Güvenir Funda Aytekin, Çayhan Vildan Selin, Balci Selman Kürşat, Dere Ragıp, Çelik Hatice Irmak, Emeksiz Serhat, Selmanoğlu Ahmet, Emeksiz Zeynep Şengül, Şenel Emrah, Misirlioğlu Emine Dibek
Division of Pediatric Allergy and Immunology, Department of Pediatrics, Ankara Bilkent City Hospital, Ankara, Turkiye.
Department of Pediatric Surgery, Ankara Bilkent City Hospital, Ankara, Turkiye.
Turk J Med Sci. 2025 Feb 5;55(2):461-469. doi: 10.55730/1300-0144.5990. eCollection 2025.
BACKGROUND/AIM: Steven Johnson Syndrome (SJS) and Toxic Epidermal Necrolysis (TEN) are rare but life-threatening severe cutaneous drug hypersensitivity reactions. The aim of our study was to evaluate the clinical features of pediatric patients diagnosed with SJS, TEN or SJS/TEN overlap and to examine treatment modalities.
Patients aged 0-18 years who were followed up with SJS, TEN or SJS/TEN overlap at Ankara Bilkent City Hospital between August 2019 and January 2024 were retrospectively analyzed.
Twelve patients who met the inclusion criteria were included in the study. Five of the patients had SJS, four had SJS/TEN overlap, and three had TEN. Eight of the patients were female, and the median age at presentation was 10.5 (IQR:6-16) years. Ten of the patients had a history of drug use. Eight patients had antibiotics, two had proton pump inhibitors, two had allopurinol, and two had antiepileptic (lamotrigine and valproic acid) use. All 12 patients received IVIG and systemic steroid therapy. Three TEN and four SJS/TEN overlap patients received cyclosporine. Two TEN patients underwent plasmapheresis. The most common long-term sequelae were dermatological sequelae.
SJS/TEN patients should be monitored with a multidisciplinary approach, and if necessary, in the burn intensive care unit. Primary treatment is supportive care. Early initiation of cyclosporine may have a positive effect on the prognosis in patients with SJS/TEN overlap and TEN.
背景/目的：史蒂文斯-约翰逊综合征（SJS）和中毒性表皮坏死松解症（TEN）是罕见但危及生命的严重皮肤药物过敏反应。我们研究的目的是评估诊断为SJS、TEN或SJS/TEN重叠的儿科患者的临床特征，并研究治疗方式。
回顾性分析2019年8月至2024年1月在安卡拉比尔肯特市医院接受SJS、TEN或SJS/TEN重叠随访的0至18岁患者。
12名符合纳入标准的患者被纳入研究。其中5例为SJS，4例为SJS/TEN重叠，3例为TEN。8例患者为女性，就诊时的中位年龄为10.5（四分位间距：6 - 16）岁。10例患者有用药史。8例患者使用过抗生素，2例使用过质子泵抑制剂，2例使用过别嘌醇，2例使用过抗癫痫药物（拉莫三嗪和丙戊酸）。所有12例患者均接受了静脉注射免疫球蛋白（IVIG）和全身类固醇治疗。3例TEN患者和4例SJS/TEN重叠患者接受了环孢素治疗。2例TEN患者接受了血浆置换。最常见的长期后遗症是皮肤后遗症。
SJS/TEN患者应采用多学科方法进行监测，必要时在烧伤重症监护病房进行监测。主要治疗方法是支持性护理。早期使用环孢素可能对SJS/TEN重叠和TEN患者的预后产生积极影响。
