Syndromes – POEMS

November 20th, 2010

POEMS Syndrome, also called Crow Fukase Syndrome, is a very rare condition which I have encountered in my The Woodlands dermatology and Conroe dermatology offices.  POEMS stands for: P- Polyneuropathy, O- Organomegaly, E- Endocrinopathy, M- M protein spike, S- Skin changes.  Every patient with POEMS presents with a unique constellation of symptoms from these categories and having signs/symptoms in at least 3 of the 5 of the POEMS categories is needed for a diagnosis of this syndrome.  Most patients are in their 50’s or 60’s when the onset of POEMS occurs and there is a high mortality rate with a 5 year survival rate of 60%.  Men are affected over 2:1 more often than women.  The etiology of POEMS is unclear but current understanding is that VEGF (vascular endothelial growth factor) plays a role in the disease process.  The polyneuropathy involves both motor and sensory nerves but spares the cranial and autonomic nerves.  It usually begins distally (away from the body) and spreads proximally.  Both demyelination and axonal degeneration are present and patients can ultimately become so debilitated that they are bedridden which has a multitude of associated morbidities.  The organomegaly involves the enlargement of the lymph nodes, spleen and liver most commonly and is occasionally associated with Castleman’s Disease in 15% of patients.  A biopsy of the lymph nodes and spleen appears identical to Castleman’s Disease histologically.  Most patients have multiple endocrinopathies in POEMS and elevated estrogen levels can explain many of the endocrine disorders.  Men usually experience low testosterone and develop impotence and gynocomastia (enlargement of breast tissue).  Hypothyroidism, hypoparathyroidism, and diabetes also occur.  A plasma cell dyscrasia occurs that can simulate multiple myeloma.  Patients may experience thrombocytosis (elevated platelets) and serum protein electrophoresis results in an M protein spike which is either IgA or IgM.  The plasma cell dyscrasia can result in bone abnormalities such as osteosclerosis.  Skin changes are very common including angiomas which are red papules that can occur anywhere on the body.  The most common histology of these angiomas is identical to a cherry hemangioma but a rare type of angioma called a glomeruloid hemangioma can also occur.  Patients can also develop hypertrichosis, increased hair production all over their body.  Skin thickening and tightening called sclerodermoid changes is also commonly found.  Other findings include clubbing or whitening of the nails, edema (swelling) of the face and extremities, and hyperhidrosis (excess sweating).  Patients are usually managed by multiple specialists but a hematologist (blood disorder physician) is the primary physician for POEMS as chemotherapy and steroids are usually needed for treatment.