Chi Le Quynh, Anh Nguyen Thi Van, Le Nguyen Ngoc Quynh, Ha Nguyen Thi Thu, Tien Hoang Minh, Dien Tran Minh
Department of Rheumatology, Allergy, and Immunology, Vietnam National Children's Hospital, Hanoi, Vietnam.
Surgical Intensive Care Unit, Vietnam National Children's Hospital, Hanoi, Vietnam.
Front Pediatr. 2022 Apr 13;10:834037. doi: 10.3389/fped.2022.834037. eCollection 2022.
Stevens-Johnson syndrome/toxic epidermal necrolysis (SJS/TEN) is a rare and life-threatening disease of the skin and mucosal surfaces. Although gastrointestinal manifestations in adults are potential prognostic factors for disease severity, there are limited data on such cases and their standard management in the pediatric population.
We herein report the case of an 8-year-old girl with a 1-year history of epilepsy, who presented with bilateral conjunctivitis and progressively widespread bullous, and pruritic eruption based on erythematous skin after administration of carbamazepine. A diagnosis of carbamazepine-induced TEN was made, and the drug was immediately discontinued. The result of genetic screening showed that the patient was positive for the HLA-B15:02 allele. Then, her condition got worse by developing gastrointestinal involvement, including hematemesis and severe watery bloody diarrhea. A combination of the intravenous immunoglobulin and the appropriate dose of systemic steroids have contributed to a favorable outcome in this case. Multidisciplinary care of mucocutaneous involvement, supplemental nutrition, and fluid replacement was also critically warranted. This report aims to contribute to the current literature on TEN-related gastrointestinal manifestations in pediatrics and highlights the need for further investigations in determining the optimal treatment in such cases.
In conclusion, we reported the successful treatment of TEN-related gastrointestinal manifestations in a pediatric patient, which should be critically considered in patients with SJS/TEN. Since it may significantly contribute to the poor prognosis of the illness, further investigations in determining standard management in such cases are necessary.
史蒂文斯-约翰逊综合征/中毒性表皮坏死松解症（SJS/TEN）是一种罕见且危及生命的皮肤和黏膜疾病。尽管成人的胃肠道表现是疾病严重程度的潜在预后因素，但关于儿科患者此类病例及其标准治疗的数据有限。
我们在此报告一名8岁癫痫女童的病例，该女童在服用卡马西平后出现双侧结膜炎，以及基于红斑皮肤的进行性广泛水疱和瘙痒性皮疹。诊断为卡马西平诱发的TEN，立即停用该药物。基因筛查结果显示患者HLA - B15:02等位基因呈阳性。随后，她因出现胃肠道受累情况而病情恶化，包括呕血和严重的水样血性腹泻。静脉注射免疫球蛋白和适当剂量的全身类固醇联合使用促成了该病例的良好结局。对皮肤黏膜受累情况的多学科护理、补充营养和液体替代也至关重要。本报告旨在为当前有关儿科TEN相关胃肠道表现的文献做出贡献，并强调在确定此类病例的最佳治疗方法方面需要进一步研究。
总之，我们报告了一名儿科患者TEN相关胃肠道表现的成功治疗案例，SJS/TEN患者应予以高度重视。由于它可能显著导致疾病预后不良，因此有必要进一步研究确定此类病例的标准治疗方法。
