POEMS syndrome with necrotizing vasculitis: A novel feature of vascular abnormalities

Abstract

Polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes (POEMS) syndrome is a rare multisystem disorder. Although its pathogenesis remains elusive, there is evidence that vasculopathy may have an important role.1-3 We report an autopsied case of POEMS syndrome in which necrotizing vasculitis was identified as a salient pathologic feature. A 63-year-old man was admitted to the hospital because of difficulty in walking and a tingling sensation in his legs. On physical examination, edema of the bilateral legs was prominent. Neurologic examination revealed muscle weakness and atrophy of the legs, more marked in the proximal muscles, and deep-tendon reflexes were diminished. Blood cell counts showed mild leukocytosis and thrombocytosis. Tests for autoantibodies such as rheumatoid factor, cryoglobulin, anti-ribonucleoprotein (RNP) antibody, anti-double stranded DNA antibody, anti-SS-A/Ro antibody, and antineutrophil cytoplasmic autoantibody (ANCA) were negative. Serum level of vascular endothelial growth factor (VEGF) was elevated (>2000 pg/mL). Hyperprolactinemia (339.7 ng/mL) was noted. The level of testosterone was decreased (0.4 ng/dL). Estradiol level was elevated (79 ng/mL). There was impaired glucose tolerance. M-protein (IgGλ) was detected in the serum. Motor and sensory conduction velocity of the peripheral nerves was reduced. …