Table 1. Diagnostic criteria for vascular EDS (Adapted from Beighton et al. [6])

Major diagnostic criteria

·      Thin, translucent skin

·      Arterial, intestinal or uterine fragility or rupture

·      Extensive bruising

·      Characteristic facial appearance (thin lips and philtrum, small chin, thin nose, large eyes)

Minor diagnostic criteria

·      Acrogeria

·      Hypermobility of small joints

·      Tendon/muscle rupture

·      Early onset varicose veins

·      Arteriovenous carotid-cavernous sinus fistula

·      Pneumo(hemo)thorax

·      Talipes equinovarus (clubfoot)

·      Gingival recession

·      Positive family history, sudden death in a close relative

The combination of any two of the major diagnostic criteria should have a high specificity for VEDS; further testing is strongly recommended to confirm the diagnosis. The presence of one or more minor criteria supports the diagnosis of VEDS but is not sufficient to establish it.