Angioid Streaks with Pseudoxanthoma Elasticum (Gronblad-Strandberg Syndrome)

Pseudoxanthoma elasticum (Grönblad-Strandberg syndrome).

Pseudoxanthoma elasticum (PXE) is a rare disorder of elastin which primarily involves the skin, cardiovascular system, and eyes, but also manifests in the central nervous system, skeleton, and gastrointestinal tract. Rigall first described the cutaneous lesions of PXE in 1881 and thought the disorder was akin to the xanthomatoses. In 1896, Darier2 showed the typical histological changes in elas...

Angioid Streaks in Psuedoxanthoma Elasticum

Pseudoxanthoma elasticum (PXE) is a hereditary disorder that affects primarily the elastic tissues of skin, eyes and blood vessels. Its estimated prevalence is thought to be 1:25,000 100,000. PXE patients also carry an increased risk for cardiovascular and gastrointestinal diseases. Early diagnosis can play a crucial role in preventing early loss of vision and systemic complications in these pa...

Fluorescein study of a family with angioid streaks and pseudoxanthoma elasticum.

Gronblad Strandberg syndrome with vertibrobasilar dolichoectasia.

The constellation of pseudoxanthoma elasticum, ocular involvement and vascular disturbances is termed Gronblad Strandberg syndrome. Pseudoxanthoma elasticum is a genetic disorder characterized by progressive calcification and fragmentation of elastic fibres in the skin, retina and the cardiovascular system. Typically the cutaneous lesions begin in childhood, but because of their asymptomatic na...

Choroidal neovascularization in angioid streaks and pseudoxanthoma elasticum: 1 year follow-up.

PURPOSE To report the efficacy of intravitreal injection of bevacizumab 1.25 mg (IVB) in the treatment of choroidal neovascularization (CNV) secondary to angioid streaks (AS). METHODS Case review of two patients with CNV secondary to AS treated with three IVB with a 1-year follow up. RESULTS In both patients after 1 year fluorescein angiography and optical coherence tomography showed comple...