VASCULAR TYPE (Formerly EDS Type IV)
This type is generally regarded as the most serious form of EDS due to the possibility of arterial or organ rupture. The skin is usually thin and translucent 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. These manifestations include large eyes, thin nose, lobeless ears, short stature and thin scalp hair. 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 arise in this type of EDS. Spontaneous arterial rupture has a peak incidence in the 3rd or 4th 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 40's. 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. Clubfoot is frequently see at birth. Other manifestations that may be found in the Vascular Type include: premature aging of the skin of the hands and feet; early onset varicose veins; an opening between an artery and vein; carotid cavernous fistula; collapse of a lung/ collapse of alung with a collection of air or gas and blood; gingival recession and complications during and after surgery (i.e. wound dehiscence).

Types of EDS Main Page