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Corneal Opacification

Educational Objective
Based on this clinical scenario and the accompanying image, understand how to arrive at a correct diagnosis.
1 Credit CME

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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Article Information

Corresponding Author: Tatyana Milman, MD, Department of Ophthalmology, Wills Eye Hospital, Thomas Jefferson University Hospital, 840 Walnut St, Ste 1410, Philadelphia, PA 19107 (tatyana.milman@gmail.com).

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.

References
1.
Ramos-Casals  M, Tzioufas  AG, Stone  JH, Sisó  A, Bosch  X.  Treatment of primary Sjögren syndrome: a systematic review.  JAMA. 2010;304(4):452-460. doi:10.1001/jama.2010.1014PubMedGoogle ScholarCrossref
2.
Cho  KJ, Mok  JW, Na  KS,  et al.  TGFBI gene mutations in a Korean population with corneal dystrophy.  Mol Vis. 2012;18:2012-2021.PubMedGoogle Scholar
3.
Milman  T, Kao  AA, Chu  D,  et al.  Paraproteinemic keratopathy: the expanding diversity of clinical and pathologic manifestations.  Ophthalmology. 2015;122(9):1748-1756. doi:10.1016/j.ophtha.2015.05.029PubMedGoogle ScholarCrossref
4.
Lisch  W, Saikia  P, Pitz  S,  et al.  Chameleon-like appearance of immunotactoid keratopathy.  Cornea. 2012;31(1):55-58. doi:10.1097/ICO.0b013e31821ddd0cPubMedGoogle ScholarCrossref
5.
Steger  B, Romano  V, Biddolph  S, Willoughby  CE, Batterbury  M, Kaye  SB.  Femtosecond laser-assisted lamellar keratectomy for corneal opacities secondary to anterior corneal dystrophies: an interventional case series.  Cornea. 2016;35(1):6-13. doi:10.1097/ICO.0000000000000665PubMedGoogle ScholarCrossref
6.
Chiang  HH, Wieland  RS, Rogers  TS, Gibson  PC, Atweh  G, McCormick  G.  Paraproteinemic keratopathy in monoclonal gammopathy of undetermined significance treated with primary keratoprosthesis: case report, histopathologic findings, and world literature review.  Medicine (Baltimore). 2017;96(50):e8649. doi:10.1097/MD.0000000000008649PubMedGoogle ScholarCrossref
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