Department of Pediatrics B, Schneider Children's Medical Center of Israel, Petach Tikva, Israel; Kipper Institute of Immunology, Schneider Children's Medical Center of Israel, Petach Tikva, Israel; Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel.
Department of Pediatrics B, Schneider Children's Medical Center of Israel, Petach Tikva, Israel.
J Allergy Clin Immunol Pract. 2018 Jul-Aug;6(4):1238-1242. doi: 10.1016/j.jaip.2017.10.016. Epub 2017 Dec 1.
Drug reaction with eosinophilia and systemic symptoms (DRESS) is a rare, potentially life-threatening delayed drug-induced hypersensitivity reaction. The most frequently reported drugs causing DRESS are aromatic antiepileptic agents. Prompt withdrawal of the offending drug and administering systemic corticosteroids is the most widely accepted and used treatment. The treatment of severe DRESS not responsive to systemic corticosteroids is uncertain.
The objective of this study was to describe a case series of pediatric patients with DRESS who were treated successfully with intravenous immunoglobulins (IVIGs).
A retrospective review of all children hospitalized in a tertiary care children's hospital with severe DRESS syndrome who received IVIG in addition to offending drug withdrawal and systemic corticosteroids during 1999-2017 is performed.
Seven severe DRESS patients (4 males, age: 9.5 ± 5.7 years) are described. The offending drugs were antiepileptics in all but one case. Clinical findings included fever, rash, lymphadenopathy, dyspnea, anasarca, and hepatic involvement. After IVIG treatment (total dosage: 1-2 g/kg), fever resolved within a median time of 1 (range, 0-5) day, rash disappeared after 6.3 ± 1.6 days, and liver enzymes substantially improved after 3.8 ± 1.6 days. Patients were discharged 6.1 ± 2.7 days after IVIG commencement. There was no mortality.
The addition of IVIG in DRESS syndrome resistant to regular drug withdrawal and systemic corticosteroid therapy may hasten disease recovery.
药物反应伴嗜酸性粒细胞增多和全身症状（DRESS）是一种罕见的、潜在危及生命的迟发性药物超敏反应。最常报告的引起 DRESS 的药物是芳香抗癫痫药。最广泛接受和使用的治疗方法是迅速停用引起过敏的药物和给予全身皮质类固醇。对全身皮质类固醇反应不佳的严重 DRESS 的治疗尚不确定。
本研究的目的是描述一组成功接受静脉注射免疫球蛋白（IVIG）治疗的 DRESS 儿科患者的病例系列。
回顾性分析了 1999 年至 2017 年间在一家三级儿童保健医院住院的所有严重 DRESS 综合征患儿的病例，这些患儿在停用引起过敏的药物和全身皮质类固醇的基础上还接受了 IVIG 治疗。
描述了 7 例严重 DRESS 患者（4 名男性，年龄：9.5±5.7 岁）。除 1 例外，所有患者的致病药物均为抗癫痫药。临床发现包括发热、皮疹、淋巴结病、呼吸困难、全身性水肿和肝受累。接受 IVIG 治疗（总剂量：1-2 g/kg）后，中位时间 1 天（范围，0-5 天）内发热消退，皮疹在 6.3±1.6 天内消失，肝酶在 3.8±1.6 天内显著改善。患者在开始 IVIG 治疗后 6.1±2.7 天出院。无死亡病例。
在常规药物停药和全身皮质类固醇治疗抵抗的 DRESS 综合征中，加用 IVIG 可能会加速疾病恢复。
