Neutrophilic Urticaria With Systemic Inflammation A Case Series
Neutrophilic Urticaria With Systemic Inflammation A Case Series
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DOI：
10.1001/jamadermatol.2013.2705
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发表时间：
2013-04-01
期刊：
影响因子：
10.9
通讯作者：
Leslie, Kieron S.
中科院分区：
文献类型：
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作者：
Belani, Hrishikesh;Gensler, Lianne;Leslie, Kieron S.
重要性：在荨麻疹患者的一个亚组中观察到主要的嗜酸性浸润。病变往往不那么痒，对标准治疗反应不良，包括抗组胺药。我们描述了2例患有嗜酸性荨麻疹伴全身性炎症（NUSI）的患者，无已知的结缔组织疾病或恶性肿瘤。我们提出的术语NUSI，以帮助分类以前未定义的多系统炎症实体可能介导的白细胞介素1（IL-1）observations：患者1，47岁的女性，被认为是荨麻疹和相关的盗汗，发烧，和多关节炎。急性期反应物随症状加重而升高。局部和全身皮质类固醇，抗组胺药和免疫抑制剂的组合的初始治疗是不成功的。阿那白滞素（一种IL-1受体拮抗剂）达到100%临床消退。患者2是一名24岁的女性，出现荨麻疹和相关的关节疼痛和肿胀。初始治疗包括抗组胺药、秋水仙碱和氨苯砜的组合。只有秋水仙碱提供了中等的好处，但停止，因为显着的胃肠道不适。开始使用阿那白滞素;患者达到100%的控制，而接受日常therapy.Conclusions和相关性：NUSI的诊断是重要的，以考虑在患者谁是抗组胺药耐药荨麻疹与全身炎症症状相结合。白细胞介素1阻断是一种可行的治疗选择。
Importance: Predominantly neutrophilic infiltrates are seen in a subset of patients with urticaria. The lesions tend to be less itchy and poorly responsive to standard therapy, including antihistamines. We describe 2 patients having neutrophilic urticaria with systemic inflammation (NUSI) without known connective tissue disorder or malignancy. We propose the term NUSI to help classify a previously undefined multisystemic inflammatory entity likely mediated by interleukin 1 (IL-1).Observations: Patient 1, a 47-year-old woman, was seen with urticaria and associated night sweats, fevers, and polyarticular arthritis. Acute-phase reactants were elevated with worsening of symptoms. Initial treatment with a combination of topical and systemic corticosteroids, antihistamines, and immunosuppressants was unsuccessful. A 100% clinical resolution was achieved with anakinra, an IL-1 receptor antagonist. Patient 2, a 24-year-old woman, was seen with urticaria and associated joint pain and swelling. Initial treatment included a combination of antihistamines, colchicine, and dapsone. Only colchicine provided moderate benefit but was stopped because of significant gastrointestinal tract discomfort. Anakinra was initiated; the patient achieved 100% control while receiving daily therapy.Conclusions and Relevance: The diagnosis of NUSI is important to consider in patients who are seen with antihistamine-resistant urticaria in combination with systemic inflammatory symptoms. Interleukin 1 blockade is a viable option for therapy.