| Degos disease | |
|---|---|
| Classification and external resources | |
| ICD-9 | 447.8 |
| OMIM | 602248 |
| DiseasesDB | 29425 |
| eMedicine | derm/931 |
| MeSH | D054853 |
Degos disease (also called malignant atrophic papulosis) is an extremely rare vasculopathy that affects the lining of the medium and small veins and arteries, resulting in occlusion (blockage of the vessel) and tissue infarction.
The blood vessels affected include those supplying the skin, gastrointestinal tract, and central nervous system. This can result in bowel ischemia (mesenteric ischemia or ischemic colitis), chronic skin lesions, ocular lesions, strokes, spinal lesions, mononeuritis multiplex, epilepsy, headaches or cognitive disorders. Pleural or pericardial effusions are also reported.[1]
The outcome of this disease can be fatal with a median survival of 2 to 3 years,[1] although some appear to have a benign form (Degos acanthoma) which affects only the skin. There are fewer than 50 living patients at present known worldwide, and fewer than 200[1] reported in the medical literature. Treatment options are limited, consist mainly of Antiplatelet drugs or anticoagulants or immunosuppressants, and effect of treatment is limited to case reports.
It has been suggested that this is not a separate disorder, but the final result of several vascular systemic disorders.[2][3]
The disease is named for Robert Degos who recognised it as a clinical entity in 1942, after it was first described by Kohlmeier in 1941.[4][5]
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