Ehlers Danlos syndrome

Question 1

What is Ehlers Danlos syndrome

Answer 1

• this is a heterogeneous group of disorders of collagen, connective tissues
• primarily affecting your skin, joints and blood vessel walls.
• commonly related to over flexible joints and stretchy, fragile skin.

Question 2

what are main different types of disorders of collagen related to Ehlers-danlos syndrome

Answer 2

• types I,II,III - inherited in an autosomal dominant fashion
• types IV (vascular type) - this is also an autosomal dominant and involves arteries, bowel and uterus as well as skin.
• type VI - recessively inherited disorder and results from a mutation in the gene that encodes lysyl hydroxylase.
• type VII - is an autosomal dominant disorder, where there is a defect in the conversion of procollagen to collagen.

Question 3

symptoms of EDS

Answer 3

hyper mobile EDS
- joint hyper mobility and loose, unstable, joints
- joint pain and clicking joints
- fatigue
- skin that bruises easily

classic EDS
- hypermobility
- loose, unstable joints
- stretchy skin
- fragile skin
- wounds that are slow to heal.

vascular EDS (most serious)
- skin that bruises easily
- thin skin with visible blood vessels
- fragile blood vessels
- risk of organ problems
- varicose veins and unusual facial features

Question 3

symptoms of EDS

Answer 3

hyper mobile EDS
- joint hyper mobility and loose, unstable, joints
- joint pain and clicking joints
- fatigue
- skin that bruises easily

classic EDS
- hypermobility
- loose, unstable joints
- stretchy skin
- fragile skin
- wounds that are slow to heal.

vascular EDS (most serious)
- skin that bruises easily
- thin skin with visible blood vessels
- fragile blood vessels
- risk of organ problems
- varicose veins and unusual facial features

Question 4

managment of EDS

Answer 4

• no cure, managment is aimed on pain reduction and prevent complications
• pain - NSAIDs
• blood pressure
  -exercise to strengthen muscles and stabilize joints.