What is the definition of 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. The cause of this condition is currently unknown.

What are the alternative names for Degos Disease?

  • Degos's malignant atrophic papulosis
  • Atrophic papulosis, malignant
  • Kohlmeier-Degos disease
  • Köhlmeier-Degos disease
  • Papulosis atrophican maligna
  • Degos disease
  • Köhlmeier-Degos-Delort-Tricort syndrome

What are the causes for 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.

What are the symptoms for 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). 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.

What are the current treatments for Degos Disease?

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. These treatments were reported to be effective in some patients. 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. Infliximab was reported to be ineffective in one case. Immunosuppressives such as corticosteroids may worsen Degos disease.For further information on your treatment options, we encourage you to discuss your questions and this information with your healthcare provider.

What is the outlook (prognosis) for 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.
  • Journal: Orphanet journal of rare diseases
  • Published —
Clinical and laboratory prognosticators of atrophic papulosis (Degos disease): a systematic review.
  • Journal: Journal of the European Academy of Dermatology and Venereology : JEADV
  • Published —
Clinical manifestations and treatment outcomes in degos disease: a systematic review.

There are no recent clinical trials available for this condition. Please check back because new trials are being conducted frequently.