Diseases & Conditions


A B C D E F G H I J K L M N O P Q R S T U V W X Y Z

POEMS Syndrome


Synonyms of POEMS Syndrome
  • Crow-Fukase Syndrome
  • PEP Syndrome
  • poem's
  • Polyneuropathy-organomegaly-endocrinopathy-M protein-skin lesions
  • Polyneuropathy-organomegaly-endocrinopathy-M protein-skin lesions
  • Shimpo syndrome
  • Takatsuki syndrome

Disorder Subdivisions



    General Discussion
    POEMS syndrome is an extremely rare multisystem disorder. POEMS is an acronym that stands for (P)olyneuropathy, disease affecting many nerves; (O)rganomegaly, abnormal enlargement of an organ; (E)ndocrinoapthy, disease affecting certain hormone-producing glands that help to regulate the rate of growth, sexual development, and certain metabolic functions (endocrine system); (M)onoclonal gammopathy or M proteins; and (S)kin defects. Common symptoms include progressive weakness of the nerves in the arms and legs, an abnormally enlarged liver and/or spleen (hepatosplenomegaly), abnormally darkening of the skin (hyperpigmentation) and excessive hair growth (hypertrichosis). Endocrine abnormalities such as failure of the ovaries and testes (gonads) to function properly (primary gonadal failure) and diabetes mellitus type I may be present. Specific endocrine abnormalities associated with POEMS syndrome vary from case to case. Other important features of the disease include swelling around the optic nerve (papilledema), abnormal fluid retention, which may occur in the ankles (edema), the abdominal cavity (ascites), or around the lungs (pleural effusions), painless scars on bone x-ray (osteosclerosis), and an elevated platelet count (a blood cell responsible for clotting). Some patients may also have abnormalities in the lung function tests, which may not be noticed because of their inability to walk well due to their neuropathy.

    POEMS syndrome is associated with a group of disorders known as monoclonal gammopathies or plasma cell dyscrasias. These disorders are characterized the uncontrolled growth of a single clone (monoclonal) of plasma cells, which results in the abnormal accumulation of M-proteins (also known as immunoglobulin M or IgM) in the blood. M-proteins are supposed to fight foreign substances in the body such as viruses and bacteria, but researchers suspect that they play a role in the development of POEMS syndrome. However, the specific role M-proteins play and the exact cause of POEMS syndrome is unknown. Research would suggest that a chemical called VEGF (vascular endothelial growth factor) plays an important role in this disease.

    Organizations related to POEMS Syndrome
    • American Autoimmune Related Diseases Association, Inc.
      22100 Gratiot Avenue
      Eastpointe MI 48021
      Phone #: 586-776-3900
      800 #: 800-598-4668
      e-mail: aarda@aarda.org
      Home page: http://www.aarda.org/
    • AutoImmunity Community
      None
      None None None
      Phone #: (91-9) -552-9057
      800 #: N/A
      e-mail: bandrews@autoimmunitycommunity.org
      Home page: http://autoimmunitycommunity.org
    • Jack Miller Center for Peripheral Neuropathy
      University of Chicago
      Chicago IL 60637
      Phone #: 773-702-5800
      800 #: N/A
      e-mail: information-millercenter@neurology.bsd.uchicago.edu
      Home page: http://millercenter.uchicago.edu
    • NIH/Developmental Endocrinology Branch
      Bldg 10, Room 10N262
      Bethesda MD 20892
      Phone #: 301-496-4686
      800 #: --
      e-mail: N/A
      Home page: N/A
    • NIH/National Arthritis and Musculoskeletal and Skin Diseases Information Clearinghouse
      1 AMS Circle
      Bethesda MD 20892-3675
      Phone #: 301-495-4484
      800 #: 877-226-4267
      e-mail: NIAMSinfo@mail.nih.gov
      Home page: http://www.niams.nih.gov/Health_Info
    • National Institute of Neurological Disorders and Stroke (NINDS)
      31 Center Drive
      Bethesda MD 20892-2540
      Phone #: 301-496-5751
      800 #: 800-352-9424
      e-mail: braininfo@ninds.nih.gov
      Home page: http://www.ninds.nih.gov/
    • Neuropathy Association
      60 East 42nd Street
      New York NY 10165
      Phone #: 212-692-0662
      800 #: N/A
      e-mail: info@neuropathy.org
      Home page: http://www.neuropathy.org



    For a Complete Report

    This is an abstract of a report from the National Organization for Rare Disorders, Inc. ? (NORD). A copy of the complete report can be obtained for a small fee by visiting the NORD website. The complete report contains additional information including symptoms, causes, affected population, related disorders, standard and investigational treatments (if available), and references from medical literature. For a full-text version of this topic, see http://www.rarediseases.org/search/rdblist.html