[46-year-old patient with polyneuropathy, hepatosplenomegaly, endocrinopathy, M-gradient, skin manifestations, sclerotic bone changes and therapy refractory ascites].
The POEMS syndrome is a rare multisystemic disorder with polyneuropathy, organomegaly, endocrinopathy of various forms, production of monoclonal (M) component, and skin changes. We describe a 46-year-old man who developed ascites one year after the onset of peripheral neuropathy with accompanying muscle atrophies and increasing weakness. Extensive evaluation revealed that the patient had no underlying liver disease, malignancy, infection, or cardiac or renal disease. The ascites initially responded to high-dose corticosteroid therapy. The patient had many clinical features of the described POEMS syndrome including sclerotic bone lesions, a persistent lambda-paraprotein and refractory ascites. In this case ascites was a main presenting feature. Thus, the POEMS syndrome must be added to the list of rare causes of refractory ascites.
Siegel
E G
EG
I. Medizinische Klinik, Allgemeine Innere Medizin, Christian-Albrechts-Universität zu Kiel.
Fölsch
U R
UR
D001201:Ascites / Q000209:etiology*; D003937:Diagnosis, Differential; D005743:Gastrectomy; D006801:Humans; D008297:Male; D008875:Middle Aged; D016878:POEMS Syndrome / Q000175:diagnosis*; D010439:Peptic Ulcer Perforation / Q000601:surgery; D011183:Postoperative Complications / Q000175:diagnosis; D013276:Stomach Ulcer / Q000601:surgery
46jähriger Patient mit Polyneuropathie, Hepatosplenomegalie, Endokrinopathie, M-Gradient, Hautveränderungen, sklerotischen Knochenveränderungen und therapierefraktärem Aszites.