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Bilateral Corneal Deposits 1 Week After Starting Oral Prednisone Therapy

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

A man in his 30s presented with bilateral blurry vision a week after starting prednisone therapy for a presumed seronegative spondyloarthropathy. He had no other pertinent medical history and no relevant ocular history other than anisometropia, for which he wears a corrective contact lens in his right eye. At his initial ophthalmology visit, his best-corrected visual acuity was 20/40 OD and 20/20 OS. In both corneas, he had numerous multicolored, highly refractile crystals located primarily in the subepithelium and anterior stroma (Figure 1). No deposits were noted in the retina of either eye on funduscopic examination.

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Paraproteinemic keratopathy due to cryoglobulinemia

B. Superficial corneal biopsy

The patient presented with a crystalline keratopathy of the anterior stroma and epithelium. No stromal haze or cellular infiltrate consistent with an inflammatory response, which would potentially benefit from topical prednisolone therapy, was observed. Topical vancomycin is used for the management of infectious crystalline keratopathy, which presents with needlelike crystalline stromal deposits owing to the presence of bacterial colonies in the corneal stroma often after long-term use of topical corticosteroids.1 The deposits in our case are not consistent with those of an infectious crystalline keratopathy, and the patient had no sign of intraocular inflammation that might be seen in cases of infectious crystalline keratopathy. Biopsies can provide invaluable information in the management of a patient presenting with crystalline keratopathy, and moving right to a corneal transplant is not appropriate in a patient who has preserved visual acuity.

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Article Information

Corresponding Author: Diva R. Salomão, MD, Department of Ophthalmology, Mayo Clinic, 200 First St SW, Rochester, MN 55905 (salomao.diva@mayo.edu).

Published Online: March 1, 2018. doi:10.1001/jamaophthalmol.2017.5316

Conflict of Interest Disclosures: All authors have completed and submitted the ICMJE Form for Disclosure of Potential Conflicts of Interest and none were reported.

Funding/Support: This study was supported by Research to Prevent Blindness, New York, NY.

Role of the Funder/Sponsor: The sponsor had no role in the design and conduct of the study; collection, management, analysis, and interpretation of the data; preparation, review, or approval of the manuscript; and decision to submit the manuscript for publication.

Additional Contributions: Samih H. Nasr, MD, Department of Pathology, Mayo Clinic, contributed regarding the paraffin staining techniques used to identify the cryocrystals. He was not compensated for this contribution. We thank the patient for granting permission to publish this information.

References
1.
Sharma  N, Vajpayee  RB, Pushker  N, Vajpayee  M.  Infectious crystalline keratopathy.  CLAO J. 2000;26(1):40-43.PubMedGoogle Scholar
2.
Brouet  JC, Clauvel  JP, Danon  F, Klein  M, Seligmann  M.  Biologic and clinical significance of cryoglobulins: a report of 86 cases.  Am J Med. 1974;57(5):775-788.PubMedGoogle ScholarCrossref
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.PubMedGoogle ScholarCrossref
4.
Garibaldi  DC, Gottsch  J, de la Cruz  Z, Haas  M, Green  WR.  Immunotactoid keratopathy: a clinicopathologic case report and a review of reports of corneal involvement in systemic paraproteinemias.  Surv Ophthalmol. 2005;50(1):61-80.PubMedGoogle ScholarCrossref
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Oglesby  RB.  Corneal opacities in a patient with cryoglobulinemia and reticulohistiocytosis.  Arch Ophthalmol. 1961;65:63-66.PubMedGoogle ScholarCrossref
6.
Kremer  I, Wright  P, Merin  S, Weiss  J, Pick  AI, Kaufman  H.  Corneal subepithelial monoclonal kappa IgG deposits in essential cryoglobulinaemia.  Br J Ophthalmol. 1989;73(8):669-673.PubMedGoogle ScholarCrossref
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