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Author: Hon A/Prof Amanda Oakley, Dermatologist, Hamilton, New Zealand, 2003.
Ehlers-Danlos syndrome (EDS) is the name given to a group of inherited disorders that involve a genetic defect in collagen and connective tissue synthesis and structure. This results in fragile and hyperelastic skin, unstable and hyperextensible (hypermobile) joints, and fragile tissue and blood vessels.
EDS may occur in males and females of all races and usually first appears in young adults.
There are six subtypes of EDS, classified according to the clinical features. Each is a distinct disorder that ‘runs true’ in a family. This means that members of a single family affected by EDS will share the same features of it. Some cases do not fit neatly into a known type of EDS and in many situations a patient may show features of more than one type.
Subtypes of EDS [view table in a new window]
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 differ in type and severity between the different types of EDS.
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