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Degos Disease - Symptoms & Treatment


Degos disease seems to be a disease of the blood vessels - a thrombotic vasculopathy. Degos disease occurs both in a limited benign, cutaneous form and in a lethal multiorgan, systemic variant. Specifically, it is a progressive, small- and medium-size arterial occluding disease, leading to tissue infarction and initially involving the skin. Patients classically develop multiple skin papules that evolve to have a dimpled appearance with a porcelain white center. Degos disease affects the lining of the small blood vessels resulting in occlusion (blockage). The blood vessels affected include those supplying the skin, gastrointestinal tract and central nervous system. Degos disease causes distinctive skin lesions and may include gastrointestinal, visual, and nervous system problems. The cause of Degos disease and how it develops in the body are unknown. The most severe form is Systemic Degos disease affecting children and adolescents with most cases occurring in young adults. Benign Degos disease also affects adults. Complications of the disease include peritonitis, intestinal perforation and, less frequently, cerebral infarction.

Sometimes the disease affects blood vessels in other parts of the body. In some people, the disease stays at this stage and other symptoms do not develop. Some authorities suggest that DD involves a primary endothelial cell defect with secondary thrombosis, leading to infarctive changes. Degos disease has been associated with systemic diseases. DD has occurred in patients with rheumatoid arthritis, HIV infection, and antiphospholipid antibodies and antiphospholipid syndrome. Degos disease is generally regarded as serious because it leads to involvement of multiple organs resulting in death within 2-3 years. But in some cases it remains limited to skin involvement and appears benign. Degos disease usually progresses through two stages. Some individuals with Degos disease experience visual problems or drooping eyelids ( ptosis ). The diagnosis of Degos disease is usually made based on the specific skin rash. There is no specific treatment for Degos disease. There is only symptomatic treatment for the condition although research continues to try to find effective drugs.

Causes of Degos disease

The common causes and risk factor's of Degos disease include the following:

  • The exect cause of Degos disease is unknown.
  • A clotting defect.
  • Abnormality in the clotting system of blood.
  • Viral infection.
  • An immune defect.
  • An allergic vasculitis.

Symptoms of Degos disease

Some sign and symptoms related to Degos diseaseare as follows:
  • Abdominal pain.
  • Diarrhea and chest pains.
  • A distinctive rash.
  • Drooping eyelids ( ptosis ).
  • Weight loss.
  • Shortness of breath.
  • Lesions in the small intestine.
  • Headaches.
  • Eye problems, including diplopia, ptosis, and visual-field defects.

Treatment of Degos disease

Here is list of the methods for treating Degos disease:

  • Anti-platelet drugs like aspirin and dipyridamole may reduce the number of new lesions in some patients with only skin involvement.
  • Individuals with Degos disease may benefit from examinations by dermatologists, gastroenterologists, and ophthalmologists.
  • Treatment with the Er: YAG laser may be a successful strategy in Degos disease.
  • If serious complications such as gastrointestinal bleeding occur, surgery may be necessary.

 

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