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Diseases

Genetic and Rare Diseases Information Center (GARD)

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Degos disease


Other Names for this Disease
  • Atrophic papulosis, malignant
  • Degos syndrome
  • Degos's malignant atrophic papulosis
  • Kohlmeier-Degos disease
  • Malignant atrophic papulosis
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Overview



What is Degos disease?

What are the signs and symptoms of Degos disease?

What causes Degos disease?

How might Degos disease be treated?

What is the typical prognosis (long term outlook) for people with Degos disease?



What is Degos disease?

Degos disease is a rare blood vessel disorder. It is characterized by blockages of small to medium sized blood vessels.  This slows or stops the flow of blood through the affected vessels. Severity of symptoms depends on the extent and location of the affected blood vessels. Some individuals with Degos disease have isolated skin involvement and develop porcelain-white macules on their skin. Other individuals have more wide spread disease. Multiorgan disease can become life threatening.[1] The cause of this condition is currently unknown.
Last updated: 3/31/2011

What are the signs and symptoms of Degos disease?

Signs and symptoms of Degos disease can vary greatly from person to person.  Skin symptoms include porcelain-white macules that tend to develop on the trunk, arms, and legs. For some people, this is their only symptom. For others, Degos disease affects multiple body organs.  

Skin macules tend to be the earliest symptom in multisystem disease. Small bowel involvement is very common, and Degos disease can cause intestinal perforation (tear).[2] Intestinal perforation is a medical emergency which requires prompt treatment. Click here to learn more about the signs and symptoms of intestinal perforation.  Unfortunately intestinal disease tends to recur in these individuals.

Degos disease can also affect the nervous system, in particular the cerebral and peripheral nerves. This may result in a variety of symptoms, such as partial paralysis, aphasia (difficulty communicating), cranial neuropathies (which affect nerves that are connected with the brain and control sight, eye movement, hearing, and taste), sensory disturbances, and seizures.[2]
Last updated: 6/6/2011

What causes Degos disease?

Currently the cause of Degos disease is not known. Similar skin macules have been described in people with systemic lupus erythematosus and in a patient without lupus who had anticardiolipin antibodies and lupus anticoagulant.[2]
Last updated: 3/31/2011

How might Degos disease be treated?

Currently, there is not a targeted therapy for Degos disease that has been proven effective. Treatment of Degos disease has been attempted with antithrombotic agents, such as aspirin and dipyridamole.[2] These treatments were reported to be effective in some patients.[2][3] Other treatments that have been tried, but have shown inconsistent results, include anticoagulants and fibrinolytic agents (drugs to help break-up and dissolve clots), ticlopidine, pentoxifylline, prostaglandin E1, and interferon alpha-2a. Treatment with intravenous immunoglobulin has also been tried, but produced conflicting results.[3] Infliximab was reported to be ineffective in one case.[3] Immunosuppressives such as corticosteroids may worsen Degos disease.[3]

For further information on your treatment options, we encourage you to discuss your questions and this information with your healthcare provider.
Last updated: 10/26/2011

What is the typical prognosis (long term outlook) for people with Degos disease?

Prognosis of individuals with Degos disease varies considerably depending on the extent of blood vessel involvement. Individuals with isolated skin macules have a good prognosis, while individuals with multisystem disease often face life threatening complications.  To learn more about your or your loved one’s prognosis we strongly recommend that you speak with a healthcare provider.
Last updated: 3/31/2011

References
  1. Malignant Atrophic Papulosis. MeSH. http://www.nlm.nih.gov/cgi/mesh/2011/MB_cgi?mode=&index=24677&view=expanded. Accessed 3/31/2011.
  2. Mark LA, Mirowski GW. Oral Disease and Oral-Cutaneous Manifestations of Gastrointestinal and Liver Disease. In: Feldman eds. Sleisenger and Fordtran's Gastrointestinal and Liver Disease, 9th ed. Philadelphia, PA: Saunders; 2010;
  3. Cheng TS. A man with generalized small white skin lesions and abdominal pain. Int J Dermatol. 2011 Jun;50(6):726-9; http://www.ncbi.nlm.nih.gov/pubmed/21595670. Accessed 10/26/2011.
  4. Wilson J . Benign cutaneous Degos disease in a 16-year-old girl. Pediatr Dermatol. 01-JAN-2007;