Ehlers-Danlos Syndrome, Type IV

**Definition:** A subtype of Ehlers-Danlos syndrome (EDS) characterized by vascular pathologies, e.g., AORTIC DISSECTION in addition to common EDS findings, e.g., hyperextensible skin and joints, skin fragility and reduced wound healing capability. It is associated with mutations in collagen type III alpha 1 chain gene (COLLAGEN TYPE III).

**Tree numbers:** - C14.907.055.448.250.256 - C14.907.454.240.500 - C15.378.463.515.240.500 - C16.131.831.428.500 - C16.320.850.260.500 - C17.300.200.310.500 - C17.800.804.428.500 - C17.800.827.260.500

**Synonyms:** - Ehlers Danlos Syndrome, Sack-Barabas Type - Type IV Ehlers-Danlos Syndrome - Vascular Type Ehlers-Danlos Syndrome - Ehlers-Danlos Syndrome, Vascular Type - Ehlers-Danlos Syndrome, Arterial Type - Ehlers-Danlos Syndrome, Ecchymotic Type - Ehlers-Danlos Syndrome, Sack-Barabas Type - EDS IV - Ehlers-Danlos Syndrome, Type IV, Autosomal Dominant - Ehlers Danlos Syndrome Type 4, Autosomal Dominant - Ehlers Danlos Syndrome, Arterial Type - Ehlers Danlos Syndrome, Ecchymotic Type - Vascular Ehlers-Danlos Syndrome