Ehlers-Danlos Syndrome (EDS)
Ehlers-Danlos Syndrome is connective tissue disorder involving collagen synthesis and structure.
There are multiple types of EDS, based on
- Severity of skin and joint involvement
- Mode of inheritance
- Type of collagen affected
Clinical manifestation
- Joint hypermobility
- Skin hyper extensibility
- Vascular fragility
- Organ rupture
Types (below are most common types)
- Classical type – it involves collagen type V defect, skin hyper extensibility and joint hypermobility
- Hypermobile type – it involves hypermobile joints and it is most common type
- Vascular type - it has type III collagen defect and blood vessels are most commonly involved. Easy bruising, berry aneurysm and sub arachnoid hemorrhage are common manifestations.
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