- Degos disease
-
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]
References
Notes
- ^ a b c De Breucker S, Vandergheynst F, Decaux G (2008). "Inefficacy of intravenous immunoglobulins and infliximab in Degos' disease". Acta Clin Belg 63 (2): 99–102. PMID 18575050.
- ^ High WA, Aranda J, Patel SB, Cockerell CJ, Costner MI (June 2004). "Is Degos' disease a clinical and histological end point rather than a specific disease?". J. Am. Acad. Dermatol. 50 (6): 895–9. doi:10.1016/j.jaad.2003.11.063. PMID 15153891.
- ^ Ball E, Newburger A, Ackerman AB (August 2003). "Degos' disease: a distinctive pattern of disease, chiefly of lupus erythematosus, and not a specific disease per se". Am J Dermatopathol 25 (4): 308–20. doi:10.1097/00000372-200308000-00005. PMID 12876488. http://meta.wkhealth.com/pt/pt-core/template-journal/lwwgateway/media/landingpage.htm?issn=0193-1091&volume=25&issue=4&spage=308.
- ^ synd/3466 at Who Named It?
- ^ R. Degos, J. Delort, R. Tricot. Dermatite papulosquameuse atrophiante. Bulletin de la Société française de dermatologie et de syphiligraphie et de ses filiales, 1942, 49, 148-150.
Further reading
- Scheinfeld N. Malignant atrophic papulosis. Clinical & Experimental Dermatology. September 2007;5:483-487.
External links
Categories:- Vascular-related cutaneous conditions
- Vascular diseases
- Rare diseases
Wikimedia Foundation. 2010.
