In Vivo Confocal Microscopy of Polymorphic Amyloid Degeneration and Posterior Crocodile Shagreen

Abstract

To report the in vivo confocal microscopic findings of combined polymorphic amyloid degeneration and posterior crocodile shagreen and correlate these findings with clinical presentations and previous histopathologic analysis. Case reports with confocal microscopic analysis and literature review. Three patients with bilateral corneal opacities presented to the Emory Eye Center Cornea Clinic for evaluation. The first patient, a 65-year-old woman, was noted to have bilateral, discrete, punctate, and filamentous refractile stromal opacifications that appeared to be concentrated in the deep stroma. The second patient, a 50-year-old man, had bilateral deep central stromal haze in a mosaic pattern with intervening cracklike clear zones. The third patient, an 80-year-old woman, had bilateral deep stromal deposits similar to patient 1 in addition to bilateral deep central stromal haze in a mosaic pattern with intervening cracklike clear zones similar to patient 2. Confocal microscopy showed bright, enhancing punctate lesions in the deep stroma and anterior stroma, with unaffected areas between these regions in patients 1 and 3, and central acellular opacification of irregular intensity with intermittent linear clear zones within the normal posterior stromal bed in cases 2 and 3. The first patient was diagnosed with isolated polymorphic amyloid degeneration, the second patient was diagnosed with isolated posterior crocodile shagreen, and the third patient was diagnosed with combined polymorphic amyloid degeneration and posterior crocodile shagreen. These cases are, to our knowledge, the first to report confocal microscopic findings in isolated polymorphic amyloid degeneration and combined polymorphic amyloid degeneration with posterior crocodile shagreen.