Department of Dermatology, University of California, San Francisco, San Francisco, CA 94115, USA.
JAMA Dermatol. 2013 Apr;149(4):453-8. doi: 10.1001/jamadermatol.2013.2705.
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).
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.
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.
在荨麻疹的一部分患者中可以看到以中性粒细胞浸润为主的病变。这些病变的瘙痒程度较低，对标准治疗（包括抗组胺药）反应不佳。我们描述了 2 例伴有全身炎症的中性粒细胞性荨麻疹（NUSI）的患者，这些患者无已知的结缔组织疾病或恶性肿瘤。我们提出 NUSI 这一术语，旨在帮助分类一种以前未定义的多系统炎症实体，可能由白细胞介素 1（IL-1）介导。
患者 1 为 47 岁女性，出现荨麻疹伴夜间出汗、发热和多关节关节炎。急性相反应物升高，症状恶化。最初的治疗方案是联合使用局部和全身皮质类固醇、抗组胺药和免疫抑制剂，但效果不佳。使用白细胞介素 1 受体拮抗剂阿那白滞素后，患者达到 100%的临床缓解。患者 2 为 24 岁女性，出现荨麻疹伴关节疼痛和肿胀。最初的治疗包括抗组胺药、秋水仙碱和氨苯砜联合治疗。只有秋水仙碱有一定疗效，但因胃肠道不适而停用。开始使用阿那白滞素后，患者每天接受治疗，达到 100%的控制。
对于接受抗组胺药治疗抵抗的荨麻疹且伴有全身炎症症状的患者，应考虑诊断为 NUSI。白细胞介素 1 阻断剂是一种可行的治疗选择。
