Drug reaction with eosinophilia and systemic symptoms (DRESS): an unusual manifestation of multi-visceral abnormalities and long-term outcome.

Kinal Paresh Bhatt, Fahed Alsoud, Adesh Prashad, Jose Ortega-Tola, Virendra Ravat Singh, Pooja Patel, George Michel
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Abstract

Drug reaction with eosinophilia and systemic symptoms (DRESS), also known as drug induced hypersensitivity (DiHS) is a rare, however a severe hypersensitivity reaction with a mortality rate of up to 10%, accounting for 10 to 20% of all cutaneous drug reactions in hospitalized patients. The clinical features of DRESS/DiHS may be challenging to recognize and diagnose, since they are delayed, stepwise, and heterogeneous. The classic presentation of DRRSS/DiHS involves a combination of cutaneous, hematologic, and internal organ involvement with a 2 to 8 weeks latency between drug exposure and the onset of symptoms. Finding the culprit drug in our case was difficult as the patient was taking multiple antibiotics. Drugs such as vancomycin and cefepime used before the rash outbreak for post-reconstructive surgery for left toal knee arthroplasty (TKA) approximately four weeks before the onset of the rash are likely offending agents. This patient also had multi-visceral involvement with eosinophilia and systemic symptoms. The current treatment guidelines for DRESS/DiHS are primarily based on expert opinion, as no randomized control trials exist. After the prompt withdrawal of the offending drug, systemic corticosteroids seem to have shown the best outcome for patients. Delaying discontinuing offending medications and initiating corticosteroid treatment may lead to poor results. The present case emphasizes that the close observation of patients with drug eruption induced by antibiotics is imperative. Primary care team should be able to promptly diagnose patients with DRESS syndrome, detect causative drug, and play a crucial role in the timely evaluation and treatment to reduce mortality rate. The later phase disease relapse or autoimmune complications may occur up to 5 years following the initial presentation. Therefore, we advised the patient to have an outpatient follow up for appropriate testing, including but not limited to genetic susceptibility due to the high risk of relapse and emerging risk of autoimmune diseases.

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药物反应伴嗜酸性粒细胞增多和全身症状(DRESS):多脏器异常的不寻常表现和长期结局。
伴有嗜酸性粒细胞增多和全身症状的药物反应(DRESS),也称为药物性超敏反应(DiHS),是一种罕见的,但死亡率高达10%的严重超敏反应,占住院患者所有皮肤药物反应的10%至20%。DRESS/DiHS的临床特征可能难以识别和诊断,因为它们是延迟的、逐步的和异质性的。DRRSS/DiHS的典型表现包括皮肤、血液学和内脏器官受累,在药物暴露和症状发作之间有2至8周的潜伏期。在我们的病例中,很难找到罪魁祸首药物,因为患者服用了多种抗生素。在左全膝关节置换术(TKA)重建手术后皮疹爆发前大约四周使用万古霉素和头孢吡肟等药物可能是起作用的药物。该患者还伴有嗜酸性粒细胞增多和全身症状。由于没有随机对照试验,DRESS/DiHS目前的治疗指南主要基于专家意见。在迅速停用药物后,全身性皮质类固醇似乎对患者效果最好。延迟停用不良药物和开始皮质类固醇治疗可能导致不良结果。本病例强调密切观察抗生素引起药疹的患者是必要的。初级保健团队应能够及时诊断DRESS综合征患者,发现致病药物,并在及时评估和治疗中发挥至关重要的作用,以降低死亡率。晚期疾病复发或自身免疫性并发症可在首次出现后5年内发生。因此,我们建议患者进行门诊随访以进行适当的检测,包括但不限于由于复发风险高和自身免疫性疾病新发风险导致的遗传易感性检测。
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