Tuesday, May 1, 2018

Ehlers-Danlos Syndrome (EDS)

Ehlers-Danlos syndrome comprises a group of clinically and genetically heterogeneous, inherited connective tissue diseases.

Connective tissue provides support to many parts of body like skin, muscles, and ligaments. People with EDS may have fragile skin or unstable joints. Different defects in the synthesis of collagen lead to an increased elasticity within different types of connective tissue (skin, joints, muscles, tendons, blood vessels and visceral organs).

The Ehlers Danlos syndromes (EDS) have fascinated people throughout the ages. The first report of this disorder dates back to Hippocrates (fourth century BC).

For many centuries, affected individuals earned their livings as The Elastic Skin Man, The India Rubber Man and The Human Pretzel, amazing their audiences in fairgrounds and circus side shows by exhibiting contortionist tricks and a remarkable ability to stretch their skin.

People with this condition have loose joints and frequently have long-term joint pain. Characteristics range from mild, such as loose joints, to severe, such as functional bowel disorders and incisional hernias. The joints and skin are most commonly affected. Joints may have a wide range of movement (hypermobility), be unstable, and tend to move out of place (dislocate) frequently. The shoulder, knee, and jaw are some of the joints that dislocate most often.

About 1 in every 20,000 babies is born with EDS. It can be inherited in an autosomal dominant, autosomal recessive, or X-linked recessive manner. There are at least 9 different types of EDS.
• The most common form of EDS is EDS Hypermobility Type (formerly known as Type III)
• Followed by EDS Classical Type (formerly known as Type I and Type II) and
• EDS Vascular Type (formerly known as Type IV)
Ehlers-Danlos Syndrome (EDS)

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