National Center for Advancing and Translational Sciences Genetic and Rare Diseases Information Center, a program of the National Center for Advancing and Translational Sciences

Malignant Atrophic Papulosis



Other Names:
Degos's malignant atrophic papulosis; Atrophic papulosis, malignant; Kohlmeier-Degos disease; Degos's malignant atrophic papulosis; Atrophic papulosis, malignant; Kohlmeier-Degos disease; Köhlmeier-Degos disease; Papulosis atrophican maligna; Degos disease; Köhlmeier-Degos-Delort-Tricort syndrome See More
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Degos disease is a rare blood vessel disorder. It is characterized by blockages of small to medium sized blood vessels.  This slows or stops the flow of blood through the affected vessels. Severity of symptoms depends on the extent and location of the affected blood vessels. Some individuals with Degos disease have isolated skin involvement and develop porcelain-white macules on their skin. Other individuals have more wide spread disease. Multiorgan disease can become life threatening.[1] The cause of this condition is currently unknown.
Last updated: 3/31/2011

Signs and symptoms of Degos disease can vary greatly from person to person.  Skin symptoms include porcelain-white macules that tend to develop on the trunk, arms, and legs. For some people, this is their only symptom. For others, Degos disease affects multiple body organs.  

Skin macules tend to be the earliest symptom in multisystem disease. Small bowel involvement is very common, and Degos disease can cause intestinal perforation (tear).[2] Intestinal perforation is a medical emergency which requires prompt treatment. Click here to learn more about the signs and symptoms of intestinal perforation.  Unfortunately intestinal disease tends to recur in these individuals.

Degos disease can also affect the nervous system, in particular the cerebral and peripheral nerves. This may result in a variety of symptoms, such as partial paralysis, aphasia (difficulty communicating), cranial neuropathies (which affect nerves that are connected with the brain and control sight, eye movement, hearing, and taste), sensory disturbances, and seizures.[2]
Last updated: 6/6/2011

This table lists symptoms that people with this disease may have. For most diseases, symptoms will vary from person to person. People with the same disease may not have all the symptoms listed. This information comes from a database called the Human Phenotype Ontology (HPO) . The HPO collects information on symptoms that have been described in medical resources. The HPO is updated regularly. Use the HPO ID to access more in-depth information about a symptom.

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Medical Terms Other Names
Learn More:
HPO ID
80%-99% of people have these symptoms
Dermal atrophy
Skin degeneration
0004334
Papule 0200034
Telangiectasia of the skin 0100585
30%-79% of people have these symptoms
Abdominal pain
Pain in stomach
Stomach pain
[ more ]
0002027
Fatigue
Tired
Tiredness
[ more ]
0012378
Gastrointestinal hemorrhage
Gastrointestinal bleeding
0002239
Gastrointestinal infarctions
Death of digestive organ tissue due to poor blood supply
0005244
Intestinal perforation 0031368
Muscle flaccidity 0010547
Nausea and vomiting 0002017
Weight loss 0001824
5%-29% of people have these symptoms
Abnormal myocardium morphology 0001637
Abnormal pericardium morphology 0001697
Abnormality of the lower urinary tract 0010936
Abnormality of the optic nerve
Optic nerve issue
0000587
Amaurosis fugax 0100576
Arterial thrombosis
Blood clot in artery
0004420
Arteritis
Inflammation of artery
0012089
Cataract
Clouding of the lens of the eye
Cloudy lens
[ more ]
0000518
Chest pain 0100749
Cranial nerve paralysis 0006824
Diplopia
Double vision
0000651
Intestinal fistula 0100819
Ischemic stroke 0002140
Migraine
Intermittent migraine headaches
Migraine headache
Migraine headaches
[ more ]
0002076
Myocardial infarction
Heart attack
0001658
Pain insensitivity 0007021
Peritonitis 0002586
Pleural effusion
Fluid around lungs
0002202
Ptosis
Drooping upper eyelid
0000508
Respiratory failure 0002878
Seizure 0001250
Vertigo
Dizzy spell
0002321
Percent of people who have these symptoms is not available through HPO
Abnormal conjunctiva morphology 0000502
Autosomal dominant inheritance 0000006
Constrictive pericarditis 0002563
Stroke 0001297
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Last updated: 7/1/2020

Currently the cause of Degos disease is not known. Similar skin macules have been described in people with systemic lupus erythematosus and in a patient without lupus who had anticardiolipin antibodies and lupus anticoagulant.[2]
Last updated: 3/31/2011

Currently, there is not a targeted therapy for Degos disease that has been proven effective. Treatment of Degos disease has been attempted with antithrombotic agents, such as aspirin and dipyridamole.[2] These treatments were reported to be effective in some patients.[2][3] Other treatments that have been tried, but have shown inconsistent results, include anticoagulants and fibrinolytic agents (drugs to help break-up and dissolve clots), ticlopidine, pentoxifylline, prostaglandin E1, and interferon alpha-2a. Treatment with intravenous immunoglobulin has also been tried, but produced conflicting results.[3] Infliximab was reported to be ineffective in one case.[3] Immunosuppressives such as corticosteroids may worsen Degos disease.[3]

For further information on your treatment options, we encourage you to discuss your questions and this information with your healthcare provider.
Last updated: 10/26/2011

Prognosis of individuals with Degos disease varies considerably depending on the extent of blood vessel involvement. Individuals with isolated skin macules have a good prognosis, while individuals with multisystem disease often face life threatening complications.  To learn more about your or your loved one’s prognosis we strongly recommend that you speak with a healthcare provider.
Last updated: 3/31/2011

If you need medical advice, you can look for doctors or other healthcare professionals who have experience with this disease. You may find these specialists through advocacy organizations, clinical trials, or articles published in medical journals. You may also want to contact a university or tertiary medical center in your area, because these centers tend to see more complex cases and have the latest technology and treatments.

If you can’t find a specialist in your local area, try contacting national or international specialists. They may be able to refer you to someone they know through conferences or research efforts. Some specialists may be willing to consult with you or your local doctors over the phone or by email if you can't travel to them for care.

You can find more tips in our guide, How to Find a Disease Specialist. We also encourage you to explore the rest of this page to find resources that can help you find specialists.

Healthcare Resources


Related diseases are conditions that have similar signs and symptoms. A health care provider may consider these conditions in the table below when making a diagnosis. Please note that the table may not include all the possible conditions related to this disease.

Conditions with similar signs and symptoms from Orphanet
The histology of early lesions resembles cutaneous lupus erythematosus (see this term). More developed lesions can imitate lichen sclerosus (see this term).
Visit the Orphanet disease page for more information.

Research helps us better understand diseases and can lead to advances in diagnosis and treatment. This section provides resources to help you learn about medical research and ways to get involved.

Clinical Research Resources

  • The Centers for Mendelian Genomics program is working to discover the causes of rare genetic disorders. For more information about applying to the research study, please visit their website.

Support and advocacy groups can help you connect with other patients and families, and they can provide valuable services. Many develop patient-centered information and are the driving force behind research for better treatments and possible cures. They can direct you to research, resources, and services. Many organizations also have experts who serve as medical advisors or provide lists of doctors/clinics. Visit the group’s website or contact them to learn about the services they offer. Inclusion on this list is not an endorsement by GARD.

Social Networking Websites

  • The Degos Disease Support Network Patient Forum is a online community for people with Degos disease and their families. You may need to register to post on the forum, but registration is free. Click on Degos Disease Support Network Patient Forum to learn more.

Living with a genetic or rare disease can impact the daily lives of patients and families. These resources can help families navigate various aspects of living with a rare disease.

Financial Resources


These resources provide more information about this condition or associated symptoms. The in-depth resources contain medical and scientific language that may be hard to understand. You may want to review these resources with a medical professional.

Where to Start

  • DermNet NZ is an online resource about skin diseases developed by the New Zealand Dermatological Society Incorporated. DermNet NZ provides information about this condition.
  • The National Organization for Rare Disorders (NORD) has a report for patients and families about this condition. NORD is a patient advocacy organization for individuals with rare diseases and the organizations that serve them.

In-Depth Information

  • Medscape Reference provides information on this topic. You may need to register to view the medical textbook, but registration is free.
  • MeSH® (Medical Subject Headings) is a terminology tool used by the National Library of Medicine. Click on the link to view information on this topic.
  • The Monarch Initiative brings together data about this condition from humans and other species to help physicians and biomedical researchers. Monarch’s tools are designed to make it easier to compare the signs and symptoms (phenotypes) of different diseases and discover common features. This initiative is a collaboration between several academic institutions across the world and is funded by the National Institutes of Health. Visit the website to explore the biology of this condition.
  • Online Mendelian Inheritance in Man (OMIM) is a catalog of human genes and genetic disorders. Each entry has a summary of related medical articles. It is meant for health care professionals and researchers. OMIM is maintained by Johns Hopkins University School of Medicine. 
    The Online Mendelian Inheritance in Man (OMIM)
    The Online Mendelian Inheritance in Man (OMIM)
  • Orphanet is a European reference portal for information on rare diseases and orphan drugs. Access to this database is free of charge.
  • PubMed is a searchable database of medical literature and lists journal articles that discuss Malignant Atrophic Papulosis. Click on the link to view a sample search on this topic.

Questions sent to GARD may be posted here if the information could be helpful to others. We remove all identifying information when posting a question to protect your privacy. If you do not want your question posted, please let us know.


  1. Malignant Atrophic Papulosis. MeSH. http://www.nlm.nih.gov/cgi/mesh/2011/MB_cgi?mode=&index=24677&view=expanded. Accessed 3/31/2011.
  2. Mark LA, Mirowski GW. Oral Disease and Oral-Cutaneous Manifestations of Gastrointestinal and Liver Disease. In: Feldman eds. Sleisenger and Fordtran's Gastrointestinal and Liver Disease, 9th ed. Philadelphia, PA: Saunders; 2010;
  3. Cheng TS. A man with generalized small white skin lesions and abdominal pain. Int J Dermatol. 2011 Jun;50(6):726-9; http://www.ncbi.nlm.nih.gov/pubmed/21595670. Accessed 10/26/2011.
  4. Wilson J . Benign cutaneous Degos disease in a 16-year-old girl. Pediatr Dermatol. 01-JAN-2007;