POEMS syndrome: a contribution to differential diagnosis of polyneuropathy
Dressnandt, J.; Näher-Noé, M.; Drosner, M.; Klingelhöfer, J.
Der Nervenarzt 64(4): 258-262
1993
ISSN/ISBN: 0028-2804 PMID: 8506014 Document Number: 413888
The POEMS syndrome is a synopsis of different symptoms such as polyneuropathy, organomegaly, endocrine disturbances, M-protein and skin changes. The leading symptoms are neuropathy and the skin symptoms. Additionally, a monoclonal light chain gammopathy is often found. The administration of immunosuppressive drugs yields a substantial improvement in some cases. We report here about a 72 year old lady who fell ill with a rapidly progressive neuropathy accompanied by hyperpigmentation and a morphea-like induration of the skin. A biopsy of the sural nerve showed a demyelinating axonal neuropathy and a focal vasculitis. Isoelectric focussing revealed oligoclonal bands in cerebrospinal fluid and serum. The cortisol serum level was very low and there were signs of a latent diabetes mellitus. These clinical features correspond to the POEMS syndrome. The prescription of initially 1 mg and later 0.5 mg prednisone improved the patient's condition dramatically.