Degos disease has benign and malignant variants. The disease is extremely rare to the degree that some researchers believe that it is not a separate disease in itself but rather an outcome of other disorders.
In medical literature the malignant form of disease is also called malignant atrophic papulosis. The disease mainly affects the vascular structures of the human body causing a blockade in it. The malignancy disrupts both arterial and venous systems. As the blood carrying vessels get blocked, the oxygen supply to the tissue is stopped. Gradually the tissue starts dying and there resides the lethality of the disease. The survival rate of the patients carrying the disease is very low. A rough estimate about the surviving patients globally is around 50 people only. As the disease does not remain confined to a particular tissue or area, it causes problems in abdominal viscera, skin, and heart tissue. As the arteries get obstructed there is a chance of stroke and epilepsy also.
The benign variant of the Degos disease affects skin tissue only and causes lesions on the skin.
The treatment options are very limited as the etiology of the disease is not fully understood. The most commonly prescribed regimen consists of blood thinning agents, so as to avoid the risk of ischemia. As the disease is thought to have its origins in the immune system and hyperactive immune system is believed to be responsible for causing the complications as the disease progresses, often immunosuppressant drugs are prescribed.
Degos Disease
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