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Diseases

Genetic and Rare Diseases Information Center (GARD)

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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
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Symptoms

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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][2]
Last updated: 7/8/2014

The Human Phenotype Ontology provides the following list of signs and symptoms for Epithelial basement membrane corneal dystrophy. If the information is available, the table below includes how often the symptom is seen in people with this condition. You can use the MedlinePlus Medical Dictionary to look up the definitions for these medical terms.

Signs and Symptoms Approximate number of patients (when available)
Autosomal dominant inheritance -
Map-dot-fingerprint corneal dystrophy -
Recurrent corneal erosions -

Last updated: 12/1/2014

The Human Phenotype Ontology (HPO) has collected information on how often a sign or symptom occurs in a condition. Much of this information comes from Orphanet, a European rare disease database. The frequency of a sign or symptom is usually listed as a rough estimate of the percentage of patients who have that feature.

The frequency may also be listed as a fraction. The first number of the fraction is how many people had the symptom, and the second number is the total number of people who were examined in one study. For example, a frequency of 25/25 means that in a study of 25 people all patients were found to have that symptom. Because these frequencies are based on a specific study, the fractions may be different if another group of patients are examined.

Sometimes, no information on frequency is available. In these cases, the sign or symptom may be rare or common.


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. Verdier D & Roy H. Dystrophy, Map-dot-fingerprint. Medscape Reference. May 21, 2014; http://emedicine.medscape.com/article/1193945-overview. Accessed 7/8/2014.
  3. Corneal dystrophy, epithelial basement membrane; EBMD. Online Mendelian Inheritance of Man (OMIM). June 19, 2009; http://omim.org/entry/121820. 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.