/Ehlers-Danlos syndrome, vascular type

Ehlers-Danlos syndrome, vascular type

Rare Disease

MONDO:0017314

Also Known As

EDS IVEDS type 4Ehlers-Danlos syndrome type 4Ehlers-Danlos syndrome type IVEhlers-Danlos syndrome, type IVEhlers-Danlos syndrome, vascular typesack-Barabas syndromeEDS IV (formerly)EDS type 4 (formerly)EDS4 (formerly)Ehlers Danlos syndrome, arterial typeEhlers Danlos syndrome, ecchymotic typeEhlers Danlos syndrome, sack-Barabas typeEhlers-Danlos syndrome type 4 (formerly)Ehlers-Danlos syndrome type IV (formerly)vEDSvascular EDSvascular Ehlers-Danlos syndrome

Definition

Ehlers-Danlos syndrome type IV, also known as the vascular type of Ehlers-Danlos syndrome (EDS), is an inherited connective tissue disorder defined by characteristic facial features (acrogeria) in most patients, translucent skin with highly visible subcutaneous vessels on the trunk and lower back, easy bruising, and severe arterial, digestive and uterine complications, which are rarely, if at all, observed in the other forms of EDS.