Ehlers-Danlos syndrome
Pronunciation: AY-lers DAN-los
Also written as: EDS — Ehlers-Danlos Syndrome
Group of connective tissue disorders with arterial fragility and risk of spontaneous vascular complications.
Full Definition
Ehlers-Danlos syndrome encompasses a group of inherited connective tissue disorders characterized by defects in collagen synthesis or structure. Of particular concern in vascular medicine is the vascular type (Type IV or vEDS), which carries a high risk of spontaneous arterial rupture, dissection, or organ perforation due to marked arterial fragility. Patients typically have characteristic facial features, thin translucent skin, and easy bruising. The median survival is reduced due to vascular complications, which can occur at any age and may be precipitated by minimal trauma or occur spontaneously.
Usage
Usage note: Specify subtype when known. Vascular type (vEDS) has highest risk of complications. Hyphenate the eponym.
In Context
- "The spontaneous carotid dissection raised suspicion for vascular Ehlers-Danlos syndrome." — Clinical assessment
- "Genetic counseling was recommended for Ehlers-Danlos syndrome given the family history of arterial rupture." — Risk assessment