Symptoms become prominent in early adulthood and throughout middle age.
Symptoms range from minor early morning irritation to significant corneal epithelial erosion. The epithelium produces an abnormal multilaminar basement membrane to which there is poor adherence. Frequently there is elevation and reduplication of this basement membrane within the epithelium. The intra-epithelial basement membrane layer blocks normal migration of epithelium below leading to inclusion cysts. The end result is a poorly adherent epithelium further destabilized by intra-epithelial inclusions.