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Degos Disease

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Degos Disease Important
It is possible that the main title of the report Degos Disease 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

  • Degos syndrome
  • Degos-Kohlmeier disease
  • Kohlmeier-Degos disease
  • malignant atrophic papulosis
  • MAP
  • papulosis atrophicans maligna

Disorder Subdivisions

  • None

General Discussion


Degos disease is an extremely rare disorder in which small and medium sized arteries become blocked (occlusive arteriopathy), restricting the flow of blood to affected areas. Degos disease usually causes characteristic skin lesions that may last for a period of time ranging from weeks to years. In some individuals, Degos disease will be limited to the skin (benign cutaneous Degos disease); other individuals will also develop symptoms affecting other organ systems (systemic Degos disease). Systemic Degos disease is most frequently characterized by lesions in the small intestine, but other organs are also affected. Major symptoms may include abdominal pain, diarrhea, and/or weight loss. The systemic form of Degos disease can cause life-threatening complications such as perforated bowels leading to inflammation of the abdominal cavity (peritonitis). The exact cause of Degos disease is unknown.

Many researchers caution that statistics concerning Degos disease are inaccurate because many individuals go undiagnosed and most medical reports disproportionately discuss the more serious systemic form. It is important to note that some individuals only develop skin lesions (which are not associated with life-threatening complications) and do not go on to develop systemic Degos disease. Affected individuals should talk to their physicians and medical team about their specific case and associated symptoms.

Resources


Digestive Disease National Coalition
507 Capitol Court, NE
Suite 200
Washington, DC 20002
Tel: (202)544-7497
Fax: (202)546-7105
Email: ddnc@hmcw.org
Internet: http://www.ddnc.org

NIH/National Institute of Diabetes, Digestive & Kidney Diseases
Office of Communications & Public Liaison
Bldg 31, Rm 9A06
31 Center Drive, MSC 2560
Bethesda, MD 20892-2560
Tel: (301)496-3583
Email: NDDIC@info.niddk.nih.gov
Internet: http://www2.niddk.nih.gov/

Degos Patients' Support Network
28 Glenville Road
Rustington
West Sussex, BN16 2EA
United Kingdom
Tel: 441903787737
Fax: 441903772954
Email: judith@degosdisease.com
Internet: http://www.degosdisease.com

Genetic and Rare Diseases (GARD) Information Center
PO Box 8126
Gaithersburg, MD 20898-8126
Tel: (301)251-4925
Fax: (301)251-4911
Tel: (888)205-2311
TDD: (888)205-3223
Internet: http://rarediseases.info.nih.gov/GARD/

Madisons Foundation
PO Box 241956
Los Angeles, CA 90024
Tel: (310)264-0826
Fax: (310)264-4766
Email: getinfo@madisonsfoundation.org
Internet: http://www.madisonsfoundation.org

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