As a consequence of aging, fine skin wrinkles appear secondary to the decreased synthesis and net loss of dermal collagen and elastin.
Ehlers-Danlos Syndrome
defective collagen synthesis due to deficiency in procollagen peptidase, the enzyme that cleaves terminal propeptides from procollagen in the extracellular space.
Type I is the most common ( among ~6 types)
- Droopy ears,
- hyperextensible skin, fragile, easy bruisability, poor wound healing
- joint hyperlaxity; tendency toward hip, shoulder, knee, and clavicular dislocation
- MVP, tricuspid valve prolapse, aortic root dilatation; dissecting aneurysm, ASD
- Pt. can develop acute MR secondary to myxomatous degeneration and rupture of the chordae tendineae
- blue sclera, myopia, glaucoma, ectopia lentis, retinal detachment
- intracranial aneurysm