Ehlers Danlos Syndrome

Ehlers-Danlos syndrome (EDS) is a group of hereditary disorders that affects mainly the skin and joints, but other organs as well. EDS results in weakness and/or excessive flexibility of the connective tissues of the body. People with EDS are born without the ability to make certain components of the normal connective tissue of the body.

Causes of Ehlers-Danlos syndrome

Collagen is one of the main building blocks of the body. It is a protein that is widely found in all organs of the body. Several types of collagen exist, each with differing properties. Collagen can provide strength and firm support, be elastic to allow movement, or it can be used to bind things together.

A genetic defect causes reduced amounts of collagen, disorganisation of collagen that is usually organised into bundles, and alterations in the size and shape of collagen. The type of EDS a patient has depends on how collagen metabolism has been affected. For example vascular EDS is caused by decreased or absent synthesis of type III collagen.

Signs and symptoms of Ehlers-Danlos syndrome

The symptoms and findings associated with Ehlers-Danlos syndrome (EDS) may vary greatly in range and severity from case to case, depending upon the specific form of the disorder present and other factors.

Treatment for Ehlers-Danlos syndrome

There is no specific cure for Ehlers-Danlos syndrome, so individual problems and symptoms must be evaluated and cared for appropriately. Physiotherapy and occupational therapy advice can help. Psychological support is also important, particularly when skin damage results in visible scarring and disfigurement.