Pseudoxanthoma elasticum is a rare inherited multisystem disorder that is
characterized by a pathological mineralization of the elastic connective tissue,
which involves predominantly the skin, eyes and cardiovascular system. Its cause lies
on mutations in the ABCC6 gene, which lead to reduction or absence of the
transmembrane transport ADP dependent protein (MRP6), causing an accumulation of
extracellular material and subsequent deposition of calcium and other minerals in the
elastic tissue. The authors report two cases of pseudoxanthoma elasticum, emphasizing
its major clinical features and the importance of early diagnosis of the disorder,
aiming for adequate therapeutic management of associated complications.