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: Hiroyuki Murota, Yukiko Shoda, Tomohiko Ishibashi, Hiroyuki Sugahara, Itaru Matsumura, Ichiro Katayama
• Journal: Journal of the American Academy of Dermatology (J Am Acad Dermatol) Vol. 61 Issue 6 Pg. 1070-5 (Dec 2009) ISSN: 1097-6787 [Electronic] United States
• PMID: 19632739 (Publication Type: Case Reports, Journal Article)

Chemical References

• Antibodies, Monoclonal
• Antibodies, Monoclonal, Murine-Derived
• Antineoplastic Agents
• Rituximab

Topics

• Aged
• Antibodies, Monoclonal (therapeutic use)
• Antibodies, Monoclonal, Murine-Derived
• Antineoplastic Agents (therapeutic use)
• Combined Modality Therapy
• Female
• Humans
• Lymphoma, B-Cell (complications, drug therapy, radiotherapy)
• Retroperitoneal Neoplasms (drug therapy, radiotherapy)
• Rituximab
• Schnitzler Syndrome (complications, diagnosis)
• Urticaria (complications)