Martinique crinkled retinal pigment epitheliopathy

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Martinique crinkled retinal pigment epitheliopathy

Synonyms: MCRPE

A rare genetic retinal disease characterized by characteristic “dried-out soil” fundus pattern due to diffuse deep white lines in the macula to the level of the retinal pigment epithelium which is slightly elevated and rippled. Macular exudation may be associated and Bruch’s membrane may be affected too. Occasionally peripheral nummular pigmentary changes may be observed associated with blindness. The lesions enlarge with time with a preferential macular extension and confluence. Complications may include polypoidal choroidal vasculopathy choroidal neovascularization or atrophic fibrous macular scarring that can lead to reduced visual acuity over time.

Data from Orphanet are used to provide information on a disease's name, synonym(s), and overview.

Reference: Access aggregated data from Orphanet at Orphadata.

Orphadata: Free access data from Orphanet. © INSERM 1999. Available on http://www.orphadata.org. Data version March 2024

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Martinique crinkled retinal pigment epitheliopathy?

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