Last updated: February 2024
Also known as…Malignant atrophic papulosis
What is Degos disease?
Degos Disease is a rare progressive condition affecting blood vessels. This causes disease in the skin, gastrointestinal tract and central nervous system.
Who gets Degos disease?
Degos disease is a very rare condition, typically manifesting in young individuals of Caucasian descent. However, cases have been documented in individuals of various ethnicities and across all age groups.
What causes Degos disease?
Degos disease results from blockage of small to medium sized arteries. The cause of the blockage is not well understood. However, abnormal blood clotting, immune abnormalities and viral infections have been suggested as playing a role.
What does Degos disease look like?
The condition may affect only the skin. In other cases, internal organs may also be affected. Skin lesions usually occur before any internal organ involvement is obvious.
Skin involvement
Crops of 30 to 40 pink-red raised small lumps (2 to 5 mm) appear over the upper limbs and torso. The condition progresses over days to weeks with the lumps developing purplish-black crusts and a small depression in the surface. These heal slowly, leaving a white scar with scale on the top. The lumps are usually painless but occasionally may have a slight burning sensation.
Organ involvement
- Gastrointestinal tract – the blockage of blood supply to the gut may cause sudden abdominal pain and bleeding
- Central nervous system – strokes, headaches, memory loss or seizures may occur
- Other organs – eyes and kidneys.
How is Degos disease diagnosed?
A skin biopsy is necessary to confirm the diagnosis. A blood test should be taken to check for any underlying abnormal clotting, as well as a full blood count and iron levels. Stool samples should also be tested for the presence of blood.
How is Degos disease treated?
Treatment options will vary depending on the individual and their needs.
Specific treatment for the skin lesions may include aspirin and other medications such as pentoxyfilline, which affect blood clotting and blood thickness.
A referral to other doctors (e.g., gastroenterologist, neurologist, and ophthalmologist) will be necessary to check for and manage any internal organ involvement.
What is the likely outcome of Degos disease?
Degos disease is commonly considered a severe vascular disorder due to its potential to affect various organs. However, in some cases, when the condition is limited to the skin and appears benign, the likely outcome is good.
| Dr Lauren Young, A/Prof Amanda Saracino and Dr Isaac (SenHong) Lee | February 2024 |
| Dr Lauren Young, Dr Amanda Saracino and Dr Adrian Mar | February 2015 |