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目的:探讨渗出性多形性红斑(重症)的临床表现特点及治疗方法。方法:收集西安市儿童医院(西安交通大学医学院附属儿童医院)风湿免疫科2016年1月至2019年6月收治的渗出性多形性红斑(重症)患儿30例的临床资料进行回顾性分析。结果:30例患儿男18例，女12例，男女比例约3∶2。发病年龄(7.57±2.48)岁，其中最大13岁，最小2岁。此30例患儿符合渗出性多形性红斑(重症)诊断条件。其中可疑致敏药物包括：抗菌药物15例，占50%；抗癫痫药8例(苯巴比妥4例、卡马西平2例、丙戊酸钠2例)，占26.7%；非甾体抗炎药5例(布洛芬4例、阿司匹林2例)，占16.7%；无明确致敏药物2例，占6%。30例患儿均有口腔黏膜及眼结膜受累，并发角膜溃疡1例；外阴及生殖器受累10例。30例均给予甲泼尼龙琥珀酸钠＋静脉用丙种球蛋白冲击治疗，其中3例因脏器功能明显受损，病情进展快行血液净化治疗。4例发展为中毒性表皮坏死松解症，其中3例加用环孢素治疗。1例留有神经系统后遗症，表现为意识障碍及肢体运动障碍，行康复治疗2个月后恢复。结论:对于渗出性多形性红斑(重症)多数患儿合并肝脏功能异常，最易引起致敏的药物为抗菌药物及抗癫痫药物，在早期识别后，尽早使用大剂量糖皮质激素加静脉用丙种球蛋白冲击治疗，大多数预后良好，如效果不佳时可加用其他免疫抑制剂如环孢素等、生物制剂及血液净化等。“,”Objective:To analyze the clinical characteristics and treatment of Stevens-Johnson syndrome.Methods:The clinical data of 30 children with Stevens-Johnson syndrome admitted to our hospital in recent 3 years(January 2016-June 2019) were retrospectively analyzed.Results:Among the 30 cases, 18 cases were male and 12 cases were female, the ratio of male to female was 3: 2.The average age of onset was (7.57±2.48)years, with the oldest age 13years and the youngest age was 2years.The 30 patients met the diagnostic criteria for Stevens-Johnson syndrome.Among the suspected allergen, there were 15 cases of antibiotics, accounting for 50%.There were 8 cases of antiepileptic drugs(4 cases of phenobarbital, 2 cases of carbamazepine and 2 cases of valproic acid), accounting for 26.7%.Five cases of NSAIDS (4 cases of ibuprofen and 2 cases of aspirin), accounted for 16.7%.Two cases had no specific sensitizing drugs, accounted for 6%.All 30 patients had oral mucosa and conjunctiva involvement, corneal ulcer complicated in 1 case.Vulva and genital were involved in 10 cases.All 30 cases received implosive therapy with methylprednisolone sodium succinate + intravenous gamma globulin, among which 3 cases were treated with plasma exchange due to obvious impairment of organ function.Four cases developed toxic epidermal necrolysis, three of which were treated with cyclosporine.One patient had neurological sequelae, manifested as consciousness disorder and limb movement disorder, and recovered after 2 months of rehabilitation treatment.Conclusion:For children with Stevens-Johnson syndrome, most of the children with abnormal liver function.The antiepileptic drugs and antibacterial drugs are the most susceptible drugs.After early identification, early use of high-dose glucocorticoid plus intravenous gamma globulin shock treatment, the prognosis of most patients is good.When the effect is not good, other immune inhibitors such as ciclosporin, biological agents and blood purification, etc.can also be used.