Extracted plasma CS was evaluated for the main two unsaturated disaccharides, non-sulfated and 4-monosulfated, and the charge density determined. The mean values were 0.54 ± 0.13 for volunteers, 0.60 ± 0.15 for PXE subjects and 0.50 ± 0.15 for the carriers. A significant increase of + 11.1 % was found between the PXE patients and healthy human group but no differences were calculated between the control group and the carriers. Furthermore, besides a CS amount, the volunteers aging from 17 to 40 years (mean 32.1) showed a charge density of 0.53 ± 0.14 while the group ranging from 50 to 74 years (mean 56.9) had a value of 0.58 ± 0.17 with a significant increase of + 9.4 % . The same trend was measured for the healthy carriers group. The CS charge density of PXE-affected subjects was found to increase significantly more than healthy controls depending on the age. In fact, the PXE patients aging from 10 to 40 years (mean 29.3) showed a charge density of 0.56 ± 0.14 while the group ranging from 50 to 74 years (mean 58.6) had a value of 0.67 ± 0.11 with a significant increase of + 19.6 % . Furthermore, the group of PXE-affected subjects ranging from 50 to 74 years (mean 58.6) showed a significant increase of 15.5 % in comparison with the group matched for age (mean 56.9) of healthy volunteers.