Stroke is also a vascular-related condition frequently reported in PXE patients (Aessopos et al., 1997; van den Berg et al., 2000) but it could well be that strokes etiology in certain PXE individuals might not be related to ABCC6 deficiency as Hornstrup et al. could not statistically link cerebrovascular diseases with the most frequent ABCC6 mutation (p.R1141X). Here, ABCC6 is linked to pseudoxanthoma elasticum (inherited or acquired).