Angiod streaks are seen in which of the following: March 2009

Ans. B: Pseudoxanthoma elasticum Angioid streaks/Knapp streaks/Knapp striae are small breaks in Bruch's membrane, an elastic tissue containing membrane of the retina that can become calcified and crack. Hermann Jakob Knapp called them "angioid streaks" because of their resemblance to blood vessels. It is believed that its pathology may be a combination of elastic degeneration of Bruch's membrane, iron deposition in elastic fibers from hemolysis with secondary mineralization, and impaired nutrition due to stasis and small vessel occlusion. Angioid streaks are often associated with: Pseudoxanthoma elasticum (PXE) Paget's disease Sickle cell anemia Ehlers-Danlos Syndrome These streaks can have a negative impact on vision due to choroidal neovascularization or choroidal rupture. Also, vision can be impaired if the streaks progress to the fovea and damage the retinal pigment epithelium