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A 33 year old man with difficulty adapting to light
Digital Journal of Ophthalmology 2004
Volume 10, Number 6
April 24, 2004
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Manuel Perrier | Université de Montréal
Diagnosis and Discussion
Best’s disease is an autosomal dominant macular dystrophy of variable penetrance in which lipofuscin granules accumulate in retinal pigment epithelial cells(1,2). It typically affects young patients in whom a macular lesion gradually evolves through several characteristic stages(3). In this report, we present an unusual case of extensive multifocal Best’s disease.

Multifocal Best’s disease is an unusual form of this disorder that can occur in patients without family history(4,5,6). The reduction in visual acuity seems more rapid and severe and the lesions can appear, disappear or coalesce(4).

In this case, the additional presence of hepatitis C (without any known risk factors) is an atypical feature. An extensive review of the english literature did not reveal any known association between hepatitis C and Best’s disease.