Peripheral Hypertrophic Subepithelial Corneal Degeneration Presenting With Bilateral Inferior Corneal Changes: An Unusual Case In An Ophthalmologist

Peripheral hypertrophic subepithelial corneal degeneration (PHSCD) is a rare, idiopathic, and often underdiagnosed disorder characterized by chronic fibrosis between the epithelium and Bowman’s layer affecting the perilimbal region. Herein, we report a case of PHSCD in an ophthalmologist with a 12-year asymptomatic course. 

This patient was evaluated at the Department of Ophthalmology, University of Health Sciences, Dışkapı Yıldırım Beyazıt Training and Research Hospital, Ankara, Turkiye.

A 31-year-old female ophthalmologist presented with bilateral inferior whitish corneal opacities that she had been aware of since medical school. Her medical history was unremarkable. Visual acuity was 20/20 in both eyes, with no history of contact lens use. Twelve years earlier, she had simple myopia (−2.50 D OD, −3.50 D OS); despite a stable spherical equivalent, regular against-the-rule astigmatism developed in both eyes over time. Slit-lamp examination revealed bilateral peripheral, subepithelial, hypertrophic corneal opacification greater in the left eye with adjacent superficial vascularization. These opacities were separated from the limbus by a clear zone and exhibited pseudopod-like extensions toward the paracentral cornea. Optical coherence tomography indicated a hyperreflective thickening between the epithelium and Bowman’s membrane, with increasing epithelial reflectivity toward the peripheral cornea. Pachymetric, stromal, and epithelial thickness maps identified inferior thickening. Corneal topography demonstrated astigmatic changes accompanied by inferior corneal thickening. In vivo confocal microscopy revealed sub-basal hyperreflectivity with gap like hiporeflective areas. Given the absence of ocular surface symptoms and visual impairment, the patient has been managed conservatively with observation.

PHSCD is an underreported condition with a female predominance that may be misdiagnosed as Salzmann’s nodular degeneration, Terrien’s marginal degeneration, or corneal intraepithelial neoplasia. Mild cases can be followed with conservative management, including lubrication; however, the development of persistent ocular surface discomfort, irregular astigmatism, or reduced visual acuity may necessitate surgical intervention, most commonly superficial keratectomy. Recognition of this rare condition is achievable through multimodal imaging. Further research into its pathogenesis may contribute to the advancement of disease-specific therapies.