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Ashley E. Brown, Charles Clifton Wykoff, Sylvia Hsu, David M Brown; Dermatopathologic and Optical Coherence Tomography “Cracked Mud Sign” in Pseudoxanthoma Elasticum.. Invest. Ophthalmol. Vis. Sci. 2018;59(9):1129. doi: https://doi.org/.
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© ARVO (1962-2015); The Authors (2016-present)
To describe and compare mid-dermis histopathology and retinal pigment epithelium (RPE)–Bruch’s membrane complex spectral domain optical coherence tomography (SD-OCT) findings in pseudoxanthoma elasticum (PXE).
3-D reconstruction (C-scans) and horizontal SD-OCT images (Heidelberg Spectralis) were compared to dermatopathologic findings examined by light microscopy in eyes and skin affected by PXE.
SD-OCT images (horizontal and 3-D constructions) revealed concave truncations and displacement of the RPE–Bruch’s membrane complex into the photoreceptor layers in eyes of advanced PXE. These findings are comparable to the dermatopathologic features of fragmentation and calcification of elastic fibers in the mid-dermis. This SD-OCT “cracked mud” sign increased with progressive aging and persisted even in areas of geographic atrophy.
As PXE eyes often present with progressing geographic atrophy, late cases can be mistaken for age-related macular degeneration. Identification of the presence of the specific SD-OCT finding of concave disruptions of the RPE–Bruch’s membrane complex (“cracked mud”) should alert clinicians of the possibility of PXE as the underlying disease mechanism. While the diagnosis of PXE via skin biopsy is well described, the non-invasive SD-OCT finding of “cracked mud” can help confirm the diagnosis and is phenotypically similar to the mid-dermis fragmentation.
This is an abstract that was submitted for the 2018 ARVO Annual Meeting, held in Honolulu, Hawaii, April 29 - May 3, 2018.
Top surface of RPE-Bruch's membrane on SD-OCT reconstructions revealed 3D nature of "cracked mud sign" (top right) as demonstrated on horizontal SD-OCT scan (top left).
Fragmentation and calcification of the mid-dermis analgous to SD-OCT findings of RPE-Bruch's membrane complex.
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