Gronblad-Strandberg syndrome

Abstract

The Grönblad-Strandberg syndrome, also known as pseudoxanthoma elasticum, is a rare congenital dysplasic disorder of the connective tissue, characterized by a progressive abnormal mineralization and dystrophic calcification of elastic tissues and collagen (1). The mode of inheritance is uncertain, but autosomal recessive inheritance can be assumed. This process affects tissues rich in elastic fibres and multiple systems of the organism, being the most common the cutaneous (pseudoxanthoma elasticum), ocular (angioid streaks), vascular (occlusive vascular disease) and gastrointestinal manifestations. These findings progress through the life of the affected person, with variable pattern clinic and evolution.