POEMS syndrome with IgA lambda monoclonal gammopathy

The various association of polyneuropathy, organomegaly, endocrinopathy, monoclonal protein and skin changes has been called the POEMS syndrome. Herein we report a case in which all the typical clinical and laboratory findings of the POEMS syndrome were present. We emphasize as the most striking features of our case the following: the similarity with scleroderma, the peripheral nerve demyelination without the presence of antibodies against nerve components, the occurrence of an IgA lambda monoclonal gammopathy and Castleman-like features at a single enlarged lymph node. To our knowledge, this is the first such case reported in Italy.