Objective: To 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. Methods: The 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. Results: A 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. Conclusion: The synthetic corticosteroid deflazacort can cause rare but severe adverse reactions such as SJS-TEN, which needs close monitoring and prompt recognition and management.
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。.