Article Text

other Versions

PDF
Letter
New insights into the visual prognosis of pseudoxanthoma elasticum
  1. Jean-Marc Ebran1,2,
  2. Dan Milea1,3,
  3. Anne Trelohan1,
  4. Pierre Bonicel4,
  5. Jean-François Hamel5,
  6. Georges Leftheriotis2,
  7. Ludovic Martin2
  1. 1Department of Ophthalmology, LUNAM University, Angers Hospital, Angers, France
  2. 2LUNAM University, PXE Consultation Centre, Angers Hospital, Angers, France
  3. 3Singapore National Eye Centre, Singapore Eye Research Centre and Duke-NUS, Singapore
  4. 4Department of Ophthalmology, Orléans Hospital, Orléans, France
  5. 5Maison de la Recherche, LUNAM University, Angers Hospital, Angers, France
  1. Correspondence to Dr Jean-Marc Ebran, Ophthalmology Department, LUNAM University, Angers Hospital, CHU Angers, 4 rue Larrey, Angers 49000, France; jmebran{at}chu-angers.fr

Statistics from Altmetric.com

Pseudoxanthoma elasticum (PXE) is a rare autosomal recessive disease of the connective tissue which primarily affects the skin, the retina and the cardiovascular system.1 PXE is characterised by mineralisation and fragmentation of elastic fibres. Eye involvement results from calcification and breaks in the Bruch’s membrane (BM), causing a variety of ocular fundus changes (figure 1).1 ,2 Among them, angioid streaks can be complicated by choroidal neovascularisation and/or macular atrophy resulting in central visual loss.1 Although central visual loss in PXE profoundly impacts quality of life,1 little is known about other risk factors for visual impairment. The aim of our study was to determine if visual impairment related to maculopathy in PXE may be associated with age and other specific retinal features.

Figure 1

Retinal features of pseudoxanthoma elasticum. (1) Angioids streaks and optic nerve drüsen (red-free imaging). (2) Peripheral ‘comet tails’ lesions (red-free imaging). (3) …

View Full Text

Request permissions

If you wish to reuse any or all of this article please use the link below which will take you to the Copyright Clearance Center’s RightsLink service. You will be able to get a quick price and instant permission to reuse the content in many different ways.