Recurrent Crow-Fukase syndrome associated with increased serum levels of vascular endothelial growth factor: A case report and review of the literature

Crow-Fukase syndrome (CFS) is a rare multi-system disorder, characterized by polyneuropathy, organomegaly, endocrinopathy, M-proteins, skin changes and anasarea, with or without myeloma. The pathophysiology, diagnosis, and treatment of CFS are controversial. CFS may be associated with the overproduction of vascular endothelial growth factor (VEGF). However, there have been no reports of monitoring the serum VEGF level after recurrence, to the best of our knowledge. We report a 54-year-old man with CFS presenting with a 3-year history of ascites, anasarca, weakness of the lower extremities, and plasmacytoma in the scapula. At the initial examination, the VEGF level was 1,590 pg/ml (the VEGF level of a healthy control, 78.4 +/- 75.2 pg/ml). After initial treatment with chemotherapy and irradiation of the affected shoulder, the VEGF level decreased to 154 pg/ml and the symptoms disappeared. Twenty one months later, gate disturbance and anasarca recurred, and the VEGF level was over 2,000 pg/dl. After total scaplectomy, the VEGF level decreased to 730 pg/dl and the symptoms disappeared. The serum level of VEGF well correlated to the clinical course of the patient. In conclusion, measurement of the VEGF level is useful for diagnosing CFS and for monitoring its clinical course.