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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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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 (firstname.lastname@example.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.
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