Ehlers-Danlos Syndrome Flashcards
1
Q
Pathogenesis
A
heterogeneous condition of abnormal elastin and collagen formation (>10 types)
2
Q
who gets it?
A
often autosomal dominant
3
Q
how does it present?
A
pain profound joint hypermobility and instability vascular fragility - ease of bruising skin hyperelasticity scoliosis heart problemsautonomic dysfunction inner organ involvement
4
Q
how is this management?
A
dislocating joints - bony surgery may be required bleeding can be a problem skin healing can be poor - stretched scars/ wound dehisence
