着装综合症

IF 0.3 Q4 ALLERGY
S. Bahçeci, Demet Can
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引用次数: 0

摘要

伴嗜酸性粒细胞增多症和全身症状的药物皮疹(DRESS)是一种罕见的、特殊的、危及生命的药物反应,具有多种临床表现,包括发烧高于38.5摄氏度、瘙痒性斑丘疹或红斑性皮疹、血液学异常、淋巴结病和多器官受累的症状。其发病率从1000分之一到10000分之一不等,估计死亡率高达10%。迄今为止,许多药物已被报道会导致DRESS综合征,但最常见的是抗惊厥药和磺酰胺类药物,尽管其发病机制尚不清楚。对芳香族抗惊厥药物代谢产物进行解毒的环氧化物羟化酶缺乏或缺陷,导致可能引发免疫反应的反应性代谢产物的药物解毒不足,一些HLA等位基因引起的易感性,以及疱疹病毒的再激活,都被认为在发病机制中发挥了作用。潜伏期从两到六周不等。血液学、肝、肾、心脏、肺、神经、胃肠道和内分泌受累;并且在DRESS综合征的临床过程中可以看到噬血细胞综合征。DRESS综合征的长期后遗症包括肝、肾和肾上腺功能衰竭;1型和2型糖尿病、Graves病、自身免疫性溶血性贫血、狼疮、系统性硬化症和自身免疫性肠病。DRESS综合征的诊断很难确定,需要高度的初步临床怀疑和排除其他病因。DRESS综合征治疗中最重要的一步是早期诊断并及时停药。在器官受累的情况下,需要全身皮质类固醇治疗。在严重和类固醇耐药的情况下,可能需要使用更强效的免疫抑制剂或静脉注射免疫球蛋白治疗。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Dress Syndrome
Drug Rash with Eosinophilia and Systemic Symptoms (DRESS) is a rare, idiosyncratic, life-threatening drug reaction with a variety of clinical manifestations including symptoms of fever higher than 38.5 oC, pruritic maculopapular or erythematous eruption, hematologic abnormalities, lymphadenopathy, and multiorgan involvement. Its incidence ranges from 1 in 1000 to 1 in 10,000 drug exposures, and it has an estimated mortality rate of up to 10%. To date, many drugs have been reported to cause DRESS syndrome, but the most common ones are the anticonvulsants and sulfonamides, although the pathogenesis is not clearly understood. Deficiency or defects in the epoxide hydroxylase enzyme, which detoxifies the metabolites of aromatic anticonvulsants, an insufficiency in the detoxification of the drug leading to reactive metabolites which may trigger immunologic reactions, predispositions due to some HLA alleles, and reactivation of herpes viruses are suggested to play a role in the pathogenesis. The latent period varies from two to six weeks. Hematologic, hepatic, renal, cardiac, pulmonary, neurologic, gastrointestinal and endocrine involvement; and hemophagocytic syndrome can be seen during the clinical course of DRESS syndrome. The long term sequels of DRESS syndrome include hepatic, renal and adrenal failure; diabetes mellitus type 1 and type 2, Graves disease, autoimmune hemolytic anemia, lupus, systemic sclerosis, and autoimmune enteropathy. Diagnosis of DRESS syndrome is difficult to establish, and requires a high degree of initial clinical suspicion and ruling out of other etiologies. The most important step in the management of DRESS syndrome is early diagnosis and prompt withdrawal of the offending drug. In cases with organ involvement, systemic corticosteroid treatment is required. In serious and steroid-resistant cases, using more potent immunosuppressive agents or intravenous immunoglobulin treatments may be required.
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来源期刊
CiteScore
0.80
自引率
0.00%
发文量
10
期刊介绍: Asthma Allergy Immunology has been published three times a year in April, August and December as the official and periodical journal of the Turkish National Society of Allergy and Clinical Immunology since 2003. All articles published in the journal have been available online since 2003. A peer reviewed system is used in evaluation of the manuscripts submitted to Asthma Allergy Immunology. The official language of the journal is English. The aim of the journal is to present advances in the field of allergic diseases and clinical immunology to the readers. In accordance with this goal, manuscripts in the format of original research, review, case report, articles about clinical and practical applications and editorials, short report and letters to the editor about allergic diseases and clinical immunology are published in the journal. The target reader population of the Asthma Allergy Immunology includes specialists and residents of allergy and clinical immunology, pulmonology, internal medicine, pediatrics, dermatology and otolaryngology as well as physicians working in other fields of medicine interested in allergy and immunological diseases.
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