Crow-Fukase syndrome

Abstract

Crow-Fukase syndrome is a unique multisystem disorder that is also known as POEMS syndrome (an acronym for polyneuropathy, organomegaly, endocrinopathy, the presence of M-protein and skin change). This syndrome is strongly associated with plasma cell dyscrasia. Circulating light chains of M component, almost invariably IgG lambda or IgA lambda, are found in 75% of patients. Neuropathologically, segmental demyelination, particularly in the proximal segment of the peripheral nerve trunk, is the primary process. Axonal degeneration and marked endoneurial edema are also characteristic. Focal excessive myelin outfolds with globular features corresponding to periodicity and paranodal enlargement of myelin are also highly characteristic of this syndrome. Vascular endothelial growth factor (VEGF) was found to be specifically and highly elevated in the serum of patients with this syndrome, suggesting a pathogenic role. M-protein, interleukin (IL)-1beta, IL-6 and tumor necrosis factor (TNF)-alpha are also considered to be involved in the pathogenesis. Treatment consists of radiation and surgical resection of the myeloma, chemotherapy, and a high dose of intravenous immunoglobulin (IVIg).