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Corneal Dystrophy, Recurrent Epithelial Erosions

OMIM ID:

autosomal dominant

Corneal Dystrophy, Recurrent Epithelial Erosions

Alternate Names

ERED
recurring corneal erosions
hereditary corneal erosions
epithelial recurrent erosion dystrophy
Franceschetti hereditary recurrent corneal erosion

Defective Genes

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Clinical Characteristics

Ocular Features

Individuals have the onset of recurrent corneal erosions as a result of as yet unknown disease processes.  Onset is in the first decade of life (even in the first year of life)  often with some subepithelial haze or blebs while denser centrally located opacities develop with time.  Erosions often are precipitated by relatively minor trauma and are often difficult to treat, lasting for up to a week.  Fortunately, the erosions become less frequent as patients age and may cease altogether by the fifth decade of life.

Systemic Features

No systemic disease is associated with ERED.

Genetics

Inheritance

The few reported families have all had an autosomal dominant pattern of inheritance.  So far no locus or molecular defect has been identified.

The clinical features of this condition are found in other corneal dystrophies and it is likely that at least some of the reported cases may have had other unrecognized corneal conditions.

Pedigree

Autosomal dominant

Autosomal dominant disorders require only one mutation for the disease to be expressed.  Since an affected parent has two chromosomes, only one of which has the mutant gene, parents can expect that half (50%) of their children will receive that one and inherit the disease.  It is common for individuals that inherit the mutation, however, to not have evidence of the disease (nonpenetrance).

Image
Sample pedigree of autosomal dominant inheritance

Autosomal dominant inheritance leads to a vertical pattern of transmission

Treatment & Management

The usual corneal erosion treatment of hypertonic solutions, bandage patching, and lubricating ointments may be helpful for acute erosions.  No long term preventative treatment has been found effective.  Corneal transplants remain clear centrally although peripheral opacities may reappear within a few years.

Publications

Displaying 1 - 4 of 4

A family history of corneal erosions

PubMedID: 13380984

A new corneal disease with recurrent erosive episodes and autosomal‐dominant inheritance

PubMedID: 18778339

Franceschetti Hereditary Recurrent Corneal Erosion

PubMedID: 22402249

HEREDITARY RECURRING CORNEAL EROSIONS A FAMILIAL STUDY WITH SPECIAL REFERENCE TO FUCHS‘ DYSTROPHY

PubMedID: 5300718