地夫可特相关Steven-Johnson综合征1例并文献复习  

Deflazacort-induced Steven-Johnson syndrome:a case report and literature review

作　　者：李崇巍[1] 孙楠[1] 李少宁 张玉慈 马继军[1] Li Chongwei;Sun Nan;Li Shaoning;Zhang Yuci;Ma Jijun(Department of Rheumatology&Clinical Immunology,Tianjin Children′s Hospital,Tianjin 300134,China)

机构地区：[1]天津市儿童医院风湿免疫科,天津300134

出　　处：《中华儿科杂志》2024年第11期1103-1107,共5页Chinese Journal of Pediatrics

基　　金：天津市医学重点学科(专科)建设项目(TJYXZDXK-040A)。

摘　　要：目的总结糖皮质激素类药物地夫可特相关Steven-Johnson综合征(SJS)-中毒性表皮坏死松解症(TEN)的发病情况和转归。方法回顾性分析2024年7月天津市儿童医院免疫科收治1例杜氏肌营养不良(DMD)患儿在地夫可特治疗过程中发生SJS的临床资料。以"地夫可特""Steven-Johnson综合征""中毒性表皮坏死松解症""deflazacort""Steven-Johnson syndrome""toxic epidermal necrolysis"为关键词,分别在中国知网、万方数据库、中国生物医学文献数据库、PubMed数据库进行检索(建库至2024年7月)。结合本例资料,总结地夫可特相关SJS-TEN的临床特征、治疗及转归。结果患儿男,12岁,因"皮疹3 d"入院。3岁确诊DMD,8岁接受泼尼松龙治疗,入院前44 d以地夫可特替换泼尼松龙,入院前3 d出现进行性加重的红色斑丘疹、水疱和表皮剥脱(约占体表面积8%)、口腔黏膜糜烂和渗出性结膜炎。停用地夫可特,予静脉注射免疫球蛋白(总计1.4 g/kg)、2剂依那西普[0.9 mg/(kg·次)]和甲泼尼龙[0.7 mg/(kg·d)]治疗后,皮损全部愈合。文献复习符合检索条件中文文献0篇,英文文献5篇,结合本例患儿共7例,均为男性,年龄3~45岁,地夫可特暴露时间为2~8周。本例皮损为SJS,5例为TEN,1例为渗出性多形红斑(地夫可特口服激发试验阳性)。治疗使用甲泼尼龙或地塞米松有5例、静脉注射免疫球蛋白6例、依那西普3例和环孢素1例,所有患者的皮损均完全恢复。结论地夫可特可以造成罕见且严重的药物不良反应SJS-TEN,须密切监测并及时识别和处理。ObjectiveTo summarize the clinical features and outcomes of deflazacort-induced Steven Johnson syndrome(SJS)-toxic epidermal necrolysis(TEN)to raise awareness among patients with Duchenne muscular dystrophy(DMD),neurologists as well as other deflazacort users.MethodsThe clinical data of a boy with DMD who had SJS induced by deflazacort treated at the Department of Rheumatology&Clinical Immunology of Tianjin Children′s Hospital in July 2024 was analyzed retrospectively.Taking"deflazacort""Steven-Johnson syndrome""toxic epidermal necrolysis"in Chinese or English as the keywords,literature was searched at CNKI,Wanfang,China Biomedical Literature Database and PubMed up to July 2024.The clinical characteristics,treatment and outcomes of deflazacort-induced SJS-TEN were summarized.ResultsA 12-year-old boy was admitted with a 3-day history of rash.He was diagnosed with DMD at the age of 3 and had been treated with prednisolone since the age of 8.Forty-four days before admission,the patient started deflazacort to replace prednisolone.Three days before admission,progressively worsening erythematous maculopapular rashes,blisters and skin peeling(8%body surface area),oral mucosal erosion,and exudative conjunctivitis occurred,thus deflazacort was discontinued.Complete remission of SJS was achieved after treatment with intravenous immunoglobulin(IVIG,total 1.4 g/kg),2 doses of etanercept(0.9 mg/kg,once),subcutaneous injection and intravenous methylprednisolone(0.7 mg/(kg·d)).Based on the literature,there were 5 reports in English while none in Chinese,altogether 7 cases were reported.All the patients were male,aged 3-45 years.Duration of deflazacort exposure was 2-8 weeks.Dermatology diagnosis of our case was SJS,and 5 cases were TEN.One patient was diagnosed with exudative erythema multiforme,and subsequent deflazacort oral challenge test was positive.Treatment included methylprednisolone or dexamethasone in 5 cases,IVIG in 6 cases,etanercept in 3 cases and cyclosporine in 1 case.All patients recovered completely.Conclusi

关 键 词：STEVENS-JOHNSON综合征 地夫可特 表皮坏死松解症 中毒性 肌营养不良 杜氏 

分 类 号：R725.9[医药卫生—儿科]