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May 1961

Ocular Findings in Hereditary Ochronosis

Author Affiliations

Los Angeles
From the Department of Pathology, and the Division of Ophthalmology, Department of Surgery, University of California Medical Center.

Arch Ophthalmol. 1961;65(5):657-668. doi:10.1001/archopht.1961.01840020659010

No feature of hereditary ochronosis is more prominent than the pigmentation that occurs in the eye. Nonetheless, the pathology of affected eyes has received only slight attention. A review of the literature revealed only a single report11 describing the gross examination of an enucleated ochronotic globe, 2 recorded biopsies of involved conjunctiva and cornea,18,20 and 1 account of the histological examination of an ochronotic globe.19

This report describes the findings of hereditary ochronosis, including gross and microscopic features of the eyes, in an 83-year-old man, who died of myocardial infarction. The ochronotic lesions demonstrate the destructive features of the disease and emphasize the diverse and widespread pigment deposition.

Report of Case  An 83-year-old retired architect entered the University of California Medical Center for evaluation of abdominal pains and nausea of 6 months' duration.For most of his life this patient had noted that his urine was yellow

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