Measurement Reliability and Functional Validity of Bruch's Membrane Calcification in Pseudoxanthoma Elasticum: PROPXE Study Report 2

Patients with PXE demonstrate significant rod-mediated dark adaptation deficits correlating with the temporal inner peau d'orange boundary extent. These findings support the use of BrM calcification extent as an objective marker for disease severity, facilitating earlier therapeutic intervention than currently possible in PXE.

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Publication Details

Journal
Investigative Ophthalmology & Visual Science
Published
2025-11-13
DOI
https://doi.org/10.1167/iovs.66.14.29
Primary Topic
Dermatological and Skeletal Disorders
Type
article
Field-Weighted Citation Impact
0.00
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article

Measurement Reliability and Functional Validity of Bruch's Membrane Calcification in Pseudoxanthoma Elasticum: PROPXE Study Report 2

Maximilian Pfau, Georg Ansari, Sharon F. Terry, Kristina Pfau et al.
Investigative Ophthalmology & Visual Science
Dermatological and Skeletal Disorders
article

Measurement Reliability and Functional Validity of Bruch's Membrane Calcification in Pseudoxanthoma Elasticum: PROPXE Study Report 2

Maximilian Pfau, Georg Ansari, Sharon F. Terry, Kristina Pfau, Peter Charbel Issa, Jason A. W. Giger
article en

Abstract

Patients with PXE demonstrate significant rod-mediated dark adaptation deficits correlating with the temporal inner peau d'orange boundary extent. These findings support the use of BrM calcification extent as an objective marker for disease severity, facilitating earlier therapeutic intervention than currently possible in PXE.

Investigative Ophthalmology & Visual ScienceVol. 66(14)
Twitter (United States) (US), University of Bonn (DE), University of Basel (CH), ORCID (US), Technical University of Munich (DE)
Openalex Percentile: Top 64%
Dermatological and Skeletal Disorders
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