title: Improvement of recurrent urticaria in a patient with Schnitzler syndrome associated with B-cell lymphoma with combination rituximab and radiotherapy.
abstract: 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.
authors.affiliation: Department of Dermatology, Course of Integrated Medicine, Graduate School of Medicine, Osaka University, Osaka, Japan. h-murota@derma.med.osaka-u.ac.jp
authors.forename: Hiroyuki
authors.initials: H
authors.lastname: Murota
authors.forename: Yukiko
authors.initials: Y
authors.lastname: Shoda
authors.forename: Tomohiko
authors.initials: T
authors.lastname: Ishibashi
authors.forename: Hiroyuki
authors.initials: H
authors.lastname: Sugahara
authors.forename: Itaru
authors.initials: I
authors.lastname: Matsumura
authors.forename: Ichiro
authors.initials: I
authors.lastname: Katayama
mesh_terms: D000368:Aged; D000911:Antibodies, Monoclonal / Q000627:therapeutic use*; D058846:Antibodies, Monoclonal, Murine-Derived; D000970:Antineoplastic Agents / Q000627:therapeutic use*; D003131:Combined Modality Therapy; D005260:Female; D006801:Humans; D016393:Lymphoma, B-Cell / Q000150:complications / Q000188:drug therapy* / Q000532:radiotherapy*; D012186:Retroperitoneal Neoplasms / Q000188:drug therapy / Q000532:radiotherapy*; D000069283:Rituximab; D019873:Schnitzler Syndrome / Q000150:complications* / Q000175:diagnosis; D014581:Urticaria / Q000150:complications*
chemical_list: D000911:Antibodies, Monoclonal; D058846:Antibodies, Monoclonal, Murine-Derived; D000970:Antineoplastic Agents; D000069283:Rituximab