Degos disease – what it is, causes, symptoms and treatments!

Degos disease (malignant atrophic papulosis) is most often characterized by lesions in the small intestine, but other organs are also affected.

Major symptoms can include abdominal pain, diarrhea, and/or weight loss. The systemic form of degos disease  can cause life-threatening complications such as perforated bowels leading to inflammation of the abdominal cavity (peritonitis).

What is degos disease?

Degos disease  (malignant atrophic papulosis) is an extremely rare disorder in which small and medium-sized arteries become blocked (occlusive arteriopathy), restricting blood flow to affected areas. Degos disease  usually causes characteristic skin lesions that can last for a period of time ranging from weeks to years. In some individuals, it will be limited to the skin (benign cutaneous); other individuals will also develop symptoms that affect other organ systems (systemic Degos disease).

Causes of degos disease :

The exact cause is unknown. The disease process causes the cells lining the arteries to multiply, which contributes to the narrowing or blockage of the arteries (arterial occlusion). Areas of severely damaged tissue (necrosis) can appear when narrowed or blocked arteries restrict blood flow (occlusive arteriopathy). The effects of degos disease  depend on the location of blocked arteries and necrotic lesions.

Many theories have been proposed as to what the underlying cause is. Three main theories include (1) viral infection, (2) defects in the body’s blood clotting ability (primary clotting disorder), and (3) autoimmunity, whereby the body’s immune system mistakenly attacks healthy tissue from water . .

Some cases of degos disease have run in families suggesting that a genetic predisposition to the disorder may exist in these cases. Furthermore, the mode of inheritance of this familial variant is unknown. Interestingly, this form of degos disease  is usually limited to the skin (benign cutaneous).

Signs and symptoms of degos disease :

In most cases, the initial symptoms are distinct skin lesions or a rash. Affected individuals develop small bumps or patches (papules) of varying shapes, usually on the trunk and arms and upper legs. Also, initially only a few lesions may be apparent.

Eventually, 10 to 40 lesions may slowly develop, and in some cases, hundreds may develop. The palms, soles and face are usually spared. The lesions can sometimes itch (pruritus). In addition, the lesions begin as reddish or pinkish bumps and eventually the center degenerates (atrophy), so that older lesions have a reddish border with white-porcelain atrophic centers.

Treatments for degos disease :

No specific therapy has been identified for. In addition, treatment is directed towards the specific symptoms that are apparent in each individual.

In diagnosed cases, examination of the gastrointestinal tract on a regular basis can detect intestinal perforation before symptoms of acute complications (ie, peritonitis) appear. Some associated complications, such as gastrointestinal bleeding or intestinal perforation, may require surgical intervention.

Investigative Therapies:

Drugs that inhibit the activity of platelets (specialized blood cells that clump together to form clots to stop bleeding at the site of injury) have been used to treat individuals with degos disease . These drugs are known as antiplatelet drugs or anticoagulants. In addition, a combination of two of these drugs (ie, aspirin and dipyridamole) was reported to improve skin and eye lesions in two subjects who had no systemic involvement.

A variety of medications, such as those that suppress the body’s immune system (immunosuppressants), have been tried with no positive effect. In addition, a case reported in the medical literature demonstrated improvement in skin lesions and gastrointestinal symptoms with the administration of nicotine patches.

Useful links: 

In addition, some researchers advocate the use of intravenous immunoglobulin as a treatment for affected individuals. More research is needed to determine the long-term safety and effectiveness of such therapies in individuals with degos disease .

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