A case of POEMS syndrome presenting as primary adrenocortical insufficiency associated with plasma cell myeloma / 대한내과학회지

ABSTRACT

POEMS syndrome is multisystemic disorder characterized polyneuropathy, organomegaly, endocrinopathies, monoclonal gammapathy. A 38 year-old man visited our hospital because of progressive general weakness, hyperpigmentation. We diagnosed him as idiopathic primary adrenal insufficiency. The symptoms of general weakness were improved after steroid replacement therapy. But after 6 months, he revisited ER with chief complaints of abdominal distension and edema in lower extremities. He had also polyneuropathy, hepatosplenomegaly, IgA light chain monoclonal gammopathy, hyperpigmentation and thickening of the skin. Bone marrow biopsy showed that the plasma cells, mainly mature forms, are about 40% of all nucleated elements and on tissue section. We think this is the first case of POEMS syndrome presenting as primary adrenocortical insufficiency in Korea, so we report the case with reviews of other literatures. And we recommend performing a rapid ACTH stimulation test in all POEMS syndrome in order to detect adrenal insufficiency.