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[儿童严重皮肤药物反应]

[Severe cutaneous drug reactions in children].

作者信息

Mockenhaupt M

机构信息

Dokumentationszentrum schwerer Hautreaktionen (dZh), Universitätsklinik für Dermatologie und Venerologie, Hauptstr. 7, 79104, Freiburg, Deutschland.

出版信息

Hautarzt. 2017 Oct;68(10):803-814. doi: 10.1007/s00105-017-4048-7.

Abstract

Among severe drug reactions in children, besides Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN), a specific form of hypersensitivity syndrome which is nowadays known as "drug reaction with eosinophilia and systemic symptoms" (DRESS) has to be mentioned. Whereas SJS/TEN is considered one reaction entity of different severity, DRESS has to be distinguished from SJS/TEN but also from other severe exanthems due to multiorgan involvement. Although SJS/TEN is generally referred to as a drug reaction, only about 75% of all cases are actually caused by medications and in children it is only about 50%. After a clear diagnosis has been made, specific therapeutic measures can follow, of which withdrawal of the inducing agent plays a key role, but further treatments differ substantially. In order to identify and withdraw the inducing agent, a detailed and thorough medication history must be obtained. Highly suspected drugs of SJS/TEN in children include, among others, antibacterial sulfonamides and various antiepileptics. DRESS in children and adolescents is also frequently induced by antiepileptics, but also by sulfonamides and minocycline. In contrast to adults, allopurinol is rarely found to be culprit in both conditions. Supportive therapy including appropriate topical treatments, pain therapy, ophthalmologic consultations, etc. is the gold standard in SJS/TEN, but a short-term immunomodulating therapy with cyclosporine A has shown very promising results in recent studies. In DRESS, however, systemic treatment with glucocorticosteroids slowly tapered over a longer period of time is recommended.

摘要

在儿童严重药物反应中,除了史蒂文斯-约翰逊综合征(SJS)和中毒性表皮坏死松解症(TEN)外,还必须提及一种特殊形式的超敏综合征,即如今被称为“伴有嗜酸性粒细胞增多和全身症状的药物反应”(DRESS)。SJS/TEN被认为是不同严重程度的一种反应实体,而DRESS必须与SJS/TEN区分开来,同时也因其多器官受累而与其他严重皮疹相区分。尽管SJS/TEN通常被称为药物反应,但实际上所有病例中只有约75%是由药物引起的,在儿童中这一比例仅约为50%。明确诊断后,可采取具体的治疗措施,其中停用诱发药物起着关键作用,但进一步的治疗方法有很大差异。为了识别和停用诱发药物,必须获取详细而全面的用药史。儿童中高度怀疑导致SJS/TEN的药物包括抗菌磺胺类药物和各种抗癫痫药物等。儿童和青少年的DRESS也常由抗癫痫药物诱发,但也可由磺胺类药物和米诺环素诱发。与成人不同,在这两种情况下很少发现别嘌醇是罪魁祸首。支持性治疗包括适当的局部治疗、疼痛治疗、眼科会诊等是SJS/TEN的金标准,但近期研究表明,短期使用环孢素A进行免疫调节治疗已显示出非常有前景的结果。然而,在DRESS中,建议长期缓慢减量使用糖皮质激素进行全身治疗。

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