Pseudoxanthoma elasticum (PXE) with angioid streaks in the fundus comprise Groenblad-Strandberg disease. There are gray or red lines radiating peripherally from the fundus with striking skin changes including thickened soft easy to lift fold in the inguinal and lower abdominal area. Angioid streaks are not specific, but are present in 85% of cases. They are due to ruptured Bruch's membrane.
PXE is a rare AR CT disease. There is a slight preponderance of women. There is multisystem involvement with premature ocular, skin and cardiovascular complications leading to reduced life expectancy. HTN is common and GI hemorrhage occurs. PXE leads to stroke premature athero, hemorrhages, sacular aneurysms, large artery disease. Skin biopsy is diagnostic.
Genetic ABCC-6
laxity of the skin, redundant skin, and ocular fundus abnormalities. Associations include MVP, cardiac abnormalities. Complications include stroke, intracranial hemorrhage, seizures and dementia.
PXE is a rare AR CT disease. There is a slight preponderance of women. There is multisystem involvement with premature ocular, skin and cardiovascular complications leading to reduced life expectancy. HTN is common and GI hemorrhage occurs. PXE leads to stroke premature athero, hemorrhages, sacular aneurysms, large artery disease. Skin biopsy is diagnostic.
Genetic ABCC-6
laxity of the skin, redundant skin, and ocular fundus abnormalities. Associations include MVP, cardiac abnormalities. Complications include stroke, intracranial hemorrhage, seizures and dementia.
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http://images.google.com/images?gbv=2&hl=en&sa=1&q=pseudoxanthoma+elasticum+pictures&aq=2&oq=pseudoxanthoma
are google images of (many)
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