Gronblad-Strandberg syndrome
Iglesia Puig, M.A.; Puig Galy, J.; Bueno Gómez, J.; Arellano Cabornero, A.; López-Areal García, B.
Anales de Medicina Interna 16(12): 639-646
1999
ISSN/ISBN: 0212-7199 PMID: 10686719 Document Number: 500313
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.