[Crow-Fukase syndrome].
The association of polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes forms a characteristic multisystem syndrome, Crow-Fukase syndrome, or acronym POEMS syndrome. The pathogenesis of the syndrome is still unknown. An M component found in more than three quarters of the patients appears not be the direct cause, because no evidence of deposition or interaction was observed in skin, peripheral nerve or endocrine organs with few exceptions. Solitary or multiple bone lesions, or plasmacytoma, are found in more than half of patients with Crow-Fukase syndrome. The nature of abnormal plasma cells of Crow-Fukase syndrome seems to be distinct from that of multiple myeloma. Substances elaborated by plasmacytoma cells are recently postulated as a pathogenesis of the disorder because of the finding that the Crow-Fukase syndrome may regress after resection or irradiation of solitary plasmacytoma, although a definite conclusion awaits further investigations.
Hitoshi
S
S
Department of Neurology, Faculty of Medicine, University of Tokyo.
D005260:Female; D006801:Humans; D008297:Male; D009101:Multiple Myeloma; D016878:POEMS Syndrome