Vascular Type: Manifestations
The skin is usually thin and traslucent with veins being seen through the skin. This is most apparent over the chest and abdomen. There are certain facial characteristics
present in some affected individuals. Also evident is a decrease in
subcutaneous tissue, particularly in the face and extremities. Minor
trauma can lead to extensive bruising.
Arterial/intestinal/uterine fragility or rupture
commonly arises in this type of EDS. Spontaneous arterial rupture has a
peak incidence in the third or fourth decade of life, but may occur
earlier. Midsize arteries are commonly involved. Arterial rupture is
the most common cause of sudden death. Acute diffuse or localized
abdominal or flank pain is a common presentation of arterial or
intestinal rupture. Life expectancy is shortened with a majority of
individuals living only into their forties.
Pregnancies may be complicated by intra-partum uterine rupture and pre- and postpartum arterial bleeding.
Joint hypermobility is usually limited to the digits.
Tendon and muscle rupture can occur. Talipes equinovarus (clubfoot) is
frequently seen at birth. Other manifestations that may be found in the
Vascular Type include:
- acrogeria (premature aging of the skin of the hands and feet)
- early onset varicose veins
- arteriovenousfistula (an opening between an artery and vein)
- pneumothorax (collapse of a lung) / pneumohemothorax (collapse of a lung with a collection of air or gas and blood)
- complications during and after surgery (i.e. wound dehiscence)