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New research · Ophthalmology
JAMA ophthalmology · 23h
Case-controlJAMA ophthalmology · 2025

High-Resolution Optical Coherence Tomography Correlates of Peau d'Orange in Pseudoxanthoma Elasticum.

Jonathan Meinke, Kristin Raming, Carolina Kessler … Kristina Pfau
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OphthalmologyCase-control

High-resolution optical coherence tomography reveals distinct structural changes in pseudoxanthoma elasticum, absent in controls.

High-Resolution Optical Coherence Tomography Correlates of Peau d'Orange in Pseudoxanthoma Elasticum.

Jonathan Meinke et al. · JAMA ophthalmology · 2025
Background

IMPORTANCE: Pseudoxanthoma elasticum (PXE) is a rare inherited disorder marked by progressive calcification of Bruch membrane (BrM).

Purpose

To identify high-resolution optical coherence tomography (HR-OCT) correlates of peau d'orange and continuously calcified BrM in patients with PXE.

Methods

DESIGN, SETTING, AND PARTICIPANTS: This prospective case-control study was conducted at the Department of Ophthalmology, University Hospital, Bonn, Germany.

n = 42 eyes
0
Results
of 28
distinct structural changes were absent in all 28 healthy control eyes
n = 42 eyes
More results

In eyes with type 1 macular neovascularization, layer splitting confirmed anatomical identification of the RPE and BrM.

Angioid streaks on fundus imaging corresponded to focal BrM disruptions on HR-OCT.

“
Conclusion · 1 of 3

This study demonstrated that HR-OCT can identify distinct in vivo correlates of Bruch membrane calcification in PXE.

Conclusion · 2 of 3

The observed transition from multilayered to monolayer RPE/BrM architecture with accompanying hyporeflective separation may serve as a sensitive imaging biomarker of disease progression in future clinical trials.

Conclusion · 3 of 3

These findings support HR-OCT as a valuable tool for diagnosis, monitoring, and clinical trial end point development in PXE.

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