Clinical spectrum of Ehlers-Danlos syndrome
Site | Cardinal features | Symptoms | Effects |
---|---|---|---|
Joints | Hypermobility | Arthralgia Dislocation | Instability Soft tissue trauma |
Skeleton | Osteoporosis | Scoliosis Fracture | Deformity |
Skin | Hyperextensibility + | Thinning Striae atrophicae Molluscoid pseudotumours | Violaceous papyraceous scars Bruising and haematoma Subcutaneous spheroids |
Vasculature | Mitral valve Intracranial (Ehlers-Danlos syndrome type IV) | Prolapse Aneurysm | SBE Subarachnoid haemorrhage |
Muscles | Hernia Intestinal/bladder diverticula | Rectal/uterine prolapse Neuromyopathy |
SBE, subacute bacterial endocarditis.