A multisystemic disorder characterized by a sensorimotor polyneuropathy (polyneuropathies), organomegaly, endocrinopathy, monoclonal gammopathy, and pigmentary skin changes. Other clinical features which may be present include edema; cachexia; microangiopathic glomerulopathy; pulmonary hypertension (hypertension, pulmonary); cutaneous necrosis; thrombocytosis; and polycythemia. This disorder is frequently associated with osteosclerotic myeloma. (From Adams et al., Principles of neurology, 6th ed, p1335; Rev Med Interne 1997;18(7):553-62)


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