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Bilateral Asymmetrical Corneal Deposits in an Asymptomatic Patient

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

A 70-year-old White woman with a history of hypertension and hyperlipidemia was referred to our institution for evaluation of bilateral asymmetric corneal lesions by her primary eye care professional. The patient required spectacles for best-corrected visual acuity but otherwise had no other significant ocular complaints. She was not taking medications known to cause corneal depositions, nor was there a known family history of corneal dystrophy. Best-corrected visual acuity was 20/20 OU. Slitlamp examination of the cornea of the right eye revealed multiple, discrete nodular opacities in the superior and inferior quadrants without neovascularization or surface elevation. Similar findings were seen in the left eye, but the superior lesions were notable for possessing well-circumscribed, plaquelike features located in the posterior stroma without involvement of the anterior surface (Figure, A). No crystalline changes were noted with these deposits.

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Monoclonal gammopathy of undetermined significance

B. Additional laboratory workup

When encountering asymmetrical, bilateral anterior and posterior stromal corneal lesions, clinicians should consider systemic causes (infectious, autoimmune, and hematologic) and pursue laboratory workup. Salzmann nodular degeneration was deemed less likely, given the variable lesion depth and lack of surface elevation. The following diagnostic workup was pursued: complete blood cell count, comprehensive metabolic panel, serum and urine protein electrophoresis, HIV, cytomegalovirus, Epstein-Barr virus, herpes simplex virus/herpes zoster virus, Lyme antibody titers (IgG/IgM), rapid plasma reagin, fluorescent treponemal antibody absorption, QuantiFERON Gold, angiotensin-converting enzyme, lysozyme, antinuclear antibody, rheumatoid factor, cyclic citrullinated peptide, antineutrophil cytoplasmic antibodies, and anti-SSA (Ro)/anti-SSB (La) antibodies. Results revealed an elevated γ globulin M spike (0.8 g/dL), cytomegalovirus IgG, and herpes simplex virus IgG. Notably, herpes simplex virus IgM, Epstein-Barr virus, and other autoimmune laboratory values were negative. The abnormal serum proteins prompted referral to a hematologist. Monoclonal gammopathies encompass a spectrum of diseases, including multiple myeloma and monoclonal gammopathy of undetermined significance (MGUS). While multiple myeloma is one of the most common hematologic malignancies, a forme fruste, premalignant stage-termed MGUS may precede it.1,2 Estimated to have a prevalence of 3% to 4% in individuals older than 50 years, MGUS progresses to multiple myeloma at a rate of 0.5% to 1% per year.2 Traditionally, it has been considered an asymptomatic disease stage and requires the absence of hypercalcemia, kidney failure, anemia, and bone lesions.2,3 Although visual symptoms are minimal in MGUS, ocular findings may be the first sign of systemic disease, providing ophthalmologists with an important role in diagnosing underlying disease.

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Article Information

Corresponding Author: Kamran M. Riaz, MD, Department of Ophthalmology, Dean McGee Eye Institute/University of Oklahoma, 608 Stanton L. Young Blvd, Oklahoma City, OK 73104 (kamran-riaz@dmei.org).

Published Online: March 11, 2021. doi:10.1001/jamaophthalmol.2020.4656

Conflict of Interest Disclosures: None reported.

Additional Contributions: We thank the patient for granting permission to publish this information. We also would like to thank Donald Stone, MD (Spokane Eye Clinic, Spokane, WA), for academic and clinical contributions to this case. Dr Stone did not receive compensation.

References
1.
Lisch  W , Wasielica-Poslednik  J , Kivelä  T ,  et al.  The hematologic definition of monoclonal gammopathy of undetermined significance in relation to paraproteinemic keratopathy (an American Ophthalmological Society thesis).   Trans Am Ophthalmol Soc. 2016;114:T7.Google Scholar
2.
Rajkumar  SV , Dimopoulos  MA , Palumbo  A ,  et al.  International Myeloma Working Group updated criteria for the diagnosis of multiple myeloma.   Lancet Oncol. 2014;15(12):e538-e548.PubMedGoogle ScholarCrossref
3.
Karakus  S , Gottsch  JD , Caturegli  P , Eghrari  AO .  Monoclonal gammopathy of “ocular” significance.   Am J Ophthalmol Case Rep. 2019;15:100471.PubMedGoogle Scholar
4.
Koo  H , Oh  DH , Chun  YS , Kim  JC .  A case of crystalline keratopathy in monoclonal gammopathy of undetermined significance (MGUS).   Korean J Ophthalmol. 2011;25(3):202-205.PubMedGoogle ScholarCrossref
5.
Klintworth  GK , Bredehoeft  SJ , Reed  JW .  Analysis of corneal crystalline deposits in multiple myeloma.   Am J Ophthalmol. 1978;86(3):303-313.PubMedGoogle ScholarCrossref
6.
Bourne  WM , Kyle  RA , Brubaker  RF , Greipp  PR .  Incidence of corneal crystals in the monoclonal gammopathies.   Am J Ophthalmol. 1989;107(2):192-193.PubMedGoogle ScholarCrossref
7.
Aragona  P , Allegra  A , Postorino  EI ,  et al.  Corneal structural changes in nonneoplastic and neoplastic monoclonal gammopathies.   Invest Ophthalmol Vis Sci. 2016;57(6):2657-2665.PubMedGoogle ScholarCrossref
8.
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
9.
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
10.
Stiefel  HC , Sandhu  RK , Miller  AK , Wilson  DJ , Chamberlain  WD .  Characterization of corneal deposition keratopathy in the setting of blood cell dyscrasia and a minimally invasive technique to clear the cornea in a single case.   Am J Ophthalmol Case Rep. 2018;13:83-88.PubMedGoogle ScholarCrossref
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