关键词: Stevens-Johnson综合征, 中毒性表皮坏死松解症, 疾病特征

Abstract: Objective To analyze the etiology, clinical characteristics, treatment management, complications, and prognosis of children with stevens-Johnson syndrome(SJS), toxic epidermal necrolysis(TEN) and SJS/TEN. 
Methods Clinical data of 32 children with SJS, 28 children with SJS/TEN and 25 children with TEN were retrospectively reviewed. 
Results The most common pathogenic drugs were antibiotics(31.7%), proprietary Chinese medicines（30.6%）， non-steroidal anti-inflammatory drugs(NSAIDs)(17.6%) and anticonvulsants(14.1%). Mycoplasma pneumoniae(21cases, 24.7%) was the most common pathogen.In addition, 32(37.6%) patients experienced at least one complication, and the most common complication was infection in 26 cases(30.6%), including 12(14.1%) children with skin infection and 8(9.4%) children with lower respiratory tract infection. During hospitalization period, 8 patients received immunoglobulin therapy alone, 35 patients received intravenous glucocorticoid therapy alone, and 37 children received both simultaneously. The mortality rate of the intravenous immunoslobulin combined hormone group was the lowest; 8 patients died, and mortality rate was 9.4%. The severity-of-illness score for toxic epidermal necrolysis(SCORTEN) score of the deceased was higher than that of the survivor on the first day of admission(P＜0.05). 
Conclusion Antibiotics and proprietary Chinese medicines are the most  common pathogenic drugs, and skin infection is the most common complication. The mortality risk will increase when SCORTEN score＞3 points, therefore, systematic therapy of glucocorticoids and early administration of intravenous immunoglobulin may be an effective means to treat SJS and TEN.

Key words: Stevens-Johnson syndrome, toxic epidermal necrolysis, disease attributes