Degos disease, also called Köhlmeier-Degos disease or malignant atrophic papulosis, is an extremely rare condition in which arteries and veins become blocked. People with it develop papules, small raised skin lesions, and the blocked vessels can also damage internal organs, which is where the serious complications arise. The underlying mechanism is still unknown and an effective treatment is still being developed. Fewer than fifty living patients are presently known worldwide and fewer than two hundred have been reported in the medical literature, though some people may go undiagnosed because the disease is so rare. Symptoms most often begin between the ages of twenty and fifty, although cases outside that range have been reported. This entry records the state of knowledge and is not medical advice.
Facts
Classification
ICD-10 ChapterIX: Diseases of the Circulatory System 2 Causative Agent
Causative Agent (category)Idiopathic / Multifactorial 3 Prevalence Band Sources
1. Degos disease (Wikipedia)
Lead sectionQuote, Lead section
extremely rare condition caused by blockage of arteries and veins
View the Source 2. Orphadata (Orphanet): Orphadata cross-references for ORPHA:656071 Degos disease
Orphadata (Orphanet)ICD-10 cross-referenceQuote, ICD-10 cross-reference
"Reference": "I77.8", "Source": "ICD-10"
View the Source 3. National Cancer Institute (NCI Thesaurus): Degos disease - NCI Thesaurus concept C84835
National Cancer Institute (NCI Thesaurus)DefinitionQuote, Definition
A rare disorder of unknown etiology.
View the Source 4. Orphadata (Orphanet): Orphadata epidemiology for ORPHA:656071 Atrophic papulosis
Orphadata (Orphanet)Prevalence, WorldwideQuote, Prevalence, Worldwide
"PrevalenceClass": "\u003c1 / 1 000 000", "PrevalenceGeographic": "Worldwide"
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