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Hypertrophic retinal pigment epithelium (first type)

The first type represented here is the typical unifocal CHRPE. It is a gray to black, well defined, minimally elevated fundus lesion in the RPE having a diameter in the range of 2 to 6 mm. (The average optic nerve is 1.5 mm in diameter.) Because the receptor cells overlying these lesions are absent, there is an absolute scotoma associated with them. (Contributed by Dr. James Walters.)

Associated Disorders

  • Bassen-Kornzweig disease
  • Cushing syndrome
  • Kearns-Sayre syndrome
  • Lawrence-Moon-Biedl syndrome
  • Mitochondrial encephalopathy with lactic acidosis and stroke-like episodes (MELAS)
  • Ocular paraneoplastic syndromes
  • Refsum disease
  • Usher syndrome
  • gastroesophageal reflux disease
  • posterior vitreous detachment
  • retinal detachment
  • systemic arterial hypertension
  • systemic lupus erythematosus