Walsh & Hoyt: POEMS Syndrome (Crow-Fukase Syndrome)

POEMS syndrome is an unusual multisystem disorder that is characterized by Polyneuropathy,Organomegaly, Endocrinopathy, Monoclonal gammopathy, and Skin changes. Polyneuropathy is frequently the initial and most disabling problem. It is usually associated with electrophysiologic and histologic evidence of mixed demyelination and axon degeneration. Enlarged organs include the liver, spleen, and kidneys. Endocrine abnormalities are diverse and include hypothyroidism, hypogonadism, hypocortisolism, and gynecomastia in men. Polycythemia is often present. Skin findings include diffuse hyperpigmentation in 93% of patients, edema of the legs in 91%, hypertrichosis in 80%, skin thickening similar to that observed in scleroderma in 77%, hyperhidrosis in 66%, and clubbing of the fingers. Rare patients have cutaneous angiomas. Additional systemic features include osteosclerotic bone lesions, lymphadenopathy, and peripheral edema. Neurologic manifestations in addition to polyneuropathy include increased ICP, papilledema, and pupillary light-near dissociation. In addition, optic disc drusen with peripapillary choroidal neovascularization has been associated with POEMS syndrome. Some patients develop an empty sella syndrome from chronically raised ICP.