This uncommon condition is characterised by breaks in Bruch's membrane which resemble blood vessels. They are frequently associated with pseudoxanthoma elasticum; less often, with sickle cell disease and Paget's disease. They may cause disciform macular degeneration (exudative).
Other associations include:
- Ehlers-Danlos syndrome
- lead poisoning
- pituitary disorders and other intracranial disorders
- Lebwohl M et al (1993).Brief report: occult pseudoxanthoma elasticum in patients with premature cardiovascular disease. NEJM, 339(17), 1237-53.
Last reviewed 01/2018