drugs (n = 2). Acute Mycoplasma pneumoniae infection was confirmed in 12 children (22%), and herpes simplex virus was confirmed in 5 children (9%). Treatment regimens differed significantly between participating sites and included systemic antimicrobial agents (67%), systemic corticosteroids (40%), and antiviral drugs (31%). Intravenous immunoglobulin was administered to 21 children (38%), of whom 8 received concomitant systemic corticosteroids. Ten children (18%) had recurrence of SJS up to 7 years after the index episode, and 3 experienced multiple recurrences. Twenty-six children (47%) suffered long-term sequelae that mostly involved the skin and eyes.
Mortality rate in children was lower than that reported in adults, but half of affected children suffered long-term complications. The recurrence rate of SJS was high (1 in 5), which suggests vulnerability and potential genetic predisposition. In the absence of standardized management guidelines for these conditions, treatment regimens differed significantly between participating institutions.
报告儿童史蒂文斯-约翰逊综合征（SJS）或中毒性表皮坏死松解症（TEN）的临床过程、病因、治疗方法和长期预后。
我们对 2000 年至 2007 年间在多伦多 SickKids 医院和波士顿儿童医院住院的所有儿童 SJS 或 TEN 患者进行了研究，特别关注临床表现、病因、死亡率和长期预后。
我们共发现 55 例 SJS（n=47）、TEN（n=5）或 SJS/TEN 重叠综合征（n=3）患者。在 29 例儿童（53%）中发现药物是最可能的病因，其中抗癫痫药物最常见（n=16），其次是磺胺类抗生素（n=7）和化疗药物（n=2）。12 例儿童（22%）被证实存在急性肺炎支原体感染，5 例儿童（9%）被证实存在单纯疱疹病毒感染。参与研究的不同机构之间的治疗方案存在显著差异，包括全身抗菌药物（67%）、全身皮质类固醇（40%）和抗病毒药物（31%）。21 例儿童（38%）接受了静脉注射免疫球蛋白治疗，其中 8 例同时接受全身皮质类固醇治疗。10 例儿童（18%）在指数发作后长达 7 年出现 SJS 复发，其中 3 例出现多次复发。26 例儿童（47%）患有长期后遗症，主要涉及皮肤和眼睛。
儿童死亡率低于成人报道的死亡率，但一半以上的患儿有长期并发症。SJS 的复发率较高（1/5），提示存在易感性和潜在的遗传易感性。由于这些疾病缺乏标准化的管理指南，参与机构之间的治疗方案存在显著差异。
