ABCC6 gene → liver produces less anti-mineralization factors due to absent ABCC6 protein
Ectopic mineralization → calcification of connective tissues (eyes, arterial walls and dermis)
Typical skin findings
Clumped, calcified elastic fibers in the dermis → grouped yellowish papules typically on the neck or flexural areas giving a “plucked chicken” appearance
Systemic risks
Angioid streaks (eyes) → weakening of Bruch membrane
Calcification of medium-sized vessels leads to atheromatous plaques → risk of peripheral artery disease