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Degos Disease

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Acute

What exactly is Degos Disease?

Malignant atrophic papulosis (Degos' disease) is a rare vascular disorder characterized by distinctive skin lesions associated with multiple GI infarctions. Skin lesions typically consist of red papules that become umbilicated in the center. The center eventually becomes porcelain white and atrophic.

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Some of these lesions may ultimately perforate. Major symptoms prior to perforation include abdominal pain, diarrhea and/or weight loss. This systemic form of Degos disease can lead to other disabling and potentially life-threatening complications if the central nervous system, heart, or lung are involved.

The most common organ systems affected are the CNS and gastrointestinal tract. The most frequent causes of death include cerebral infarction, intestinal perforation, and secondary peritonitis. Complications of Degos disease include the following: Gastrointestinal infarcts, bleeding, and perforation.

Degos disease is caused by multiplication of the cells lining small arteries, resulting in the narrowing or blocking of affected vessels termed arterial occlusion. Areas of severely damaged tissue (necrosis) may appear in locations where these affected arteries restrict blood flow, known as occlusive arteriopathy

Administration of medications that inhibit increased platelet aggregation (especially aspirin and dipyridamole) may be helpful in some cases. Immunosuppression may worsen the skin lesions of Degos disease.

Degos disease is caused by multiplication of the cells lining small arteries, resulting in the narrowing or blocking of affected vessels termed arterial occlusion. Areas of severely damaged tissue (necrosis) may appear in locations where these affected arteries restrict blood flow, known as occlusive arteriopathy.

How is it diagnosed?

How is it diagnosed?

Fibrinolytic and immunosuppressive therapeutic regimens were tested and found to be mostly unsuccessful as treatment methods. After treating conditions comorbid with Degos disease, physicians have recently found improvement in symptoms with the use of eculizumab and treprostinil.

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How is it treated?

Treatment for acute myeloid leukemia is vital. It varies with the patient and stage of the disease. Treatment options include

Degos disease most frequently presents in white male teenagers, with the initial symptom being skin lesions on the trunk (Figure 1). Sometimes, within days, the patient presents with generalized acute abdominal pain and fever. Patients in this situation are frequently treated with surgery (exploratory laparotomy).

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