Puxkandl Viktoria, Currie Antonia, Hoetzenecker Wolfram, Altrichter Sabine
Department of Dermatology, Comprehensive Allergy Center, Kepler University Hospital Linz & Johannes Kepler University, Krankenhausstrasse 9, 4020, Linz, Austria.
Departement of Dermatology and Allergology, Charité - Universitätsmedizin Berlin, Berlin, Germany.
Allergy Asthma Clin Immunol. 2023 Jul 26;19(1):64. doi: 10.1186/s13223-023-00819-x.
Recurring therapy resistant hives, accompanied by IgM-gammopathy, fever and joint pain can indicate Schnitzler syndrome, a rare autoimmune disorder. There is currently no approved treatment, but complete remission of symptoms can be induced with IL-1 antagonists.
A patient with a history of chronic urticaria presented frequently at the outpatient clinic with severe hives and was treated unsuccessfully with antihistamines and omalizumab. After several years, additional symptoms such as joint pain, recurrent fever, and IgM-gammopathy developed. After the diagnostic criteria for Schnitzler syndrome were met, treatment with anakinra was initiated and resulted in an improvement of the symptoms. Shortly after the first injection, the patient developed large and painful erythematous lesions at the injection sites, leading to discontinuation of treatment and a rapid recurrence of symptoms. Subsequently, treatment with a longer-acting IL-1 antagonist (canakinumab) was initiated, resulting in a complete remission of symptoms.
This case report demonstrates that patients with urticarial symptoms that are not relieved by typical treatments should prompt repeated reassessments of the diagnosis, even years later, because gammopathy and other diagnostic criteria for Schnitzler syndrome can occur with a delay.
复发性难治性荨麻疹，伴有IgM丙种球蛋白病、发热和关节疼痛，可能提示施尼茨勒综合征，一种罕见的自身免疫性疾病。目前尚无获批的治疗方法，但使用白细胞介素-1拮抗剂可诱导症状完全缓解。
一名有慢性荨麻疹病史的患者经常到门诊就诊，出现严重荨麻疹，使用抗组胺药和奥马珠单抗治疗无效。数年后，出现了关节疼痛、反复发热和IgM丙种球蛋白病等其他症状。符合施尼茨勒综合征的诊断标准后，开始使用阿那白滞素治疗，症状有所改善。首次注射后不久，患者在注射部位出现大的疼痛性红斑病变，导致治疗中断，症状迅速复发。随后，开始使用长效白细胞介素-1拮抗剂（卡那单抗）治疗，症状完全缓解。
本病例报告表明，典型治疗无法缓解荨麻疹症状的患者应促使反复重新评估诊断，即使数年之后，因为丙种球蛋白病和施尼茨勒综合征的其他诊断标准可能会延迟出现。
