Ehlers-Danlos syndrome

Ehlers-Danlos syndrome (EDS) is a group of heritable disorders associated with a defect in collagen formation leading to skin fragility and hyperelasticity of skin, hypermobility of joints and poor wound healing with scarring. Surgical patients with EDS have suboptimal wound healing and multiple complications following surgery secondary to defects in collagen metabolism.

Ehlers-Danlos syndrome has been subdivided into separate types according to the predominant areas affected and the degree of abnormality. The malignant form of EDS (type IV) owes its bad reputation to a proneness to spontaneous rupture of bowel or large arteries. Paradoxically, other manifestations are less dramatic than in some other forms of EDS. For example, joint hypermobility may be confined largely to the fingers and whereas the skin is strikingly thin and translucent, it is only mildly hyperextensible. Bruisability, however, is very striking.

Ehlers-Danlos syndrome (EDS) has an incidence of approximately 1 in 5000 births.