Schnitzler综合征1例

A Case of Schnitzler Syndrome

患者女,45岁,四肢、躯干反复荨麻疹样皮损伴发热、关节痛1年。体检:双腋下和腹股沟可触及肿大淋巴结。皮损组织病理示:真皮浅层血管周围散在淋巴细胞和中性粒细胞浸润。实验室检查:白细胞、C反应蛋白和红细胞沉降率持续升高;血液免疫固定电泳示:IgM轻链κ型单克隆免疫球蛋白。骨扫描示:右侧肱骨远端、双侧股骨中下段、左侧胫骨异常代谢活跃。诊断:Schnitzler综合征。经环孢素联合秋水仙碱治疗后,症状部分缓解。

A 45-year-old female patient presented with recurrent urticarial rash in extremities and trunk, accompanied by fever and joint pain for one year.Physical examination showed double axilla and groin could be touched with enlarged lymph nodes.Histopathological examination showed scattered lymphocytes and neutrophils were observed around superficial dermal vessels.Laboratory tests showed WBC,CRP and ESR were consistently elevated. Blood immunofixation electrophoresis showed IgM light chain κ monoclonal immunoglobulin.Bone scans showed abnormal metabolic activity in the right distal humerus, bilateral middle and lower femurs, and left tibia.A diagnosis of Schnitzler syndrome was made.After treatment with cyclosporine and colchicine, the symptoms was partially alleviated.