Peripheral hypertrophic subepithelial corneal degeneration presenting with bilateral nasal and temporal corneal changes

Abstract

Purpose To characterise the history, clinical and histopathological features of patients with bilateral nasal and temporal peripheral hypertrophic subepithelial corneal degeneration in a German population. Methods A detailed ophthalmological and dermatological history and clinical findings were recorded of nine patients with bilateral simultaneous nasal and temporal peripheral corneal degeneration from two centers in Germany. Excised tissues were studied by histopathology, immunohistochemistry, and transmission electron microscopy. Results Foreign body sensation and need of artificial tear substitutes were the only symptoms reported regularly. Schirmer's and Jones-test were normal in all, but fluorescein break-up time of 410 s was found in five eyes of four patients. Best corrected visual acuity was reduced only under glare conditions. Corneal topography revealed irregular astigmatism in 13 of 14 eyes. Follow-up median time was 35 months. Most cases were stable within the follow-up period. Light and electron microscopy revealed the findings of superficial vascularised corneal hypertrophic scars, oxytatlan fibers, and discontinued Bowmans layer. Conclusion In this series of German patients with peripheral hypertrophic subepithelial corneal degeneration, the changes were predominantly located in the palpebral aperture and often present in both eyes. No associated surface disease could be established in this study. Light and transmission electron microscopy showed histological features that are similar to Salzmann's corneal changes without any inflammation. We hypothesise that light exposure and a localised limbal insufficiency could be involved in the pathogenesis.

Re: Peripheral hypertrophic subepithelial corneal degeneration – clinical and histopathological features

“…This is in line with the refined PHSD criteria of Ja¨rventausta et al (2014) and supports the concept of a self-contained entity. Similar findings have also been reported by Schargus et al (2015). We recently discussed that Salzmann's original paper comprised several patients who would meet the PHSD criteria (Salzmann 1925;Sundmacher 2012;Gore et al 2013;Eberwein et al 2014).…”

Corneal Dystrophies and Degenerations

“…It tends to occur in white women with unknown etiology and, however, resembles Salzmann's nodular degeneration in appearance (Figure 16A and B) [62,63]. Ultraviolet radiation and limbal insufficiency have been proposed in the pathogenesis [64].…”

Peripheral Hypertrophic Subepithelial Corneal Degeneration

“…Peripheral hypertrophic subepithelial corneal degeneration is a typically bilateral, slowly progressive perilimbal disorder that, to our knowledge, has rarely been described in the literature and needs to be distinguished from Salzmann nodular degeneration . The lack of histologic inflammation is pathognomonic, yet the cause of peripheral hypertrophic subepithelial corneal degeneration is still unknown …”

“…1,2 The lack of histologic inflammation is pathognomonic, yet the cause of peripheral hypertrophic subepithelial corneal degeneration is still unknown. 3,4…”