A 46-year-old man with polyneuropathy, hepatosplenomegaly, endocrinopathy,monoclonal gammopathy, skin changes, sclerotic bone lesions and refractoryascites
Eg. Siegel et Ur. Folsch, A 46-year-old man with polyneuropathy, hepatosplenomegaly, endocrinopathy,monoclonal gammopathy, skin changes, sclerotic bone lesions and refractoryascites, Z GASTROENT, 37(4), 1999, pp. 283-286
The POEMS syndrome is a rare multisystemic disorder with polyneurppathy. or
ganomegaly. endocrinopathy of various forms, production of monoclonal (M) c
omponent, and skin changes. We describe a 46-year-old man who developed asc
ites one year after the onset of peripheral neuropathy with accompanying mu
scle atrophies and increasing weakness. Extensive evaluation revealed that
the patient had no underlying liver disease, malignancy. infection, or card
iac or renal disease. The ascites initially responded to high-dose corticos
teroid therapy. The patient had many clinical features of the described POE
MS syndrome: Including sclerotic bone lesions. a persistent lambda-paraprot
ein and refractory ascites. In this case ascites was a main presenting feat
ure. Thus, the POEMS syndrome must be added to the list of rare causes of r
efractory ascites.