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May 1982

Scleropachynsis Maculopathy

Author Affiliations

From the Eye Pathology Laboratory of the Wilmer Institute (Drs Conn and Green and Ms de la Cruz) and the Departments of Pathology (Drs Conn and Green and Ms de la Cruz) and Biostatistics (Dr Hillis), The Johns Hopkins Medical Institutions, Baltimore.

Arch Ophthalmol. 1982;100(5):793-799. doi:10.1001/archopht.1982.01030030797015

• To our knowledge, this is the first clinicopathologic report of a previously unrecognized maculopathy in which there is thickening and mucopolysaccharide deposition in the sclera subjacent to the macula. A healthy 50-year-old man had bilateral mottling of the retinal pigment epithelium (RPE) in the macular region. The left eye was enucleated because of a choroidal melanoma. Histopathologic examination disclosed a thickened sclera with abnormal collagen fibrils with diameters up to 5,800 A and compressed choroid. The overlying RPE had areas of hypopigmentation, hyperpigmentation, and hyperplasia and contained intracytoplasmic lipofuscin.

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