Your browser does not support javascript:   Search for gard hereSearch for news-and-events here.

Diseases

Genetic and Rare Diseases Information Center (GARD)

Print friendly version

Epithelial basement membrane corneal dystrophy


Other Names for this Disease

  • Cogan corneal dystrophy
  • Corneal dystrophy, anterior basement membrane
  • Map-dot-fingerprint dystrophy of cornea
  • Microcystic dystrophy of the cornea
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.

Overview

What is epithelial basement membrane corneal dystrophy?

What are the signs and symptoms of epithelial basement membrane corneal dystrophy?

How might epithelial basement membrane corneal dystrophy be treated?

What is epithelial basement membrane corneal dystrophy?

Epithelial basement membrane corneal dystrophy is a condition where the epithelium of the cornea (the outermost region of the cornea) loses its normal clarity due to a buildup of cloudy material. It gets its name from the unusual appearance of the cornea during an eye exam. This dystrophy occurs when the epithelium's basement membrane develops abnormally, causing the epithelial cells to not properly adhere to it. This leads to recurrent epithelial erosions, which can cause blurred vision and severe pain.[1] This condition is usually not inherited. However, families with autosomal dominant inheritance and mutations in the TGFBI gene have been identified.[2]
Last updated: 7/8/2014

What are the signs and symptoms of epithelial basement membrane corneal dystrophy?

A chronic problem seen in this condition is the epithelial erosions. They can alter the cornea's normal curvature, causing periodic blurred vision. These erosions may also expose the nerve endings that line the tissue, resulting in moderate to severe pain lasting as long as several days. Generally, the pain will be worse upon awakening in the morning. Other symptoms include sensitivity to light, excessive tearing, and foreign body sensation in the eye. This condition usually affects adults between the ages of 40 and 70, although it can develop earlier in life.[1]

It gets its name from the unusual appearance of the cornea during an eye exam. Most often, the affected epithelium will have a map-like appearance, i.e., large, slightly gray outlines that look like a continent on a map. There may also be clusters of opaque dots close to the map-like patches. Less frequently, the irregular basement membrane will form concentric lines in the central cornea that resemble small fingerprints.[1]

Epithelial basement membrane corneal dystrophy is not a progressive condition. Typically, it will flare up occasionally for a few years and then go away on its own, with no lasting loss of vision. Most people never know that they have this condition, since they do not have any pain or vision loss.[1][3]
Last updated: 7/8/2014

How might epithelial basement membrane corneal dystrophy be treated?

Because most people do not develop noticeable signs or symptoms, treatment usually is not necessary. However, if treatment is needed, doctors will try to control the pain associated with the epithelial erosions. They may patch the eye to immobilize it, or prescribe lubricating eye drops and ointments. With treatment, these erosions usually heal within three days, although periodic flashes of pain may occur for several weeks thereafter. Other treatments include anterior corneal punctures to allow better adherence of cells; corneal scraping to remove eroded areas of the cornea and allow regeneration of healthy epithelial tissue; and use of the excimer laser to remove surface irregularities.[1]

An article from eMedicine Journal provides additional information on treatment for epithelial basement membrane corneal dystrophy at the following link. You may need to register to view the article, but registration is free.
http://emedicine.medscape.com/article/1193945-treatment#showall
Last updated: 6/30/2011

References
  1. Facts About the Cornea and Corneal Disease. National Eye Institute (NEI). May 2013; http://www.nei.nih.gov/health/cornealdisease/#j. Accessed 7/8/2014.
  2. Corneal dystrophy, epithelial basement membrane; EBMD. Online Mendelian Inheritance of Man (OMIM). June 19, 2009; http://omim.org/entry/121820. Accessed 7/8/2014.
  3. Verdier D & Roy H. Dystrophy, Map-dot-fingerprint. Medscape Reference. May 21, 2014; http://emedicine.medscape.com/article/1193945-overview. Accessed 7/8/2014.


Other Names for this Disease
  • Cogan corneal dystrophy
  • Corneal dystrophy, anterior basement membrane
  • Map-dot-fingerprint dystrophy of cornea
  • Microcystic dystrophy of the cornea
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.