Clinical manifestation of POEMS syndrome with features of connective tissue disease

The POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopa thy, skin changes) syndrome is a rare plasma cell disease with multiorgan i nvolvement and varying clinical manifestations. We report a 38-year-old man who presented with scleroderma-like skin changes of the hands and feet, si cca and Raynaud's syndrome, pleural effusions, glomerulopathy, polyneuropat hy, hepatosplenomegaly and lymphadenopathy. Steroid treatment was started o n the assumption of a connective tissue disease and led to a temporary impr ovement. During the further course of the disease, hypothyreosis, monoclona l gammopathy and osteosclerotic bone lesions were detected, leading to the diagnosis of POEMS syndrome. This case emphasises the need to consider POEM S syndrome as a differential diagnosis in patients with signs of connective tissue disease and polyneuropathy.