Clinicopathological profile of polyneuropathy, organomegaly, endocrinopathy, M protein and skin changes (POEMS) syndrome

Patients with polyneuropathy, organomegaly, endocrinopathy, M protein and skin changes (POEMS) syndrome caused by underlying plasma cell dyscrasias, although rare, may present with peripheral neuropathy. In this retrospective study we describe the clinical, electrophysiological and pathological profile of 29 patients (27 males and two females, with a mean age of 45.9 ± 7.9 years) seen between 1983 to 2009 who satisfied the Dispenzieri criteria. Polyneuropathy with weakness and paresthesias of the limbs evolving over 8.3 ± 9.1 months, organomegaly, endocrinopathy, monoclonal gammopathy and skin changes were seen in all patients. Other features included pedal edema (25 patients), papilledema (24), weight loss (23), clubbing (20) and Castleman disease (three patients). We report the pathological features seen in nerve biopsies, osteosclerotic lesions, skin biopsies, lymph node biopsies and bone marrow aspirates. We report the method of a diagnosis confirmation and compare the clinicopathological features of our patients with other published series. Our findings highlight the importance of a meticulous systemic examination and systematic laboratory investigations in the diagnosis of POEMS syndrome.