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DYSTROPHIE AILES PAPILLON [2 records]

Record 1 2025-09-11

English

Subject field(s)
  • Visual Disorders
CONT

Retinal pattern dystrophies are a slowly progressive heterogeneous group of primarily autosomal dominantly inherited macular diseases whose unifying element involves the deposition of pigment in the retinal pigment epithelium (RPE) of the macula. Although findings are classically and most often centered in the macula, pigment deposition may also occur in the periphery. Depending on the distribution pattern, these pattern dystrophies can be separated into major categories or types. These include reticular dystrophy, fundus pulverulentus, butterfly-shaped pigment dystrophy, adult-onset foveomacular vitelliform dystrophy, and multifocal pattern dystrophy simulating Stargardt disease.

French

Domaine(s)
  • Troubles de la vision
CONT

Les dystrophies réticulées regroupent de nombreux phénotypes, incluant entre autres la dystrophie pseudo-vitelliforme de l'adulte, les dystrophies réticulées en ailes de papillon, les dystrophies réticulées de l'EP [épithélium pigmentaire], les pseudo-Stargardt, les fundus pulveruluntus.

Spanish

Save record 1

Record 2 2025-08-26

English

Subject field(s)
  • Visual Disorders
CONT

Butterfly-shaped pigment dystrophy ... In this autosomal dominant macular dystrophy, a spoke-like pigment pattern that may resemble the shape of a butterfly is observed in the macula. The phenotype belongs to the group of pattern dystrophies, a heterogeneous spectrum of retinal disorders, characterized by a variety of yellow, orange, grey-green or darkly pigmented patterns.

OBS

Butterfly-shaped dystrophy is ... first evident in late childhood, characterized by normal or mildly reduced visual acuity, and normal color vision, peripheral visual fields, and dark adaptation.

French

Domaine(s)
  • Troubles de la vision
DEF

Affection maculaire rare et peu évolutive où l’épithélium pigmenté maculaire est remanié et présente des travées radiaires d’hyperplasie, aux extrémités renflées, centrées sur la fovéola et de la longueur d’un diamètre papillaire, formant ainsi une image en croix ou en ailes de papillon.

OBS

La dystrophie maculaire en ailes de papillon est une affection autosomique dominante.

Spanish

Save record 2

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