Table 1.

Alterations found histologically and radiologically in the two PXE patients

	Histology	Radiology
Macroscopically altered skin	Degeneration and calcification of the elastic fibres in the mid and lower dermis	Thickened skin
Macroscopically normal skin	Degeneration and calcification of the elastic fibres in the septa between the subcutaneous fatty layer	–
Heart	Degeneration and calcification of elastic fibres mainly underneath the endocardial layer and to a lesser extent in fibrous tissue between the cardiomyocytes	–
Arteries (lower extremity, gastroepiploic artery)	Both atherosclerotic intimal lesions and calcifications in the medial layer	More or less circumferential calcifications in case of medial calcification, thick dots of calcification in case of intimal calcification
Arteries (other)	Small scattered calcified elastic fibres in the media and/or internal and external elastic lamina Atherosclerotic intimal lesions	Thick dots of calcification in case of intimal calcification
Central nervous system	Lacunar infarction	–
Central nervous system	White matter abnormalities	Non-specific abnormalities in the white matter area
Central nervous system	Calcification of the small arteries in the area of the globus pallidus and hippocampal area	–
Kidney	Kidney stone	Kidney stone
Adrenal gland	Myelolipoma with calcifications and bony transformation	Calcified adrenal gland
Gallbladder	Gallstones	Gallstones
Lung	Some degenerated and calcified elastic fibres in the pleura	Thickened pleura
Oesophagus	Some degenerated and calcified elastic fibres around the oesophagus	–