POEMS syndrome (Polyneuropathy, Organomegaly, Endocrinopathy,
Monoclonal protein, Skin changes) is characterized by the
presence of a monoclonal plasma cell disorder, peripheral
neuropathy, and one or more of the following features:
osteosclerotic myeloma, Castleman's disease, increased levels of
serum vascular endothelial growth factor (VEGF), organomegaly,
endocrinopathy, edema, typical skin changes, and papilledema .
The clinical features, diagnosis, and treatment of POEMS
syndrome will be discussed here. A discussion of Castleman's
disease is presented separately. (See "Castleman's disease".) In
1938, a patient was described with sensorimotor peripheral
neuropathy, hyperpigmentation, elevated cerebrospinal fluid
protein, and a solitary plasmacytoma . Eighteen years later, two
patients were reported with peripheral neuropathy,
hyperpigmentation, elevation of cerebrospinal fluid protein, and
plasmacytomas with new bone formation . In 1980 the acronym
"POEMS" was suggested for this disorder, representing the