Degos Disease

Degos disease, also known as malignant atrophic papulosis, causes clogging (occlusion) of small- and medium-size arteries in the body. Degos disease causes distinctive skin lesions and may include gastrointestinal, visual, and nervous system problems. The cause of Degos disease and how it develops in the body are unknown.

Degos disease is rare, but in the known cases females have been affected more often than males. The disease may occur at any age and affects people of all ethnic backgrounds.

Symptoms

• Degos disease usually causes a distinctive rash, which is often its first symptom. The rash develops slowly, beginning as pink or red bumps (papules) that are 2-15 mm in diameter. These papules heal leaving scars with white centers.
• Degos disease may affect the gastrointestinal system, causing abdominal pain, distention, cramps, nausea, vomiting, diarrhea, or constipation.
• Some individuals with Degos disease experience visual problems or drooping eyelids (ptosis).

Some individuals may also experience weakness, shortness of breath, and chest pains.

Diagnosis
The diagnosis of Degos disease is usually made based on the specific skin rash. There is no specific laboratory test for Degos disease. Tests such as endoscopy or magnetic resonance imaging (MRI) may be done to identify problems in the gastrointestinal tract or other systems of the body.

Treatment
There is no specific treatment for Degos disease. Researchers have tried different medications, such as aspirin or dipyridamole, with some success, but no one medication has yet proven always effective in relieving or reducing symptoms. If serious complications such as gastrointestinal bleeding occur, surgery may be necessary. Individuals with Degos disease may benefit from examinations by dermatologists, gastroenterologists, and ophthalmologists.

Last updated 12/16/05

Information for this article was taken from: Scheinfeld, N. S. (2005). Degos disease. eMedicine, accessed at http://www.emedicine.com/derm/topic931.htm