Overview
Malignant atrophic papulosis, also known as Degos disease or Köhlmeier-Degos disease, is a very rare and serious blood vessel disorder. It mainly affects the small blood vessels (arteries) in the skin, gut, and brain, causing them to become blocked. When blood flow is cut off, the tissues supplied by those vessels begin to die. The disease gets its name from the distinctive skin spots it causes — small, painless bumps that develop a white, sunken center with a reddish border, looking almost like tiny craters. These spots are often the first visible sign that something is wrong. The disease can affect just the skin (a milder, limited form) or spread to internal organs, which is the more dangerous form. When the gut or brain is involved, the consequences can be life-threatening. Blockages in the intestines can cause them to rupture, and blockages in the brain can lead to strokes. There is currently no cure for Degos disease, and treatment focuses on managing symptoms and trying to prevent new blood vessel blockages. Some medications that thin the blood or reduce inflammation have been tried, and a drug called eculizumab has shown promise in some patients, though it is not yet formally approved specifically for this condition. Because the disease is so rare, treatment decisions are often made case by case with specialist guidance.
Key symptoms:
Small skin bumps with a white, sunken center and red or pink border (called papules)Stomach pain or crampingNausea and vomitingDiarrhea or bloody stoolsHeadachesStroke-like symptoms such as sudden weakness, numbness, or difficulty speakingVision changes or double visionSeizuresConfusion or memory problemsFatigue and general feeling of being unwellWeight lossChest pain (if the lining around the heart or lungs is affected)
Clinical phenotype terms (34)— hover any for plain English
Variable
Can be inherited in different ways depending on the underlying gene
Adult
Begins in adulthood (age 18 or older)
FDA & Trial Timeline
10 eventsRush University Medical Center — NA
SWOG Cancer Research Network — PHASE2
Mackay Memorial Hospital — NA
The First People's Hospital of Lianyungang
National Heart, Lung, and Blood Institute (NHLBI)
Cairo University — PHASE3
St. Justine's Hospital
National Cancer Institute, Naples — NA
Vastra Gotaland Region
Data sourced from FDA regulatory filings and ClinicalTrials.gov. Updated periodically.
Treatments
No FDA-approved treatments are currently listed for Malignant atrophic papulosis.
View clinical trials →Clinical Trials
View all trials with filters →No actively recruiting trials found for Malignant atrophic papulosis at this time.
New trials open frequently. Follow this disease to get notified.
Rare Disease Specialist
Treatment Centers
8 centersBaylor College of Medicine Rare Disease Center ↗
Baylor College of Medicine
📍 Houston, TX
🏥 NORDStanford Medicine Rare Disease Center ↗
Stanford Medicine
📍 Stanford, CA
🔬 UDNNIH Clinical Center Undiagnosed Diseases Program ↗
National Institutes of Health
📍 Bethesda, MD
🔬 UDNUCLA UDN Clinical Site ↗
UCLA Health
📍 Los Angeles, CA
🔬 UDNBaylor College of Medicine UDN Clinical Site ↗
Baylor College of Medicine
📍 Houston, TX
🔬 UDNHarvard/MGH UDN Clinical Site ↗
Massachusetts General Hospital
📍 Boston, MA
🏥 NORDMayo Clinic Center for Individualized Medicine ↗
Mayo Clinic
📍 Rochester, MN
👤 Mayo Clinic Center for Individualized Medicine
🏥 NORDUCLA Rare Disease Day Program ↗
UCLA Health
📍 Los Angeles, CA
Travel Grants
No travel grants are currently matched to Malignant atrophic papulosis.
Community
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Start the conversation →Latest news about Malignant atrophic papulosis
Disease timeline:
New recruiting trial: Effect of Adding a Low-Dose Epinephrine Bolus Prior to Infusion on Maternal Hemodynamic Stability During Cesarean Section
A new clinical trial is recruiting patients for Malignant atrophic papulosis
New recruiting trial: Single Nuclei RNA-seq to Map Adipose Cellular Populations and Senescent Cells in Older Subjects
A new clinical trial is recruiting patients for Malignant atrophic papulosis
New recruiting trial: Delta Variation to Guide Hemodynamic Optimization
A new clinical trial is recruiting patients for Malignant atrophic papulosis
New recruiting trial: Evaluation of a CAM System for Colorectal Polyp Size Measurement
A new clinical trial is recruiting patients for Malignant atrophic papulosis
New recruiting trial: MAP( Mean Arterial Pressure) and Mortality Relation in Patients With Heart Failure
A new clinical trial is recruiting patients for Malignant atrophic papulosis
New recruiting trial: Impact of Intraoperative Hemodynamic Instability on Outcomes in Cardiac Surgery
A new clinical trial is recruiting patients for Malignant atrophic papulosis
New recruiting trial: Impact Assessment of the Jockey Club REACH & Map Program for Hard-to-reach Older Adults
A new clinical trial is recruiting patients for Malignant atrophic papulosis
New recruiting trial: Comparative Intra-procedural Evaluation of Farapulse and FARAWAVE Nav Catheters
A new clinical trial is recruiting patients for Malignant atrophic papulosis
New recruiting trial: Targeted Treatment for Advanced Non-Small Cell Lung Cancer That Has Increased Copies of the MET Gene (An Expanded Lung-MAP Treatment Trial)
A new clinical trial is recruiting patients for Malignant atrophic papulosis
New recruiting trial: Adding the Immunotherapy Drug Cemiplimab to Usual Treatment for People With Advanced Non-Small Cell Lung Cancer Who Had Previous Treatment With Platinum Chemotherapy and Immunotherapy (An Expanded Lung-MAP Treatment Trial)
A new clinical trial is recruiting patients for Malignant atrophic papulosis
Caregiver Resources
NORD Caregiver Resources
Support, advocacy, and financial assistance for caregivers of rare disease patients.
Mental Health Support
Rare disease caregiving can be isolating. Connect with counseling and peer support.
Family & Caregiver Grants
Financial assistance programs specifically for caregivers of rare disease patients.
Social Security Disability
Learn how rare disease patients may qualify for SSDI/SSI benefits.
Questions for your doctor
Bring these to your next appointment
- Q1.Is my disease limited to the skin, or are my internal organs involved — and how will you monitor for internal involvement?,Should I be tested for the SAMHD1 gene mutation, and what would a positive result mean for me and my family?,What medications do you recommend for me, and what are the risks and benefits of blood thinners or eculizumab in my case?,What symptoms should prompt me to go to the emergency room immediately?,How often do I need follow-up appointments and what monitoring tests will I need?,Are there any clinical trials I might be eligible for?,Can you connect me with other specialists or a center that has experience treating Degos disease?
Common questions about Malignant atrophic papulosis
What is Malignant atrophic papulosis?
Malignant atrophic papulosis, also known as Degos disease or Köhlmeier-Degos disease, is a very rare and serious blood vessel disorder. It mainly affects the small blood vessels (arteries) in the skin, gut, and brain, causing them to become blocked. When blood flow is cut off, the tissues supplied by those vessels begin to die. The disease gets its name from the distinctive skin spots it causes — small, painless bumps that develop a white, sunken center with a reddish border, looking almost like tiny craters. These spots are often the first visible sign that something is wrong. The disease ca
At what age does Malignant atrophic papulosis typically begin?
Typical onset of Malignant atrophic papulosis is adult. Age of onset can vary across affected individuals.
Which specialists treat Malignant atrophic papulosis?
11 specialists and care centers treating Malignant atrophic papulosis are listed on UniteRare, sourced from ClinicalTrials.gov principal investigators, published research, and the NPPES NPI registry.