A family of genetic disorders, w/variable affects on connective tissues
Risk Factors
Family hx
Symptoms
Kyphoscoliosis
Vary based on degree of dysfxn and types of collagen affected
Skin hyper-extensability, joint hyper-mobility, frequent dislocations w/o sig force, easy bruising, poor wound healing, sig chronic pain of joints, back as age
Aneurysms in vascular tree - can cause symptom if they rupture (pain, hypovolemia, hypotension, death), contribute to embolic events or are involved with a dissection
Physical Exam Findings
Varies based on type of collagen affected, can include: joint subluxation, joint hyper-mobility, stretchy skin that snaps back
Skin can be paper thin or velvety
Echymoses, hematomas
Wide "cigarette paper" scars that bleed easily
Palpable aneurysms if located in an artery that that's accessible during exam (e.g. abdominal aortic aneurysm if patient thin, peripheral artery)