[POEMS syndrome (or Crow-Fukase syndrome)].
POEMS is an acronym for polyneuropathy, organomegaly, endocrinopathy, M-protein and skin changes. POEMS syndrome is also called Crow-Fukase syndrome, chiefly in Japan. The 5 above mentioned features are not always present at the first examination. The minimal criteria to establish the diagnosis are the presence of a demyelinating and axonal polyneuropathy associated with an IgA or IgG monoclonal gammopathy, the light chain being almost always lambda, and at least 2 of the 8 other features: sclerosing plasmocytoma, endocrinopathy, skin changes, organomegaly, Castleman's disease, anasarca, papillary edema or thrombocytosis. Among these features, only cutaneous glomeruloid angioma are specific. Ultrastructural identification of uncompacted myelin lamellae on the peripheral nerve biopsy is also a strong argument in favor of the diagnosis. An associated "osteosclerotic" bone lesion must be carefully searched, because its treatment may improve the other features of the syndrome, especially the neuropathy. Cytokines and the vascular growth endothelial factor might play a role in the pathogenesis of this rare multisystemic disorder.
Lagueny
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Service de Neurologie, Hôpital Haut-Lévêque, CHU Bordeaux, Pessac, France.
Bouillot
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S
Vital
C
C
Ferrer
X
X
Larrieu
J-M
JM
Vital
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A
D006801:Humans; D016878:POEMS Syndrome / Q000150:complications / Q000175:diagnosis / Q000209:etiology / Q000628:therapy