POEMS-Syndrom

Abstract

POEMS syndrome is a rare paraneoplastic syndrome due to a plasma cell dyscrasia, which includes peripheral neuropathy, organomegaly, endocrinopathy, monoclonal plasma cell proliferation and skin changes. Elevated levels of VEGF (vascular endothelial growth factor) in the serum of patients are suggested to play a pivotal role in the pathophysiology. A 60-year-old male presented with POEMS syndrome and painful edema, skin thickening of the distal extremities and livid-erythematous discoloration. The sclerotic changes resulted in a severe limitation of joint flexibility. Furthermore, the patient showed clubbing, white nails and a facial lipoatrophy. In addition to the skin changes, the patient was diagnosed with polyneuropathy, monoclonal gammopathy (type lambda), high elevated VEGF-levels, hepatomegaly, lymphadenopathy, hypothyreosis, hypogonadism and thrombocytosis in the course of POEMS syndrome. Treatment with 4 cycles of bortezomib and dexamethasone resulted in improvement of symptoms.