Purpose: To report the clinical features, laboratory investigation, and histological findings of an individual with a 14-year history of an idiopathic multifocal vortex epithelial keratopathy.
Methods: We describe the clinical appearance, the clinical course, and the response to treatment. We report the results of corneal epithelial culture, laboratory investigation for potential pathogens, and histopathology.
Results: The signs were consistent with focal areas of corneal inflammation that produced abnormal epithelial cells that were carried in vortex patterns by the direction of epithelial flow. In vivo confocal microscopy demonstrated hyperreflective structures within the epithelial cell layer, but with only minor signs of stromal disease, similar to Thygeson superficial punctate keratitis. Culture, polymerase chain reaction, and serology did not support an infectious etiology, and histopathology showed nonspecific inflammation of the epithelial layer. Symptoms of photophobia and blur, with multiple exacerbations, required the almost continuous use of topical corticosteroid or ciclosporin over 14 years.
Conclusions: The clinical course, as well as the response to topical corticosteroid and ciclosporin, suggests that this is distinct from Thygeson superficial punctate keratitis. The etiology is unknown.