Murota Hiroyuki, Shoda Yukiko, Ishibashi Tomohiko, Sugahara Hiroyuki, Matsumura Itaru, Katayama Ichiro
Department of Dermatology, Course of Integrated Medicine, Graduate School of Medicine, Osaka University, Osaka, Japan.
J Am Acad Dermatol. 2009 Dec;61(6):1070-5. doi: 10.1016/j.jaad.2008.12.040. Epub 2009 Jul 25.
Schnitzler syndrome is a rare condition defined by chronic urticaria, osteosclerotic bone lesions, and monoclonal IgM gammopathy. Schnitzler syndrome can precede the onset of a true lymphoproliferative disorder including Waldenström macroglobulinemia and rarely systemic marginal zone B-cell lymphoma. We describe a case of intractable chronic urticaria accompanied by a retroperitoneal neoplasm. IgM monoclonal gammopathy, lumber pain, intermittent fever, and elevation of C-reactive protein were the clues for the diagnosis of Schnitzler syndrome. An evaluation for malignancy using systemic computed tomography scan and fluorodeoxyglucose positron emission tomography revealed the retroperitoneal tumor, and a subsequent bone-marrow aspirate confirmed the diagnosis of B-cell lymphoma. Combined rituximab and radiotherapy ameliorated the skin symptoms. This case indicates that a detailed search for malignant neoplasms might be required for the long-term management of Schnitzler syndrome, and that B-cell lymphomas may contribute to the pathogenesis of this condition.
施尼茨勒综合征是一种罕见疾病，其特征为慢性荨麻疹、骨硬化性骨病变和单克隆IgM丙种球蛋白病。施尼茨勒综合征可先于包括华氏巨球蛋白血症在内的真正淋巴增殖性疾病发作，很少先于系统性边缘区B细胞淋巴瘤发作。我们描述了一例伴有腹膜后肿瘤的顽固性慢性荨麻疹病例。IgM单克隆丙种球蛋白病、腰痛、间歇性发热和C反应蛋白升高是诊断施尼茨勒综合征的线索。使用全身计算机断层扫描和氟脱氧葡萄糖正电子发射断层扫描进行的恶性肿瘤评估发现了腹膜后肿瘤，随后的骨髓穿刺确诊为B细胞淋巴瘤。利妥昔单抗联合放疗改善了皮肤症状。该病例表明，施尼茨勒综合征的长期管理可能需要详细排查恶性肿瘤，且B细胞淋巴瘤可能参与了该疾病的发病机制。
