Ehlers-Danlos Syndrome

What is EDS?

Ehlers-Danlos syndrome (EDS) is a group of inherited disorders of the body’s connective tissue.  These disorders affect the supportive connective tissue of the skin, bones, blood vessels as well as many organs and body tissues. There are different types of EDS which are defined by their symptoms. It is estimated that 1 in 2,500 to 5,000 people have EDS worldwide. EDS affects all races and genders equally.

What are the symptoms of EDS?

Most commonly the symptoms of EDS affect the skin and joints. Symptoms differ due to the type of EDS and include:

• Skin: EDS causes soft velvet like skin which is very fragile and highly elastic. EDS results in easy bruising, fragile skin which tears easily, poor healing and characteristic widening scars known as “cigarette paper” scars.
• Joints: EDS leads to loose, unstable, highly flexible joints that dislocate or sprain easily. Other effects on the joints include tearing of muscles and tendons as well as deformities of the fingers, neck and spine.
• Cardiovascular: Some forms of EDS can affect the vessels and organs of the cardiovascular system. These symptoms include sudden vessel rupture, internal bleeding, stroke, heart valve disease, heart rhythm disorders and varicose veins. These symptoms can be fatal.
• Other symptoms: EDS can also cause malfunction in the digestive tract, vessels of the brain and respiratory system. Women with EDS may experience complications with pregnancy.

What causes EDS?

EDS is caused by inherited defects in collagen and its related proteins. These proteins are necessary for proper structure and function of the body’s tissues.

What treatment options are available for EDS?

There is no cure for EDS.  Treatment is focused on preventing damage and improving quality of life. These include:

• Braces for unstable joints
• Physical therapy
• Medications for pain and heart problems
• Emotional support and therapy to help cope with lifestyle limitations and chronic symptoms
• Surgery to repair damaged joints
• Close monitoring of cardiovascular system

Where can I find more information about EDS?

• NIH
• NORD
• EDS