Degos disease

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Degos Disease

Degos disease (pronounced: /ˈdeɪɡoʊz/), also known as Kohlmeier-Degos disease or malignant atrophic papulosis, is a rare, systemic, occlusive vascular disease that affects the skin, gastrointestinal tract, and central nervous system.

Etymology

The disease is named after the French dermatologist Robert Degos who first described the condition in 1942. The term "malignant atrophic papulosis" refers to the characteristic skin lesions that occur in this disease.

Symptoms

The primary symptom of Degos disease is the appearance of small, red or white, porcelain-like skin lesions. These lesions typically appear on the torso, arms, and legs. Other symptoms may include abdominal pain, diarrhea, and neurological symptoms such as seizures or loss of consciousness.

Causes

The exact cause of Degos disease is unknown. However, it is believed to be an autoimmune disease, where the body's immune system mistakenly attacks its own tissues. Some researchers believe that a genetic predisposition may also play a role.

Diagnosis

Diagnosis of Degos disease is based on the characteristic skin lesions and other symptoms. A skin biopsy may be performed to confirm the diagnosis. Other tests may include blood tests, imaging studies, and a neurological examination.

Treatment

There is currently no cure for Degos disease. Treatment is aimed at managing symptoms and preventing complications. This may include medications to suppress the immune system, pain management, and supportive care.

Prognosis

The prognosis for individuals with Degos disease varies. Some individuals may have a relatively mild course of the disease, while others may experience severe complications such as intestinal perforation or stroke.

See also

External links

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