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Pseudoxanthoma elasticum

ORPHA:758· ICD-10 Q82.8

Definition

A rare, genetic, metabolic disease with connective tissue and eye involvement, characterized by progressive ectopic mineralization and fragmented elastic fibers in the skin, retina and vascular walls.

Prevalence
1-9 / 100 000
Inheritance
Autosomal recessive
Age of onset
All ages