Degos Disease: Causes, Symptoms, and Treatment
Medically reviewed by Sumiran Racherla, MD, Internal Medicine. Last reviewed: October 23, 2026.
What kind of doctor treats Degos Disease?
A dermatologist, a skin doctor, usually leads care for Degos disease. A gastroenterologist (a gut doctor) or a neurologist (a nerve doctor) may join the team. A direct primary care or concierge doctor can be a first stop for an evaluation and can refer you to a specialist when needed.
What is Degos disease?
Degos disease is an extremely rare disorder. It is also called malignant atrophic papulosis. Small arteries become blocked, which limits blood flow to the affected areas.
Most people have only skin symptoms, called benign cutaneous Degos disease. This form has an excellent prognosis, or outlook. About two-thirds of people only develop skin lesions that are not life-threatening.
Other people develop systemic Degos disease, which affects organs inside the body. It can affect the skin, the digestive tract, and the nervous system.
Symptoms
In most people, the first sign is skin lesions. These are small, raised bumps or spots, often on the trunk, upper arms, and upper legs.
Over weeks to months, the bumps turn into flat or sunken white spots with a red rim. The palms, soles, and face are usually not affected.
Systemic disease can cause serious and possibly life-threatening problems. The digestive tract is the most common inside organ affected. Symptoms there can include:
- Belly pain and cramping.
- Nausea, vomiting, diarrhea, bloating, or constipation.
- Weight loss and tiredness from poor absorption of nutrients.
The bowel lesions can tear, which lets bowel contents leak into the belly. This causes peritonitis, swelling of the lining of the belly. It is life-threatening and the most common cause of death in people with Degos disease.
Some people have nervous system involvement. Symptoms can include headaches, dizziness, seizures, weakness on one side of the body, and strokes. Nervous system involvement nearly always means severe disease.
Causes and who gets it
Degos disease is caused by growth of the cells that line small arteries. This narrows or blocks the arteries. The reason this happens is still unknown.
Ideas include a virus, inflamed blood vessels, or abnormal blood clotting. Another idea is that the immune system attacks healthy tissue by mistake. Some cases are believed to be inherited. Other cases have no known genetic cause.
It can affect any age. Symptoms most often start between ages 20 and 50. Men have been affected more often than women.
How doctors diagnose it
The first step is usually a look at the typical skin lesions. A skin biopsy confirms the diagnosis. This means a small skin sample is studied under a microscope. It shows dead tissue and blocked small arteries.
No specific lab test can help diagnose Degos disease. Most lab tests are normal. Some people with systemic disease have anemia (low red blood cells).
Baseline tests for systemic disease are also advised. They include a chest x-ray, a heart ultrasound (echocardiogram), an EKG (a heart rhythm test), an eye exam, and a brain MRI. A laparoscopy uses a small camera placed through the belly wall. It is the most effective way to check the gut.
Treatment
The diagnosis can cause a great deal of worry. Quick testing for systemic disease is the best way to address it. If systemic disease is found, a visit with an expert in Degos disease is advised.
Some medicines block C5, a part of the immune system. These C5 inhibitors have been lifesaving for several people with gut Degos disease.
NORD notes that these medicines have not been approved by the FDA specifically for Degos disease. Many articles suggest antiplatelet therapy, which keeps blood cells from clumping, even for skin-only disease. The evidence of benefit is sparse.
People with skin-only disease should learn the signs that could point to systemic disease. Treatment is more likely to prevent lasting organ damage or death when the disease is found early.
When to see a doctor
Anyone with these typical skin lesions should be checked for Degos disease. Get checked for new belly symptoms that keep happening, because doctors consider a repeat laparoscopy then.
Call 911 or go to the emergency room for severe belly pain, vomiting blood, a seizure, sudden weakness, or trouble speaking.
How a direct primary care or concierge doctor fits in
A direct primary care doctor charges a flat monthly fee instead of billing insurance, while a concierge doctor usually charges more and often works alongside insurance. A direct primary care doctor can look at skin changes, refer you to a dermatologist, and track new symptoms over time. They can also help coordinate visits with specialists.
Common questions
Is Degos disease life threatening?
It can be, but about two-thirds of people only develop skin lesions that are not life-threatening. Systemic disease can be life-threatening. Recent advances in drug therapy have greatly improved the chance of recovery.
What is the difference between skin-only and systemic Degos disease?
Skin-only disease is limited to the skin and has an excellent prognosis. Systemic disease also affects organs such as the digestive tract and the nervous system.
Is Degos disease inherited?
Some cases are believed to be inherited, but other cases have no known genetic cause.
Is there a treatment for Degos disease?
Yes, doctors have treatments that can help. NORD notes that the medicines used have not been approved by the FDA specifically for Degos disease. Complement-blocking medicines have been lifesaving for several people with digestive tract disease.
Sources
- NORD (National Organization for Rare Disorders) - Degos Disease
- PubMed (National Library of Medicine) - Malignant atrophic papulosis (Degos disease)
Medical Disclaimer
This content is for general educational and informational purposes only and is not medical advice. It is not a substitute for professional medical advice, diagnosis, or treatment, and reading it does not create a doctor-patient relationship. Always seek the advice of your physician or another qualified health provider with any questions about a medical condition, and never disregard or delay seeking professional advice because of something you read here. If you think you may have a medical emergency, call 911 (or your local emergency number) immediately.
