POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes) syndrome
B. Serum vascular endothelial growth factor
POEMS syndrome is a rare multisystem disorder caused by a disorder of plasma cells and is theorized to result from an overproduction of proinflammatory cytokines and vascular endothelial growth factor (VEGF).1,2 Diagnosis requires the presence of all mandatory criteria, 1 major criteria, and 1 minor criteria. Mandatory criteria include polyneuropathy and monoclonal plasma cell disorder; major criteria include osteosclerotic lesions, Castleman disease, or elevated VEGF; and minor criteria include organomegaly, volume overload, endocrinopathy, skin changes, papilledema, or thrombocytosis.2
Studies have shown that about half of patients with POEMS syndrome have optic disc edema, which is typically bilateral with intact vision and can mimic papilledema caused by elevated intracranial pressure (ICP).3 However, at least half of patients with bilateral optic disc edema in the setting of POEMS syndrome will have normal ICP.3 Therefore, the etiology of the optic disc edema in POEMS syndrome is unclear, but high systemic VEGF levels are thought to be contributory, potentially causing increased vascular permeability.3 In addition to optic disc edema, other more rare ocular manifestations of POEMS syndrome include macular edema, serous macular detachment, uveitis, and neovascularization of the disc and choroid.4