POEMS Syndrome

Important

It is possible that the main title of the report POEMS Syndrome is not the name you expected. Please check the synonyms listing to find the alternate name(s) and disorder subdivision(s) covered by this report.

Synonyms

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• Crow-Fukase Syndrome
• PEP Syndrome
• Shimpo syndrome
• Takatsuki syndrome
• Polyneuropathy-organomegaly-endocrinopathy-M protein-skin lesions

Disorder Subdivisions

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• None

General Discussion

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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.



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.

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Resources

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American Autoimmune Related Diseases Association, Inc.

22100 Gratiot Avenue

Eastpointe, MI 48021-2227

Tel: (586)776-3900

Fax: (586)776-3903

Tel: (800)598-4668

Email: aarda@aarda.org

Internet: http://www.aarda.org/



NIH/Developmental Endocrinology Branch

Bldg 10, Room 10N262

10 Center Drive

Bethesda, MD 20892

Tel: (301)496-4686

Fax: (301)402-0574



NIH/National Arthritis and Musculoskeletal and Skin Diseases Information Clearinghouse

1 AMS Circle

Bethesda, MD 20892-3675

USA

Tel: 3014954484

Fax: 3017186366

Tel: 8772264267

TDD: 3015652966

Email: NIAMSinfo@mail.nih.gov

Internet: http://www.niams.nih.gov



National Institute of Neurological Disorders and Stroke (NINDS)

31 Center Drive

8A07

Bethesda, MD 20892-2540

Tel: (301)496-5751

Fax: (301)402-2186

Tel: (800)352-9424

Email: braininfo@ninds.nih.gov

Internet: http://www.ninds.nih.gov/



Neuropathy Association

60 East 42nd Street

Suite 942

New York, NY 10165

USA

Tel: 2126920662

Fax: 2126920668

Email: info@neuropathy.org

Internet: http://www.neuropathy.org



Jack Miller Center for Peripheral Neuropathy

University of Chicago

5841 S. Maryland Ave, MC 2030

Chicago, IL 60637

Tel: (773)702-5800

Fax: (773)702-5577

Email: information-millercenter@neurology.bsd.uchicago.edu

Internet: http://millercenter.uchicago.edu

For a Complete Report

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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

The information provided in this report is not intended for diagnostic purposes. It is provided for informational purposes only. NORD recommends that affected individuals seek the advice or counsel of their own personal physicians.

It is possible that the title of this topic is not the name you selected. Please check the Synonyms listing to find the alternate name(s) and Disorder Subdivision(s) covered by this report.

This disease entry is based upon medical information available through the date at the end of the topic. Since NORD's resources are limited, it is not possible to keep every entry in the Rare Disease Database completely current and accurate. Please check with the agencies listed in the Resources section for the most current information about this disorder.

For additional information and assistance about rare disorders, please contact the National Organization for Rare Disorders at P.O. Box 1968, Danbury, CT 06813-1968; phone (203) 744-0100; web site www.rarediseases.org or email orphan@rarediseases.org

Last Updated:   3/8/2004

Copyright   1991, 2000, 2002, 2003, 2004 National Organization for Rare Disorders, Inc.