title: Centrifugal fundus abnormalities in pseudoxanthoma elasticum.
abstract: OBJECTIVE
  To investigate the Bruch's membrane-retinal pigment epithelium (RPE) complex in pseudoxanthoma elasticum (PXE) by imaging techniques capable of visualizing deep retinal structures with high contrast.
  
  
  METHODS
  Prospective cross-sectional study.
  
  
  METHODS
  Patients with PXE, confirmed by mutation analysis, skin histopathologic examination, or both.
  
  
  METHODS
  Sixteen patients were investigated by indocyanine green (ICG) and fluorescein angiography, confocal near-infrared (NIR) reflectance, and fundus autofluorescence imaging using a confocal scanning laser ophthalmoscope (Spectralis HRA-OCT; Heidelberg Engineering, Heidelberg, Germany). Composite digital fundus photography also was performed.
  
  
  METHODS
  Characterization and topographic distribution of abnormalities detected by retinal imaging.
  
  
  RESULTS
  On late-phase ICG angiography, a central area of decreased fluorescence centered on the posterior pole was a characteristic finding in all patients. A second area characterized by increased fundus reflectivity on NIR reflectance imaging extended further into the periphery. A third and most eccentric area showed neither decreased ICG fluorescence nor increased fundus reflectivity. These 3 areas were separated by 2 transition zones, the second being the equivalent of peau d'orange. Angioid streaks did not extend into the third area.
  
  
  CONCLUSIONS
  The abnormalities detected by this multimodal imaging approach suggest a centrifugal spread of the retinal pathologic features of the Bruch's membrane-RPE complex in PXE. Decreased fluorescence on late-phase ICG angiography is a consistent sign of retinal pathologic features in PXE. Bruch's membrane calcification may be the underlying cause for the increased reflectivity observed on NIR reflectance imaging.
  
  
  BACKGROUND
  Proprietary or commercial disclosure may be found after the references.
authors.affiliation: Nuffield Laboratory of Ophthalmology, University of Oxford, United Kingdom.
authors.forename: Peter
authors.initials: P
authors.lastname: Charbel Issa
authors.forename: Robert P
authors.initials: RP
authors.lastname: Finger
authors.forename: Christian
authors.initials: C
authors.lastname: Götting
authors.forename: Doris
authors.initials: D
authors.lastname: Hendig
authors.forename: Frank G
authors.initials: FG
authors.lastname: Holz
authors.forename: Hendrik P N
authors.initials: HP
authors.lastname: Scholl
mesh_terms: D000328:Adult; D000793:Angioid Streaks / Q000175:diagnosis; D016570:Bruch Membrane / Q000473:pathology*; D004396:Coloring Agents; D003430:Cross-Sectional Studies; D005260:Female; D005451:Fluorescein Angiography; D005654:Fundus Oculi; D006801:Humans; D007208:Indocyanine Green; D008297:Male; D018613:Microscopy, Confocal; D008875:Middle Aged; D011446:Prospective Studies; D011561:Pseudoxanthoma Elasticum / Q000175:diagnosis*; D012164:Retinal Diseases / Q000175:diagnosis*; D055213:Retinal Pigment Epithelium / Q000473:pathology*
chemical_list: D004396:Coloring Agents; D007208:Indocyanine Green