Kurian Anil, Lee Jason K, Vadas Peter
Department of Medicine, McMaster University, Hamilton, Ontario, Canada.
J Dermatol Case Rep. 2010 Dec 31;4(4):50-3. doi: 10.3315/jdcr.2010.1060.
Schnitzler syndrome encompasses monoclonal gammopathy, urticaria, inflammation, recurrent fever, bone pain and arthralgia, with occasional lymphadenopathy and/or hepatosplenomegaly. It is a rare condition with approximately 100 cases reported in the literature. To our knowledge, this is the first reported case of cold-induced physical urticaria with Schnitzler syndrome.
A 43-year-old woman presented to an allergy and immunology clinic with a 7 year history of chronic urticaria, angioedema with anaphylaxis, monoclonal gammopathy and bone pain. Her urticaria was triggered by cooler temperatures and an ice cube test for cold induced urticaria was positive. In spite of aggressive therapies this patient remains symptomatic.
Schnitzler syndrome is under-recognized, particularly variants of the classical description of Schnitzler syndrome. Other diseases, especially those of hematologic origin, should be ruled out. This condition is largely refractory to conventional therapies, although novel treatments, such as interleukin-1 receptor inhibitor, may show promise.
施尼茨勒综合征包括单克隆丙种球蛋白病、荨麻疹、炎症、反复发热、骨痛和关节痛，偶尔伴有淋巴结病和/或肝脾肿大。这是一种罕见疾病，文献报道约100例。据我们所知，这是首例报道的伴有施尼茨勒综合征的冷诱发性物理性荨麻疹病例。
一名43岁女性因慢性荨麻疹、伴有过敏反应的血管性水肿、单克隆丙种球蛋白病和骨痛7年病史就诊于过敏与免疫诊所。她的荨麻疹由较低温度诱发，冷诱发性荨麻疹冰块试验呈阳性。尽管采取了积极治疗，该患者仍有症状。
施尼茨勒综合征未得到充分认识，尤其是施尼茨勒综合征经典描述的变异型。应排除其他疾病，尤其是血液系统起源的疾病。尽管新型治疗方法，如白细胞介素-1受体抑制剂，可能有前景，但这种疾病对传统治疗大多无效。
