Subretinal fibrosis is associated with fundus pulverulentus in pseudoxanthoma elasticum

Ebran, Jean‐Marc; Martin, Ludovic; Leftheriotis,; Navasiolava, Nastassia; Ferré, Marc; Miléa, Dan; Leruez, Stéphanie

doi:10.1007/s00417-018-3937-5

Cited by 2 publications

(1 citation statement)

References 30 publications

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“…The extent of the subretinal fibrosis was atypical and uncharacteristic of previously described fibrovascular scarring due to secondary CNVM in patients with PXE. 4,5 Furthermore, there was no evidence of active CNVM visualized on FA or ICG on prior ophthalmic imaging (however, blockage was noted in the area of peripapillary fibrosis in the right eye). Given the presence of vitreous cells in both eyes, ellipsoid zone attenuation on OCT, multifocal chorioretinal lesions, the rapid progression of subretinal fibrosis, and persistent IRF fluid despite antivascular endothelial growth factor injections, an ocular inflammatory phenotype of PXE was considered.…”

Section: Case Reportmentioning

confidence: 96%

Extensive Subretinal Fibrosis Associated with Pseudoxanthoma Elasticum

Oyeniran

Wiley

Bellur

et al. 2023

RETINAL Cases &Amp; Brief Reports

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Purpose: The purpose of this study was to report an unusual case of pseudoxanthoma elasticum presenting with an inflammatory phenotype associated with atypical and rapidly progressive subretinal fibrosis.Methods: This was an observational case report.Results: A patient with a history of pseudoxanthoma elasticum presented with rapidly progressive subretinal fibrosis, particularly in the left eye, over the course of one year. The patient was noted at presentation to have intraocular inflammation, outer retinal attenuation, multifocal choroiditis-like lesions, and intraretinal fluid (in the absence of obvious clinical or angiographic signs of exudative choroidal neovascular membranes). An ocular inflammatory phenotype was diagnosed, and the patient was treated with a combination of local steroids and systemic corticosteroids/immunomodulatory agents. After initiation of these agents, the patient demonstrated functional and structural improvement, with partial outer retinal reconstitution, decreased intraretinal fluid, and lack of further progression of subretinal fibrosis.Conclusion: This report describes an inflammatory phenotype of pseudoxanthoma elasticum associated with severe and atypical subretinal fibrosis. This case expands upon the currently known spectrum of inflammatory phenotypes associated with pseudoxanthoma elasticum. Treatment with corticosteroids or immunomodulatory treatment should be considered in similar cases.

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Section: Case Reportmentioning

confidence: 96%