Planning Ahead for Corneal Epithelial Dystrophy: A Teaching Case Report

Epithelial basement membrane dystrophy (EBMD) is the most common corneal dystrophy seen in clinical practice. Its appearance varies, which leads to frequent misdiagnosis, but presentation most often includes dot-like epithelial opacities, whorl-like fingerprint lines and circumscribed gray map-like patterns. It is for this reason that EBMD is also referred to as a map-dot-fingerprint dystrophy. A consistent feature in all presentations is the formation of microcysts in the corneal epithelium with alterations in the basement membrane. Histology shows thickening of the basement membrane with fibrillary protein deposited between the basement membrane and Bowman’s layer. The absence of hemidesmosomes within the basal epithelial cells is responsible for faulty epithelial adhesion to the underlying basement membrane, which results in recurrent corneal erosion (RCE). Management of EBMD focuses on maintaining patient comfort and treating situational RCE. Typical initial onset of EBMD is in the second decade of life. Approximately 10% of patients develop RCE in the third decade, while the remainder do not develop symptoms associated with RCE.