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Table 2 Vascular Ehlers-Danlos syndrome: Villefranche diagnostic criteria (adapted from [3]

From: Ehlers-Danlos syndrome type IV

Major diagnosis criteria

Arterial, digestive or uterine fragility or rupture

 

Thin, translucent skin

 

Extensive bruising

 

Characteristic facial appearance

Minor diagnosis criteria

Positive family history, sudden death in a close relative

 

Acrogeria

 

Hypermobility of small joints

 

Tendon and muscle rupture

 

Talipes equinovarus (clubfoot)

 

Early onset varicose veins

 

Spontaneous pneumothorax or haemothorax