What is Degos Disease?
The information provided on this website is designed to support, not replace, the relationship that exists between a patient/site visitor and their physician.
Disclaimer: While Degos disease and scleroderma share some similarities, such as small blood vessel damage and fibrosis affecting organs like the heart and lungs, having scleroderma does not mean you will develop Degos disease.
Why we mention Degos disease: The Degos Disease Foundation is an integral part of the Steffens Scleroderma Foundation. Our connection began with Ernie DuPont (read their full story here), son of a former board president, who bravely fought Degos disease until his passing in 2018. His physician, Dr. Shapiro—now our Chief Medical Officer along with all the other doctors on our Medical Leadership Team—continue the fight. Ernie’s legacy drives our commitment to advancing research, education, and treatment for both scleroderma and Degos disease. Degos disease shares potential overlap with scleroderma, dermatomyositis, and lupus. A deeper understanding of one could improve treatments and shed light on the vascular aspects of them all.
Visit DegosDisease.org to learn more about Degos disease and Degos Disease Foundation.
Degos disease almost always presents with skin lesions with a very distinct appearance, a porcelain-white center with a red rim consisting of clusters of blood vessels.
Degos disease, also known as Malignant Atrophic Papulosis (MAP), Atrophic Papulosis, or Kohlmeier-Degos disease is an “ultra-rare” disease which can exist in a benign, cutaneous (skin only) or systemic form.
But, Degos disease can affect any age group.
The systemic form of Degos disease affects one or more body organs, most commonly the gastrointestinal tract or brain. Complications can include bowel perforation and a variety of neurologic and visual symptoms.
For patients where skin lesions are missed or not noted, no diagnosis or a misdiagnosis is common, which could lead to delays or mistakes in treatment. For those with systemic disease, serious complications could happen fast and even lead to death.
We have developed a tool to help patients talk to their physicians and hopefully result in an earlier diagnosis. Dive into our path to diagnosis infographic to unravel the mystery!
Resources
Looking for more information on this ultra-rare disease? Check out our resources section.
Patient Stories
Hear firsthand accounts of resilience and hope from individuals with Degos disease.
References
Sattler, S.S., Magro, C.M., Shapiro, L. et al. Gastrointestinal Kohlmeier–Degos disease: a narrative review. Orphanet J Rare Dis 17, 172 (2022). https://doi.org/10.1186/s13023-022-02322-9
Theodoridis, A., Makrantonaki, E., & Zouboulis, C. C. (2013). Malignant atrophic papulosis (Köhlmeier-Degos disease) - a review. Orphanet journal of rare diseases, 8, 10. https://doi.org/10.1186/1750-1172-8-10