Surviving Degos Disease from 32 Mile Media on Vimeo.

This video highlights people being treated for and surviving with systemic Degos disease.

Degos Disease (Malignant Atrophic Papulosis, Köhlmeier-Degos Disease)

Degos Disease is an ultra-rare vascular disease presenting as skin lesions (porcelain plaques) with an erythematous, telangiectatic rim.

Degos patient skin2.jpg

There are cases of cutaneous only lesions, however about 1/3 of patients eventually progress to systemic disease.  

The clinical manifestations of the systemic form most commonly in the Gastrointestinal (GI) Tract and Central Nervous System (CNS). Systemic lesions can also occur in the brain, pericardium, bladder, and eye.  Untreated systemic Degos Disease typically results in gastrointestinal perforations/sepsis and strokes.

What does Degos Disease have to do with Scleroderma?

Potential for overlap with Degos disease can be found in individuals with scleroderma, dermatomyositis, & lupus. Better understanding of Degos Disease may lead to improved therapies & greater insight into the vascular aspect of these other disorders.

Degos Disease Research Centers

 

For more information:

The Degos Disease Support Network:  a patient run informational hub for those affected by Degos disease. www.degosdisease.com 

 

Publications

Degos disease: a C5b-9/interferon-α-mediated endotheliopathy syndrome. Magro CM1, Poe JC, Kim C, Shapiro L, Nuovo G, Crow MK, Crow YJ. Am J Clin Pathol. 2011 Apr;135(4):599-610. doi: 10.1309/AJCP66QIMFARLZKI.

The effects of Eculizumab on the pathology of malignant atrophic papulosis. Magro CM, Wang X, Garrett-Bakelman F, Laurence J, Shapiro LS, DeSancho MT. Orphanet J Rare Dis. 2013 Nov 26;8:185. doi: 10.1186/1750-1172-8-185. PMID: 24279613

Effective treatment of malignant atrophic papulosis (Köhlmeier-Degos disease) with treprostinil--early experience.Shapiro LS, Toledo-Garcia AE, Farrell JF. Orphanet J Rare Dis. 2013 Apr 4;8:52. doi: 10.1186/1750-1172-8-52. PubMed PMID: 23557362; PubMed Central PMCID: PMC3636001.