Crow-Fukase syndrome, POEMS syndrome

Crow-Fukase syndrome, also called POEMS syndrome, is a rare paraneoplastic syndrome due to an underlying plasma cell neoplasm, characterized by polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes as its salient features. Major diagnostic criteria include sclerotic bone lesions, elevated serum or plasma levels of vascular endothelial growth factor (VEGF), and Castleman disease. The misdiagnosis is very common due to its rarity and complicated clinical manifestations. Recognition of the complex of clinical complications is the first step in effectively managing the disease. The pathogenesis is not well understood. VEGF appears to play an important role in the disease and is especially useful for monitoring therapy, but it is not likely the sole factor driving the disease. The most commonly used therapies include alkylators and steroids, high-dose chemotherapy with peripheral blood stem cell transplantation, lenalidomide, and bortezomib.