Ehlers Danlos Syndrome – Inherited Condition

Ehlers Danlos syndrome – EDS is related to a group of inherited conditions which affect the collagen proteins in the body. It features are stretchy skin, fragile body tissue and loose joints. It is a disorder affecting the connective tissues primarily the skin and blood vessel walls, besides the joints.

The connective tissues are a complex combination of proteins and other substances which provides strength and elasticity to the underlying structure of the body. Collagen is a building block which strengthens and supports the various body tissues and is found in tendons, ligaments, skin, cartilage, bone, blood vessels, in the gut and the spine.

 The cause of EDS is due to the alteration of certain genes, which make collagen weaker and sometimes the quantity of collagen is reduced in the body. These faulty genes can be passed from the parents to their children.

Flexible Joints and Stretchy Skin

People suffering from Ehlers Danlos syndrome tend to have flexible joints and stretchy fragile skin which can become a problem if they have a wound that may need stitches since the skin is not strong enough to hold them. There are various forms of EDS such as Classical wherein the skin is stretchy, soft, elastic and fragile.

The joints tend to be loose and flexible. Hypermobility is another form wherein the joints are noticeably loose and besides being flexible are sometimes painful particularly after exercise though unlike other types of EDS, the skin is normal except that it gets bruised easily. The loose joints tend to bend more than usual and can cause floppy joints in infant while some children who are affected take longer time to sit, stand and even walk.

Sometimes the joints can also be very unstable and may tend to dislocate easily. Joint unstability may at times lead to osteoarthritis though this is uncommon and may happen mostly with adults. Vascular is one of the most severe kinds of this disorder that may cause the walls of the blood vessels, intestines or uterus to rupture.

Kyphoscoliotic disorder is where the spine is severely curved in childhood. Dermatospraxis is another type wherein the skin is loose and wrinkled with a tendency to sag and fold though this is a rare case probably affecting few people. Periodontal, this form resembles classical EDS with the combination of fragile gums.

Prone to Bruises

In most of the EDS cases, the skin is stretchy than normal and easily pulls away from the body springing back once released which could be tested best at the neck, elbow or knee. Bruising of the skin is another form wherein the small surface blood vessels may be fragile and break easily.

In the case of classical EDS, the skin can be extremely fragile and split easily especially in the areas over the forehead, knees, shins and the elbow while the scars can be papery and wide. In the case of vascular EDS, the skin seems transparent especially over the chest with the veins clearly visible beneath. People with this disorder tend to tire easily and should conserve their energy and pace their activities.