Disease Information for Degos' acanthomatosis syndrome

Degos Disease; Degos Syndrome; Degos-Kohlmeier Disease;

Kohlmeier-Degos Disease; Malignant Atrophic Papulosis

Degos disease is a rare systemic disorder that affects small and medium sized arteries, causing them to become blocked (occlusive arteriopathy); Degos disease usually progresses through two stages: During the first stage, characteristic skin lesions appear that may last for a period of time ranging from weeks to years; The second stage of Degos disease is most frequently characterized by lesions in the small intestine, but other organs may also be affected; Major symptoms may include abdominal pain, diarrhea, and/or weight loss; Intestinal lesions may break through the wall of the bowel (perforation), a potentially life-threatening complication; The exact cause of Degos disease is unknown; ----------[NORD 2005]----------------------------------

Degos disease, also known as malignant atrophic papulosis is a rare disorder. It generally occurs in young Caucasian adults; Degos disease affects the lining of the small blood vessels resulting in occlusion (blockage); The blood vessels affected include those supplying the skin, gastrointestinal tract and central nervous system;

Skin lesions are the characteristic feature of Degos disease. The lesions are usually multiple and occur predominantly on the trunk and arms; They usually start as small red raised spots of 2-5mm in diameter; After a few days they enlarge and develop a central white spot that is depressed in comparison to the red skin around it; They heal leaving depressed porcelain-white scars;

Gastrointestinal complications result from lack of blood supply to the lining of the gut and may lead to serious complications like perforation of the bowel; The usual symptoms are sudden onset of abdominal pain or gastrointestinal bleeding (vomiting blood or passing blood with the bowel motion);Involvement of the nervous system can lead to strokes, headaches, epilepsy or non-specific neurological symptoms like memory loss or altered sensation;

Other organs can also be involved such as eyes, kidneys, heart and liver. Prognosis: Degos disease is generally regarded as serious because it leads to involvement of multiple organs resulting in death within 2-3 years; But in some cases it remains limited to skin involvement and appears benign (harmless);

[DermNet NZ 2005]---------------------------------------------

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