Abstract
Gelatinous drop-like corneal dystrophy is a very rare autosomal recessive disease
classified as an epithelial and subepithelial corneal dystrophy. Patients typically
present under the age of 20 with drop-like corneal lesions showing high corneal fluorescein
uptake. Their disease course is typically protracted and prone to frequent relapses.
The condition is caused by a dysfunction of the epithelial barrier, leading to protein
deposits most likely originating from tear fluid. Histology typically shows subepithelial
amyloid deposits with corresponding defects of Bowmanʼs layer and epithelial atrophy.
Where topical lubricating and anti-inflammatory therapy proves insufficient, penetrating
allogenic limbokeratoplasty can be considered in a curative approach. In this report,
we present disease courses of 2 unrelated patients. Current findings on pathogenesis
are discussed.
Key words
cornea - pathology - corneal dystrophy - gelatinous drop-like dystrophy - limbokeratoplasty
- perforating limbokeratoplasty