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Related Experiment Videos

Spectrum of pattern dystrophy in pseudoxanthoma elasticum.

Anita Agarwal1, Purnima Patel, Tony Adkins

  • 1Vanderbilt Eye Institute, Vanderbilt University Medical Center, Nashville, TN 37232, USA.

Archives of Ophthalmology (Chicago, Ill. : 1960)
|July 13, 2005
PubMed
Summary

Pattern dystrophy occurs in pseudoxanthoma elasticum (PXE), with five types observed, including two new associations. Angioid streaks and other ocular findings are common in PXE patients with pattern dystrophy.

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Area of Science:

  • Ophthalmology
  • Medical Genetics
  • Dermatology

Background:

  • Pseudoxanthoma elasticum (PXE) is a rare genetic disorder affecting elastic tissue, primarily the skin and eyes.
  • Ocular manifestations of PXE, such as angioid streaks, are well-documented.
  • The association between PXE and specific types of pattern dystrophy requires further elucidation.

Purpose of the Study:

  • To investigate the prevalence and characteristics of pattern dystrophy in patients diagnosed with PXE.
  • To identify the specific types of pattern dystrophy present in PXE patients.
  • To document associated ocular findings in this cohort.

Main Methods:

  • Retrospective review of photographic records from Vanderbilt Eye Institute.
  • Keyword search for

Related Experiment Videos

  • angioid streaks and pseudoxanthoma elasticum
  • identified 28 potential cases.
  • Confirmed PXE diagnosis through medical history, fundus findings, skin examination, or biopsy in 22 subjects.

    • Main Results:

    • Pattern dystrophy was observed in 16 out of 22 PXE patients (73%), involving 27 eyes.
    • The most common types were fundus pulverulentus (14 patients) and butterfly-shaped dystrophy (3 patients).
    • New associations included solitary vitelliform and reticular dystrophy; two patients showed progression between types. Angioid streaks were universal, and other findings included peau d'orange, optic nerve drusen, crystalline bodies, and choroidal neovascularization.

    Conclusions:

    • All five major types of pattern dystrophy can occur in patients with PXE.
    • Two types of pattern dystrophy were identified in PXE patients for the first time.
    • Fluorescein angiography is a valuable tool for characterizing pattern dystrophy in PXE.