What Is Degos Disease?

Degos disease, also known as malignant atrophic papulosis (MAP), is a relatively rare condition that affects arteries and veins and can hinder circulation. This disease often occurs only on the skin, where it typically causes swelling of and damage to the capillaries beneath the skin. Degos disease usually first manifests as tiny, elevated, inflamed mole-like lesions that cover the torso and upper body. These lesions typically collapse days later, leaving a bright white scar typically ringed in red. Many people with Degos disease suffer only skin symptoms, and can continue to lead normal, relatively healthy lives unless the disease progresses to the organs, in which case it can generally be expected to cause death within two years.

The symptoms of this disease typically begin with lesions of the skin. Many patients live with no further symptoms for many years, and it is not known whether Degos disease always progresses to serious systemic illness. The lesions almost always appear first on the torso, upper body and face, and may then spread to other parts of the body. If the disease becomes systemic, it will usually spread first to the central nervous system or digestive system. From there it can spread to the heart, lungs, and eyes.

Patients have been known to live with Degos disease of the skin for years, or even decades. The condition is not usually considered life-threatening until it begins to affect the organs, so skin symptoms usually aren't treated. Doctors do not understand what causes this condition or how to successfully treat it, so there is little understanding of how to stop the progression of the disease. If the disease spreads to the central nervous system or other organs, it can be deadly in a matter of months. Treatments for the disease include immunosuppressant drugs, aspirin, drugs that stop the action of blood-clotting factors, and drugs to thin the blood.

MAP is believed to have first been identified in 1942, after physicians discovered the first case in 1941. The disease is believed to have a hereditary component, though some physicians have suggested that other factors, such as auto-immune problems, allergies, or infections may contribute to its development. Physicians suspect that Degos disease may have something to do with a malfunction of the body's blood clotting mechanism. Doctors do not, however, have a full understanding of this disease, its cure, or its causes.