Polypoidal choroidal vasculopathy in a patient with angioid streaks secondary to pseudoxanthoma elasticum

  • Stéphanie Baillif-Gostoli
  • Maddalena Quaranta-El Maftouhi
  • Martine Mauget-Faÿsse
Letter to the Editor

Dear Editor,

Pseudoxanthoma elasticum (PXE) is an inherited disorder characterized by the accumulation of calcified and fragmented elastin fibers in the skin, vascular walls, and Bruch’s membrane [1]. Angioid streaks (AS), often complicated by secondary choroidal neovascularization (CNV), are the main ophthalmic manifestations of PXE.

Polypoidal choroidal vasculopathy (PCV) has been proposed to represent a variant of CNV [2]. It now seems that alterations of the elastin matrix in vascular walls may be implicated in the occurrence of PCV.

We report a case of PCV in a patient diagnosed with PXE and formerly treated for subfoveal CNV secondary to AS.

A 51-year-old woman with skin biopsy-proven PXE was referred in April 2005 for left subfoveal CNV secondary to angioid streaks. At first presentation, best-corrected visual acuity was 20/125 in the left eye (OS) and 20/20 in the right eye (OD). Fundus examination revealed bilateral peripapillary AS associated, in the left eye, with a large...


Intravitreal Injection Polypoidal Choroidal Vasculopathy Polypoidal Lesion Verteporfin Serous Retinal Detachment 
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Copyright information

© Springer-Verlag 2010

Authors and Affiliations

  • Stéphanie Baillif-Gostoli
    • 1
    • 3
  • Maddalena Quaranta-El Maftouhi
    • 2
  • Martine Mauget-Faÿsse
    • 2
  1. 1.Saint Roch University HospitalNiceFrance
  2. 2.Rabelais Ophthalmologic CenterLyonFrance
  3. 3.Department of OphthalmologySaint Roch HospitalNiceFrance

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