Degos disease

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Degos disease
Classification and external resources
Degos-disease.jpg
Skin lesions in a person with Degos disease
ICD-9 447.8
OMIM 602248
DiseasesDB 29425
eMedicine derm/931
MeSH D054853

Degos disease (also called malignant atrophic papulosis) is a rare vasculopathy that affects the lining of the medium and small veins and arteries, resulting in occlusion (blockage of the vessel) and tissue infarction.[1]

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.[2]

There are fewer than 50 living patients at present known worldwide, and fewer than 200[2] reported in medical literature.

The outcome of this disease can be fatal with a median survival of 2 to 3 years,[2] although some appear to have a benign form (Degos acanthoma) which affects only the skin. Laparoscopy may be an effective means of screening those with cutaneous lesions to determine if systemic disease is present. Treatment options are limited, and until recently consisted mainly of Antiplatelet drugs or anticoagulants or immunosuppressants with few reports of prolonged survival. More recently eculizumab and treprostinil have been employed. Response to eculizumab is often immediate and dramatic, but has been of limited duration in all so treated. Treprostinil use has been reported to result in clearing of gastrointestinal and CNS findings as well as clearing of cutaneous lesions, but reports are limited. Treprostinil may be more effective than other vasodilators because it may also increase the population of circulating endothelial cells, allowing angiogenesis.

It has been suggested that Degos disease is not a separate disorder, but the final result of several vascular systemic disorders.[3][4]

History[edit]

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.[5][6]

References[edit]

Notes[edit]

  1. ^ Athanasios Theodoridis, Evgenia Makrantonaki, Christos C Zouboulis (2013). "Malignant atrophic papulosis (Köhlmeier-Degos disease) - A review". Orphanet Journal of Rare Diseases 8 (10). 
  2. ^ 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. doi:10.1179/acb.2008.63.2.007. PMID 18575050. 
  3. ^ 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. 
  4. ^ 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. 
  5. ^ synd/3466 at Who Named It?
  6. ^ Degos R., Delort J., Tricot R. (1942). "Dermatite papulosquameuse atrophiante". Bulletin de la Société française de dermatologie et de syphiligraphie et de ses filiales 49: 148–150. 

Further reading[edit]

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