Ehlers-Danlos syndromes

Posted February 8, 2016

The Ehlers-Danlos syndromes (EDS) are a group of inherited conditions that fit into a larger group known as heritable disorders of connective tissue. Connective tissues are present in many areas of the body including the walls of blood vessels, organs, skin, bones and tendons. Collagen proteins are the main component of connective tissues and there are a number of different types.

The different types of EDS are separate conditions with different causes, but they can have some characteristics in common.1 Key features include: joint hypermobility, hyperextensible skin and tissue fragility. The diagnosis is currently based on the Villefranche classification written in 1997.2 Although there are currently no particularly good prevalence figures, the most frequent type is hypermobile EDS, followed by classical EDS and vascular EDS. The other types of EDS are exceptionally rare. Since 1997 there have been advances in the understanding of EDS and a number of new subtypes have been described.

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