Vascular Ehlers-Danlos Syndrome

Vascular-type Ehlers-Danlos syndrome is a severe subtype of a genetic connective tissue disorder. People with this condition have very fragile tissues and are at high risk for severe bleeding and internal injuries. While it isn’t curable, this condition is often manageable, and the complications are often treatable.


What is vascular Ehlers-Danlos syndrome?

Ehlers-Danlos syndrome (EDS) refers to a group of genetic disorders that mainly affect your body's connective tissues, such as ligaments, tendons, and cartilage. There are 13 types of this condition, with vascular EDS being type IV. While extremely rare, it’s also especially severe. People with this subtype of EDS have very fragile arteries and internal organs.


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Who does it affect?

Vascular Ehlers-Danlos syndrome is genetic, meaning people often inherit it from one or both parents. When it happens to people with no family history of this condition, it’s because this mutation happened spontaneously.

How common is this condition?

Ehlers-Danlos syndrome overall is a rare condition, only affecting 1 in 5,000 people. The vascular type of this condition is even rarer, with only 1 case per 200,000 to 250,000 people.


How does this condition affect my body?

Vascular Ehlers-Danlos syndrome affects how strong and durable certain tissues are in a person’s body, especially the arteries and organs. That means people with this condition bruise easily and have a high risk of dangerous bleeding from internal injuries.

Symptoms and Causes

What are the symptoms?

The most common symptoms of vascular Ehlers-Danlos syndrome include:

  • Skin differences. People with this condition have thinner, translucent and delicate skin (making veins very visible). Certain areas of skin will also age faster than others, especially skin on your hands and feet.
  • Distinctive facial features. People with this condition usually have unusually thin lips and nose, a small chin and eyes that are large and far apart. They may have very small or no eyebrows. They also are more likely to have very small earlobes or no earlobes at all, and their ears will stick out unusually far.
  • Circulatory problems. Vascular EDS causes people to bruise more easily. It’s also more common for people with this condition to develop varicose veins at earlier ages. High blood pressure (hypertension) and problems with heart valves (such as mitral valve prolapse) happen very frequently. People with vascular EDS also have a high risk of arterial injuries, including arterial dissections (where inside layers of the artery separate). These can lead to dangerous aneurysms (weakened blood vessel walls that bulge outward) or ruptures.
  • Fragile internal organs. Collapsed lung (pneumothorax) and ruptured internal organs, causing severe bleeding, are possible dangerous complications.
  • Skeletal changes. People with this condition often have pectus excavatum (sunken or funnel chest). They are also usually shorter in height than average.


What causes the condition?

This condition is genetic, meaning it happens because of a genetic mutation that affects certain parts of your DNA. Your body uses DNA like an instruction manual, following the instructions exactly to determine how your cells and organs do their job. Mutations are like typos in the instruction manual, but your body still follows the faulty instructions.

Vascular Ehlers-Danlos syndrome happens because of a mutation in a gene that affects a protein called collagen III. Your body normally uses collagen III to make certain tissues and structures stronger and more durable. That mutation means your body either doesn't make enough collagen III or makes flawed collagen III that doesn't strengthen various tissues as it should.

Because this condition is genetic, people who have it can pass it to their children. A person with this condition has a 50% chance of passing it to their child with each pregnancy (if both parents have this condition, the chance is 100%).

Is it contagious?

No, this condition can’t pass from person to person.

Diagnosis and Tests

How is it diagnosed?

A doctor may begin to suspect vascular Ehlers-Danlos syndrome based on your medical history, physical examination, symptoms or a family history of this condition. Once they suspect this condition, they can use genetic testing to confirm or rule out that suspicion. Genetic testing is the only way to be certain of this diagnosis because there are only two genetic mutations known (for now) that cause this condition.

What tests will be done to diagnose this condition?

Because this condition is very rare, healthcare providers may perform several other tests, such as an echocardiogram or CT scan, before running genetic tests. The early tests may focus on ruling out blood disorders that increase the risk of severe bleeding or easy bruising. However, genetic testing is the only way to be certain that you have vascular Ehlers-Danlos syndrome.

Management and Treatment

How is this condition treated, and is it curable?

Vascular EDS is not curable. This condition is genetic, meaning that it’s a lifelong condition. Because it isn’t curable, healthcare providers will focus on treating and minimizing the impact of any symptoms.

What medications/treatments are used?

People with vascular Ehlers-Danlos syndrome have a high risk of developing dangerous internal bleeding because of aneurysms and torn arteries. Because of this, you may need regular medical screenings to look for aneurysms if you have this condition. In some cases, you may need surgery to repair internal injuries or aneurysms. Pregnancy can also lead to a high risk of severe or life-threatening complications from this condition, such as rupture of the uterus or severe bleeding.

A healthcare provider is the best person to explain the potential medications, treatments and procedures that you may need. They can best explain what's happening in your situation, taking into account your circumstances and the details of the care you need or receive.

Complications/side effects of the treatment

Because this condition causes you to have very fragile tissues, you will also have a high risk of bleeding. You may also have trouble recovering after surgery for this same reason. A healthcare provider is the best person to explain the potential complications and risks that you may face.

How to take care of myself/manage symptoms?

Vascular EDS is a complicated condition that needs close medical monitoring and regular visits to one or more healthcare providers. Because of how complex this condition is, it’s not something you can take care or manage without help from a healthcare provider.

How soon after treatment will I feel better?

A healthcare provider is the best person to explain what you can expect from this condition, especially if you need treatment to repair an injury or other problem.


How can I reduce my risk of developing this condition, and is it preventable?

Because vascular EDS is a genetic condition, there’s no way to prevent it or reduce your risk of developing it. People with this condition should consider talking to a healthcare provider if they become pregnant or want to have children, so they can know what to expect if their child inherits this condition.

Outlook / Prognosis

What can I expect if I have this condition, and what is the outlook?

People with vascular EDS have a high risk of complications and problems related to bleeding or fragile internal tissues and organs. Most people with this condition will have at least one instance of severe complications or related conditions by age 20. The risk of having life-threatening complications is 80% by age 40. Half of the people with this condition will live to at least age 48.

How long does this condition last?

Vascular EDS is a condition that you have when you’re born and lasts for your entire life.

Living With

How do I take care of myself?

People with vascular EDS should see a healthcare provider regularly who can monitor their condition and look for any evidence of new problems. They should also avoid dangerous sports or activities, heavy lifting or other activities that bring a higher risk of injury. They may also want to avoid elective surgery because of the high risk of dangerous bleeding and internal injuries.

When should I see my healthcare provider/When should I seek care?

You should see your healthcare provider regularly as recommended. They can also tell you any signs or symptoms that mean you need to call or see them.

When should I go to ER?

If you have this condition, you should seek emergency medical attention if you have any unexplained severe pain, especially in your chest or abdomen. You should also get emergency medical attention if you have an external wound or injury that is or was bleeding heavily.

A note from Cleveland Clinic

Vascular Ehlers-Danlos syndrome is a complicated genetic condition that needs close medical monitoring and care. While it's a condition with high risks, advances in medical care and understanding of this condition mean that people with this disorder live longer and have better outcomes than in years past.

Medically Reviewed

Last reviewed on 04/10/2022.

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