儿童重症多形性红斑19例

Clinical analysis of 19 children with erythema multiforme major

目的 探讨儿童重症多形性红斑(EMM)的临床特征,提高临床诊治水平。方法 回顾分析19 例 EMM的临床特征及治疗。结果 100%的病例均有发热、皮疹及黏膜损害,伴有不同程度的内脏器官受累和免疫学指标异常。94.7%的病例早期给予糖皮质激素治疗,57.9%的病例同时给予丙种球蛋白静滴(IVIG)。临床症状迅速缓解,实验室指标大都在 6 周内完全恢复正常。伴有肺部受累的6例,随访期间,3例恢复正常,2例病情缓解,1例3个月后死于肺间质纤维化。结论 EMM是儿童急重症之一,肺部并发症严重威胁儿童健康甚至生命,早期糖皮质激素及 IVIG治疗有助于降低死亡率及重要内脏器官并发症的发生。

Objective To investigate the clinical characteristic and treatment of children with erythema multiforme major(EMM),and to improve the diagnosis and treatment ability clinically.Methods The clinical feature, diagnosis and treatment of 19 children with EMM were summarized and analyzed.Results All cases had fever, skin eruption, mucosa lesion, with different degree organs involvement and immunological abnormality.94.7% patients treated with glucocorticoid in the earlier period,57.9% patients received intravenous immunoglobulin (IVIG) treatment meanwhile. Clinical symptoms quickly relieved, laboratory markers almost completely recovered in 6 weeks. In the follow-up period, among 6 cases accompanied with lung involvement, 3 cases recovered, 2 cases relieved, 1 case died of pulmonary interstitial fibrosis after 3 months.Conclusion EMM is one of the emergencies in children, pulmonary complication severely endangers children's health even life, glucocorticoid and IVIG treatment in the earlier period can reduce mortality and important organs complication.