what is EDS

What is Ehlers-Danlos syndrome?

Ehlers-Danlos syndrome is known as a group of inherited disorders that affect the body’s connective tissues, especially the joints, skin, and blood vessels. Connective tissue is a composite mixture of proteins and other substances that gives the body’s underlying structures strength and elasticity.

Ehlers-Danlos syndrome is characterized by excessively flexible joints and stretchy, porous skin. If you have a wound that needs stitches, this may be a challenge because the skin isn’t always tight enough to hold them.

Vascular Ehlers-Danlos syndrome is a more severe type of condition that can cause the walls of your blood vessels, intestines, or uterus to burst. You should consult a genetic counselor before starting a family because vascular Ehlers-Danlos syndrome can cause severe complications during pregnancy.

Signs and Symptoms

There are several signs and symptoms of Ehlers-Danlos syndrome, but the following are the most common signs and symptoms:

Overly flexible joints

Your joints will travel well outside their natural range of motion because the connective tissue that keeps them together become loose. Dislocations of joints and joint pain are very common.

Stretchy skin

Your skin will extend much further than average due to weakened connective tissue. You may be able to take a sliver of skin away from your flesh, but once you let go, it will snap back into place. It’s also possible that your skin would feel exceptionally smooth and velvety.

Fragile skin

Skin that has been damaged does not always heal well. Stitches used to close a wound, for example, sometimes tear out and leave a gaping scar. These scars can appear crinkly and small.

The severity of this syndrome symptoms varies from one person to another and is determined by the type of Ehlers-Danlos syndrome you have. Hypermobile Ehlers-Danlos syndrome is the most common form.

Vascular Ehlers-Danlos syndrome

A narrow nose, thin upper lip, small earlobes, and prominent eyes are common facial features in people with vascular Ehlers-Danlos syndrome. Their skin is also thin and transparent, and they bruise easily. The underlying blood vessels are evident through the skin of fair-skinned individuals.

The largest artery in your heart (the aorta), as well as arteries to other parts of the body, maybe weakened by vascular Ehlers-Danlos syndrome. Any of these larger blood vessels will burst and cause death. The vascular type may also weaken the uterine or large intestine walls, causing them to burst.

Causes

Ehlers-Danlos syndrome is caused by several genetic factors, some of which are inherited and passed on from parent to child. If you have the most common type of Ehlers-Danlos syndrome, i.e., hypermobile Ehlers-Danlos syndrome, you have a 50-55% chance of passing the gene on to each of your children.

Difficulties/Complications

The types of complications you have are determined by the signs and symptoms you have. Joint dislocations and early-onset arthritis, for example, maybe caused by excessively flexible joints. Scarring is more likely to appear on fragile skin.

The uterus and intestines are two organs that can burst. The risk of uterine rupture is increased during pregnancy.