The diagnosis of POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, M-protein, skin changes), based on the current Dispenzieri diagnostic criteria [1], requires the presence of both mandatory criteria (a polyneuropathy and a monoclonal plasma cell-proliferative disorder, almost always lambda restricted), and at least one major and one minor criterion (Table 1). This evidence concerns the gene MYOM2 and Abnormality of the skin.