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A woman in her late 60s presented with progressive, painless blurring of vision in both eyes over the past 6 months. Medical history was significant for Sjögren syndrome, for which she was taking hydroxychloroquine sulfate. Ocular history was notable for neovascular age-related macular degeneration under good control with intravitreal injections in the right eye and intermediate dry age-related macular degeneration in the left eye. Family history was negative for corneal disease.
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Paraproteinemic keratopathy from smoldering myeloma
D. Perform superficial corneal biopsy
The patient presented with bilateral subepithelial and anterior stromal corneal opacification. Antiviral agents, such as acyclovir (choice A), would not be preferred because bilaterality of the process and the lack of inflammatory response are not typical of an infectious origin. Topical cyclosporine (choice C) was not recommended because the absence of appreciable epithelial involvement suggests that keratopathy is unlikely related to Sjögren syndrome.1
The subacute, noninflammatory, and bilateral nature of corneal opacification suggests corneal deposits that are either dystrophic or nondystrophic. Noninvasive genetic testing on peripheral blood for corneal epithelial-stromal dystrophies may be informative. However, although corneal dystrophies can occasionally manifest at an older age without documented familial inheritance, this type of presentation is uncommon.2 Immunoglobulin corneal deposition is an important diagnostic consideration that should prompt systemic evaluation for paraproteinemia, obviating the need for diagnostic corneal biopsy.3,4
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Corresponding Author: Tatyana Milman, MD, Department of Ophthalmology, Wills Eye Hospital, Thomas Jefferson University Hospital, 840 Walnut St, Ste 1410, Philadelphia, PA 19107 (firstname.lastname@example.org).
Published Online: June 13, 2019. doi:10.1001/jamaophthalmol.2019.1646
Conflict of Interest Disclosures: None reported.
Additional Contributions: We thank the patient for granting permission to publish this information.
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