(Crow-Fukase Syndrome; Takatsuki Disease; PEP Syndrome)
POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, skin changes) is a nonautoimmune polyglandular deficiency syndrome.
POEMS syndrome is probably caused by circulating immunoglobulins caused by a plasma cell dyscrasia. Circulating cytokines (IL-1-β, IL-6), vascular endothelial growth factor, and tumor necrosis factor-alpha are also increased.
Patients may have the following:
Other symptoms and signs of POEMS syndrome may include edema, ascites, pleural effusion, papilledema, and fever. About 15% of patients with POEMS syndrome have associated Castleman disease (a lymphoproliferative disorder, some forms of which are associated with infection by HIV or human herpesvirus 8).
Like other syndromes of undefined pathophysiology, POEMS syndrome is diagnosed based on the constellation of symptoms and signs. Criteria include the presence of polyneuropathy and monoclonal paraproteinemia plus any 2 of the other manifestations of the disorder.