Corneal epithelial lesions in keratosis follicularis (Darier's disease)

The prevalence of eyelid keratotic plaques and unique corneal changes associated with Darier'sdisease, peripheral epithelial nebular opacities associated with irregular surface of the central corneal epithelium, are described. These corneal lesions occurred in 16 of 21 patients. The corneal lesions were asymptomatic, stable, and did not respond to oral retinoid therapy. Histopathology of the peripheral corneal opacities showed epithelial cell edema especially in the basal layer, and decreased desmosomes.