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Diagnosing Paraproteinemic Keratopathy: A Case Report

Authors
  • Mok, Eugenie
  • Kam, Ka Wai
  • Aldave, Anthony J.
  • Young, Alvin L.
Type
Published Article
Journal
Case Reports in Ophthalmology
Publisher
S. Karger AG
Publication Date
May 07, 2021
Volume
12
Issue
2
Pages
337–343
Identifiers
DOI: 10.1159/000514375
PMID: 34054481
PMCID: PMC8136328
Source
PubMed Central
Keywords
Disciplines
  • Case Report
License
Unknown

Abstract

A 65-year-old man presented with bilateral, painless, progressive blurring of vision over 9 years. Slit-lamp examination revealed bilateral subepithelial corneal opacities in clusters located at the mid-periphery. Anterior segment optical coherence tomography, in vivo confocal microscopy (IVCM), serum protein electrophoresis, and molecular genetic testing were performed to evaluate the cause of corneal opacities. Anterior segment optical coherence tomography revealed a band-like, hyperreflective lesion in the Bowman layer and anterior stroma of both corneas. IVCM revealed hyperreflective deposits in the epithelium, anterior stroma, and endothelium. Serum protein electrophoresis identified the presence of paraproteins (immunoglobulin kappa), and molecular genetic testing revealed absence of mutations in the transforming growth factor beta-induced gene ( TGFBI ) and collagen type XVII alpha 1 gene ( COL17A1 ). The ocular diagnosis of paraproteinemic keratopathy eventually led to a systemic diagnosis of monoclonal gammopathy of undetermined significance by our hematologist/oncologist. Paraproteinemic keratopathy is a rare differential diagnosis in patients with bilateral corneal opacities and therefore may be misdiagnosed as corneal dystrophy or neglected as scars. In patients with bilateral corneal opacities of unknown cause, serological examination, adjunct anterior segment imaging, and molecular genetic testing play a role in establishing the diagnosis.

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