POEMS syndrome: Are current diagnostic criteria too exclusive?

Abstract

POEMS: polyneuropathy, organomegaly, endocrinopathy, elevated serum M protein, and skin changes P OEMS syndrome is a rare multisystem disease typically associated with monoclonal plasma cell disorders. In 1980, Bardwick et al coined the acronym to embody a syndrome characterized by polyneuropathy, organomegaly, endocrinopathy, elevated serum M protein, and skin changes. No acknowledged set of criteria existed for the diagnosis of the POEMS syndrome; although most authors stipulated that at least 3 or 4 of the 5 features for which the acronym stands were required, ambiguity remained. Current criteria were proposed by Dispenzieri et al, who established that the diagnosis should be based on two essential major variables (polyneuropathy and monoclonal gammopathy) and at least one minor variable: Castleman’s disease, sclerotic bone lesions, organomegaly (splenomegaly, hepatomegaly, lymphadenopathy), edema (peripheral edema, ascites, pleural effusion), endocrinopathy (adrenal, thyroid, pituitary, parathyroid, gonadal, pancreatic), skin changes (cherry, lobular, tufted or glomeruloid type hemangiomas, hyperpigmentation, hypertrichosis, thickened skin, leukonychia) or papilledema. Other features common for this disease, but not included in these criteria, are fever, arthralgia, pulmonary hypertension, fatigue, weight loss, diarrhea, thrombocytosis, polycythemia, and renal involvement. Because of the numerous cutaneous findings in patients with POEMS syndrome, dermatologists should have a thorough knowledge of this entity.