- Malignant atrophic papulosis
- Köhlmeier - Degos disease
- Köhlmeier disease
- Degos - Köhlmeier disease
- Degos disease
- Degos syndrome
- Erythrokeratoderma en cocardes
- Thromboangiitis obliterans
The disease is named for Robert Degos who recognized it as a clinical entity in 1942, after it was first described by Kohlmeier in 1941.
What is Degos ?
Degos Disease seems to be a disease of the blood vessels - a thrombotic vasculopathy.
Cells in the linings of the walls of the medium and small veins and arteries under the skin swell when they become inflamed.
This causes the blood flow to be restricted.
Scars left after healing. Lesions on foot had coalesced, broken down and ulcerated. Cellulitis developed with MRSA, and treated with Vancomycin. Ulcers eventually healed after 5 months with daily applications of Manuka honey and then neat Vitamin E (D-Alpha-tocopherol). Scars are sensitive and severe neurological pain has occurred at intervals, treated with Ketamine.
Scars left after healing. Lesions on foot had coalesced, broken down and ulcerated. Cellulitis developed with MRSA, and treated with Vancomycin. Ulcers eventually healed after 5 months with daily applications of Manuka honey and then neat Vitamin E (D-Alpha-tocopherol). Scars are sensitive and severe neurological pain has occurred at intervals, treated with Ketamine.
Smaller groups of lesions, now about 8 years old - all scars and no sensitivity at all.
Lesions about 8 years old. Many have coalesced. Some still become inflamed but most are now scars. The white atrophic centres are flaky.
Lesions about 8 years old. Many have coalesced. Some still become inflamed but most are now scars. The white atrophic centres are flaky.
Where this happens ?
spots (lesions) appear on the skin. They are small and red, slightly raised.
As they develop, the centre becomes dry and white (atrophic). Sometimes the spots itch.
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.
The outcome of this disease can be fatal with a median survival of 2 to 3 years, although some appear to have a benign form (Degos acanthoma) which affects only the skin. There are fewer than fifty living patients at present known worldwide, and less than 200 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.
Sometimes the disease affects blood vessels in other parts of the body. Most commonly, the gut, the central nervous system or the eyes are involved.
Most case histories in the medical literature are of ‘worst case’ scenarios.
Don't get panic?
You will read statistics and numbers which might scare you - but they are often based on projections and on published case reports. There are lots of Degos patients whose cases haven’t been written up and who are alive and well.
There are also lots of confident assertions which just aren’t true! For example: “This disease affects mainly young men” - not in our experience! Or “The lesions don’t appear on the soles of the feet” - wrong again! Percentages and actual numbers can’t be right, as no-one has compared living patients until now. Take it all with a pinch of salt and add your experience to our site and to the data being collected in Dessau,Germany.
Other names for Degos disease are:
Post Title
→Degos Disease...Extremely Rare Vasculopathy !!
Post URL
→https://shoocers.blogspot.com/2010/10/degos-diseaseextremely-rare.html
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