Cloudy corneas as an initial presentation of multiple myeloma

Abstract

SummaryWe report a case of previously unsuspected myeloma, presenting with cornea verticillata due to intracorneal paraprotein deposition.HistoryAn 85-year-old female presented via her optician with a 4-month history of cloudy vision. She had undergone an uneventful bilateral phacoemulsification surgery 7 years earlier. Extensive spiraling corneal epithelial opacification was noted on slit-lamp examination. On further investigation, she was found to have a previously unsuspected low-grade multiple myeloma. We established the nature of the corneal deposits with corneal epithelial biopsy histopathology and electron microscopy. It is very rare for multiple myeloma to present in this fashion. Ophthalmologists should be aware that such a presentation may rarely be due to systemic multiple myeloma.

Drug-induced corneal epithelial changes

“…59 In the vortex keratopathy characteristic of Fabry disease, the underlying cause is an abnormality in an intralysosomal hydrolase. 11 Systemically, the lysosomal accumulation is first characterized by pain and hypohidrosis, but in adulthood, patients can have severe renal, cardiac, and Multiple myeloma Spiraling intraepithelial corneal opacities 68 and microcysts in a vortex pattern 10 were reported in 2 cases; deposits are usually not in a vortex pattern 68 Intracellular accumulation of immunoglobulin theorized as due to elevated immunoglobulin levels in the tears and aqueous humor; deposits entering from the limbal blood vessels; or synthesis of the precipitated immunoglobulin by keratocytes 68 Worsening photophobia and visual acuity, 10 cloudy vision 68 Subconjunctival hemorrhages, peripheral ulcerative keratitis, prominent corneal nerves, band keratopathy 68…”

Whorl pattern keratopathies in veterinary and human patients

“…We note, however, that the term vortex keratopathy is occasionally employed interchangeably with hurricane keratopathy (ie, employed in cases wherein the whorl pattern is made more evident after the application of fluorescein). Cornea verticillata is a descriptive term and can have numerous underlying causes including drugs, heritable metabolic disorders (eg, Fabry disease), and dysproteinemias such as multiple myeloma . It is characterized by corneal deposits in the epithelium and Bowman's layer forming a fine opaque golden‐brown whorl pattern .…”

“…As simply descriptors of spatial patterns, these terms are used more promiscuously and often used interchangeably. Broadly, these terms are most often used to describe whorl patterns of the corneal surface clinically observable by slit lamp examination that are not accentuated by application of topical fluorescein and are most often made visible due to deposits within the epithelium and/or superficial stroma . We note, however, that the term vortex keratopathy is occasionally employed interchangeably with hurricane keratopathy (ie, employed in cases wherein the whorl pattern is made more evident after the application of fluorescein).…”

Corneal Structural Changes in Nonneoplastic and Neoplastic Monoclonal Gammopathies

“…Monoclonal gammopathies may involve the eye because of direct localization of the disease 3 or deposition of light chains in the ocular tissues, 6 or as a consequence of blood hyperviscosity. 4,5 Among the ocular complications, corneal involvement is less well recognized than others.…”

“…1,2 The eye can be involved in monoclonal gammopathies as a consequence of the ophthalmic localization, 3 blood hyperviscosity, 4,5 or deposition of light chains in the ocular tissues. 6 Among the ocular localizations, corneal involvement is the less well recognized than others. 7 In fact, in gammopathies not accompanied by general symptoms and signs, the diagnosis of primary corneal diseases, such as lattice dystrophy, 8,9 Groenouw type I granular dystrophy, 10 or deep filiform dystrophy, 11 or the diagnosis of systemic diseases such as cystinosis, 12,13 was established.…”