Moos Łukasz, Kułakowska Aleksandra, Szydłowska Dorota, Chodak Weronika, Brzoza Zenon
Department of Internal Diseases, Allergology, Endocrinology and Gastroenterology, Institute of Medical Sciences, University of Opole, University Clinical Hospital, Al. Witosa 26, 45-401 Opole, Poland.
Students' scientific association "Alergos", Institute of Medical Sciences, University of Opole, University Clinical Hospital, Al. Witosa 26, 45-401 Opole, Poland.
Oxf Med Case Reports. 2025 Jul 27;2025(7):omaf118. doi: 10.1093/omcr/omaf118. eCollection 2025 Jul.
Schnitzler syndrome (SchS) is a very rare acquired systemic disease that has many similarities to hereditary autoinflammatory syndromes. The condition is characterized by the presence of monoclonal gammopathy and chronic urticaria. In this case report, a 64-year-old male patient with SchS was initially misdiagnosed with chronic spontaneous urticaria (CSU). The patient was treated with anakinra, showing good tolerance and no need for steroid therapy for 14 months. SchS is often underdiagnosed and presents symptoms such as prolonged urticarial wheals and systemic manifestations. The differential diagnoses include mastocytosis, urticarial vasculitis, and autoimmune diseases. The diagnostic criteria were elevated CRP levels, neutrophilic skin infiltration, leukocytosis, and abnormal bone remodeling on scintigraphy. Treatment options include highly effective interleukin-1 blockade therapies such as anakinra, canakinumab, and rilonacept. This case emphasizes the importance of a thorough differential diagnosis of chronic urticaria and encourages clinicians to participate in the SchS database for improved recognition and management.
施尼茨勒综合征（SchS）是一种非常罕见的获得性全身性疾病，与遗传性自身炎症综合征有许多相似之处。该病症的特征是存在单克隆丙种球蛋白病和慢性荨麻疹。在本病例报告中，一名64岁的施尼茨勒综合征男性患者最初被误诊为慢性自发性荨麻疹（CSU）。该患者接受了阿那白滞素治疗，显示出良好的耐受性，并且在14个月内无需使用类固醇疗法。施尼茨勒综合征常常诊断不足，表现出如持续性风团和全身症状等。鉴别诊断包括肥大细胞增多症、荨麻疹性血管炎和自身免疫性疾病。诊断标准为CRP水平升高、中性粒细胞皮肤浸润、白细胞增多以及骨闪烁显像显示骨重塑异常。治疗选择包括高效的白细胞介素-1阻断疗法，如阿那白滞素、卡那单抗和利罗那肽。本病例强调了对慢性荨麻疹进行全面鉴别诊断的重要性，并鼓励临床医生参与施尼茨勒综合征数据库，以提高识别和管理水平。
