Pseudoxanthoma elasticum (PXE) is an inherited connective tissue disorder, characterized predominantly by skin, eye, cardiac, and vascular abnormalities. Hypertension is common, and elevated blood pressure and vascular lesions often lead to strokes and damage to many body organs. The skin manifestations were first described by the French dermatologist Rigal in 1881. Two Swedish physicians, Grnblad (an ophthalmologist) and Strandberg (a dermatologist), in 1929 recognized that the skin findings were accompanied by angioid streaks in the retina. PXE is often referred to as Gronblad-Strandberg disease after these two physicians.
ASJC Scopus subject areas