High-Resolution Optical Coherence Tomography Correlates of Peau d'Orange in Pseudoxanthoma Elasticum.
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.
IMPORTANCE: Pseudoxanthoma elasticum (pseudoxanthoma elasticum) is a rare inherited disorder marked by progressive calcification of Bruch membrane (bruch membrane).
To identify high-resolution optical coherence tomography (high-resolution optical coherence tomography) correlates of peau d'orange and continuously calcified bruch membrane in patients with pseudoxanthoma elasticum.
DESIGN, SETTING, AND PARTICIPANTS: This prospective case-control study was conducted at the Department of Ophthalmology, University Hospital, Bonn, Germany.
In eyes with type 1 macular neovascularization, layer splitting confirmed anatomical identification of the retinal pigment epithelium and bruch membrane.
Angioid streaks on fundus imaging corresponded to focal bruch membrane disruptions on high-resolution optical coherence tomography.
This study demonstrated that high-resolution optical coherence tomography can identify distinct in vivo correlates of Bruch membrane calcification in pseudoxanthoma elasticum.
The observed transition from multilayered to monolayer retinal pigment epithelium/bruch membrane architecture with accompanying hyporeflective separation may serve as a sensitive imaging biomarker of disease progression in future clinical trials.
These findings support high-resolution optical coherence tomography as a valuable tool for diagnosis, monitoring, and clinical trial end point development in pseudoxanthoma elasticum.