Chatproedprai Susheera, Wutticharoenwong Vanvara, Tempark Therdpong, Wananukul Siriwan
Department of Pediatrics, Faculty of Medicine, Chulalongkorn University and King Chulalongkorn Memorial Hospital, Bangkok 10330, Thailand.
Dermatol Res Pract. 2018 May 7;2018:3061084. doi: 10.1155/2018/3061084. eCollection 2018.
To determine the probable causative factors, clinical features, and treatment outcomes of Stevens-Johnson syndrome (SJS), toxic epidermal necrolysis (TEN), and SJS-TEN overlap in children.
A 20-year database review of all children diagnosed with SJS/TEN/SJS-TEN overlap at the King Chulalongkorn Memorial Hospital, Thailand.
36 patients (M : F, 16 : 20) with the mean age of 9.2 ± 4.0 years were identified. There were 20 cases of SJS, 4 cases of SJS-TEN overlap, and 12 cases of TEN. Drugs were the leading cause for the diseases (72.3%); antiepileptics were the most common culprits (36.1%). Cutaneous morphology at presentation was morbilliform rash (83.3%), blister (38.9%), targetoid lesions (25.0%), and purpuric macules (2.8%). Oral mucosa (97.2%) and eye (83.3%) were the 2 most common mucosal involvements. Majority of the cases (77.8%) were treated with systemic corticosteroids, intravenous immunoglobulin, or both. Treatment outcomes between those who received systemic therapy and those who received only supportive care were comparable. Skin and eye were the principal sites of short-term and long-term complications.
SJS/TEN are not common but are serious diseases which lead to significant morbidities in children. Early withdrawal of suspicious causes and meticulous supportive care are very important. This study found that the systemic therapy was not superior to supportive care because the treatment outcomes for both groups were comparable.
确定儿童史蒂文斯-约翰逊综合征(SJS)、中毒性表皮坏死松解症(TEN)及SJS-TEN重叠综合征的可能致病因素、临床特征及治疗结果。
对泰国朱拉隆功国王纪念医院20年间所有诊断为SJS/TEN/SJS-TEN重叠综合征的儿童病例进行数据库回顾。
共确定36例患者(男∶女 = 16∶20),平均年龄9.2±4.0岁。其中SJS 20例,SJS-TEN重叠综合征4例,TEN 12例。药物是这些疾病的主要病因(72.3%);抗癫痫药是最常见的致病药物(36.1%)。发病时的皮肤形态为麻疹样皮疹(83.3%)、水疱(38.9%)、靶形损害(25.0%)及紫癜性斑疹(2.8%)。口腔黏膜(97.2%)和眼(83.3%)是最常见的两个黏膜受累部位。大多数病例(77.8%)接受了全身用糖皮质激素、静脉注射免疫球蛋白或两者联合治疗。接受全身治疗的患者与仅接受支持治疗的患者的治疗结果相当。皮肤和眼是短期及长期并发症的主要发生部位。
SJS/TEN并不常见,但却是导致儿童出现严重发病情况的严重疾病。尽早停用可疑病因并进行细致的支持治疗非常重要。本研究发现全身治疗并不优于支持治疗,因为两组的治疗结果相当。