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Reticular-like pigmentary patterns in pseudoxanthoma elasticum.
H R McDonald1, H Schatz, T M Aaberg
1Department of Ophthalmology, University of California, San Francisco.
Ophthalmology
|March 1, 1988
Summary
Pseudoxanthoma elasticum (PXE) can cause distinctive retinal pigmentary changes. These changes, including reticular patterns, are associated with an increased risk of subretinal neovascularization in PXE patients.
Area of Science:
- Ophthalmology
- Medical Genetics
- Retinal Diseases
Background:
- Pseudoxanthoma elasticum (PXE) is a rare genetic disorder affecting elastic tissue.
- PXE can manifest with characteristic skin and eye findings.
Observation:
- Retinal pigmentary changes were observed in 9 of 14 (64%) patients with PXE.
- These changes included scattered dots, linear "string of pearls" patterns, and reticular (fishnet) patterns.
Findings:
- Subretinal neovascularization (SRNV) occurred in 12 of 18 (67%) eyes with pigmentary changes.
- SRNV was less common (2 of 10 eyes, 20%) in eyes without these specific pigmentary findings.
Implications:
- Specific retinal pigmentary patterns in PXE may indicate a higher risk for developing SRNV.
- Ophthalmologists should carefully examine PXE patients for these retinal signs to assess their risk for vision-threatening complications.