Hypertrophy with hyperpigmentation of the retinal pigment epithelium

Fifty-two patients (one bilateral) exhibiting hypertrophy with hyperpigmentation of the retinal pigment epithelium (RPE) are described. Visual acuity was not affected, and the patients were asymptomatic. There was no correlation with systemic diseases, family history of eye disease, associated fundus lesions, anterior segment abnormalities, or intraocular pressure. Field defects were rarely demonstrated using standard clinical techniques. There were three characteristic locations and four characteristic pigmentary variations that occurred alone or in any combination. The lesions were gray, black, or brown. The clinical characteristics and histopathology suggest that hypertrophy with hyperpigmentation of the RPE and congenital grouped pigmentation are different expressions of a similar condition, with the former being focal and the latter multifocal.