Equipping People To Make Sense Of What They Are Told
Degos Disease - An extremely rare vasculopathy. Degos disease affects the lining
of the medium and small veins and arteries.
Also called malignant atrophic papulosis.
The blood vessels are affected and include those supplying the skin, gastrointestinal
tract, and central nervous system. Degos disease also affects the eyes, the intestines,
the brain, and other organs with a variety of physical findings. The main physical
finding of Degos disease is a skin rash that manifests with porcelain white scars.
Occurs more in men than women 3:1
Treatment options are limited and effect of treatment is limited to case reports.
It has been considered that Dagos Disease is not a separate disorder, but the final
result of several vascular systemic disorders.
Bowel ischemia (mesenteric ischemia or ischemic colitis).
Chronic skin lesions.
Ocular lesions.
Strokes.
Spinal lesions.
Mononeuritis multiplex.
Epilepsy.
Headaches.
Cognitive disorders.
Pleural or pericardial effusions are also reported.
The outcome of this disease can be fatal with a median survival of 2 to 3 years.
Although some people appear to have a benign form (Degos acanthoma) which affects
only the skin.
Degos' disease". Acta Clin Belg 63 (2): 99–102.
Katz SK, Mudd LJ, Roenigk HH Jr. Malignant atrophic papulosis (Degos' disease) involving
three generations of a family. J Am Acad Dermatol. Sep 1997;37(3 Pt 1):480-4.
