Lam Nga-Shuen, Yang Yao-Hsu, Wang Li-Chieh, Lin Yu-Tsan, Chiang Bor-Luen
Department of Pediatrics, National Taiwan University Hospital, Taipei, Taiwan, ROC.
J Microbiol Immunol Infect. 2004 Dec;37(6):366-70.
Erythema multiforme (EM), Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are mucocutaneous diseases associated with significant morbidity and mortality. This study compared childhood EM, SJS and TEN in terms of clinical courses, laboratory data, etiologies and outcomes in Taiwan. The initial laboratory findings, clinical presentations, etiologies and subsequent clinical courses of 30 patients with a diagnosis of EM, SJS or TEN, who were admitted between 1995 and 2003 at National Taiwan University Hospital were included and analyzed. There were 19 cases of EM, 8 cases of SJS, 2 cases of SJS/TEN and 1 case of TEN. The most common etiology in EM was infection (84.2%), and the most common implicated organism was Mycoplasma pneumoniae (42.1%). In contrast, 75% of SJS and 100% of TEN were induced by drugs. The most common offending drug was carbamazepine. Those patients with underlying diseases had more protracted courses and longer hospitalization stays. No mortalities were found in our cases. Early short-term steroid equivalent to 1-2 mg/kg/day of prednisolone for 3-5 days was used in 87.5% of SJS patients, without any significant side effects. Those with poor responsiveness to steroids and protracted courses were treated with additional intravenous immunoglobulin (IVIG) [1 g/kg/day], with satisfactory results. Early ophthalmic consultations were performed in all cases. No ocular complications were found in our cases. In conclusion, EM, SJS and TEN were associated with significant morbidity. Early ophthalmic consultations and withdrawal of the offending medication was necessary. Early short-term use of steroids in SJS showed promising results without significant side effects. The additional IVIG in those who had a poor response to steroid treatment may be helpful.
多形红斑（EM）、史蒂文斯 - 约翰逊综合征（SJS）和中毒性表皮坏死松解症（TEN）是伴有显著发病率和死亡率的皮肤黏膜疾病。本研究比较了台湾地区儿童EM、SJS和TEN在临床病程、实验室数据、病因及预后方面的情况。纳入并分析了1995年至2003年间在台湾大学附属医院住院的30例诊断为EM、SJS或TEN患者的初始实验室检查结果、临床表现、病因及后续临床病程。其中有19例EM、8例SJS、2例SJS/TEN和1例TEN。EM最常见的病因是感染（84.2%），最常见的相关病原体是肺炎支原体（42.1%）。相比之下，75%的SJS和100%的TEN由药物引起。最常见的致病药物是卡马西平。有基础疾病的患者病程更长，住院时间也更长。我们的病例中未发现死亡病例。87.5%的SJS患者早期使用相当于1 - 2 mg/kg/天泼尼松龙的短期类固醇药物，持续3 - 5天，未出现任何显著副作用。对类固醇反应不佳且病程迁延的患者加用静脉注射免疫球蛋白（IVIG）[1 g/kg/天]治疗，效果良好。所有病例均早期进行眼科会诊。我们的病例中未发现眼部并发症。总之，EM、SJS和TEN伴有显著的发病率。早期进行眼科会诊和停用致病药物是必要的。SJS患者早期短期使用类固醇显示出有前景的结果且无显著副作用。对类固醇治疗反应不佳的患者加用IVIG可能会有帮助。
