eMedicine Specialties > Ophthalmology > Cornea

Dystrophy, Map-dot-fingerprint

Author: David Verdier, MD, Clinical Professor, Department of Surgery, Division of Ophthalmology, Michigan State University College of Human Medicine
Contributor Information and Disclosures

Updated: May 26, 2009

Introduction

Background

Corneal map-dot-fingerprint dystrophy is by far the most common corneal dystrophy and is named from the appearance of its characteristic slit lamp findings. Map-dot-fingerprint dystrophy is also known as Cogan’s dystrophy, Cogan microcystic epithelial dystrophy, epithelial basement membrane dystrophy, and anterior basement membrane dystrophy. 

Historically, corneal dystrophies are usually described as hereditary, bilateral, progressive, and not associated with systemic or local disease. However, in most cases, map-dot-fingerprint dystrophy is not familial. Map-dot-fingerprint dystrophy is also not progressive but rather variable and fluctuating in its course. In addition, map-dot-fingerprint dystrophy is usually bilateral, but it can be unilateral or very asymmetric in presentation.

A new classification of corneal dystrophy has been proposed. According to the International Committee for Classification of Corneal Diseases (IC3D), corneal dystrophies are still classified by the anatomic layer of corneal involvement, but they are increasingly defined on a genetic basis.  Map-dot-fingerprint dystrophy is placed in Category 4, which is "reserved for suspected new or previously documented corneal dystrophy, while the evidence for it being a distinct entity is not yet convincing." Except for the few cases of map-dot-fingerprint dystrophy described in several families with a presumed autosomal dominant pattern, possibly coded on the TGFBI/BIGH3 gene, map-dot-fingerprint dystrophy might be more accurately categorized as a corneal degeneration.

Pathophysiology

The corneal epithelium produces and adheres to its underlying basement membrane. Corneal abnormalities associated with map-dot-fingerprint dystrophy are the result of a faulty basement membrane, which is thickened, multilaminar, and misdirected into the epithelium. Deeper epithelial cells that normally migrate to the surface can become trapped. Epithelial cells anterior to aberrant basement membrane may have difficulty forming viable hemidesmosomes and basement membrane complexes, which attach to the underlying stroma, resulting in recurrent erosions. Irregular epithelium centrally can cause decreased vision.

Frequency

United States

Estimates of the prevalence of map-dot-fingerprint dystrophy range from 2-43% of the general population. Of patients with map-dot-fingerprint dystrophy, 10-33% have recurrent corneal erosions. As many as 50% of patients with recurrent corneal erosions have map-dot-fingerprint dystrophy.

Mortality/Morbidity

Patients with map-dot-fingerprint dystrophy may be asymptomatic. Others experience painful recurrent erosions, decreased vision, or both.

Sex

This condition is slightly more common in females than in males.

Age

This condition is uncommon in children.

Clinical

History

  • Most patients are asymptomatic.
  • The past eye history may be positive for recurrent corneal erosions.
  • Visual symptoms are usually mild and occasionally debilitating. Vision is variable and fluctuating due to migratory and intermittent corneal involvement. Refractions often are unstable and are not the fault of the doctor or the patient. Visual complaints include the following:
    • Blurred vision
    • Ghosting or monocular diplopia
    • Glare
    • Distortion
  • Pain symptoms
    • Foreign body sensation
    • Photophobia

Physical

  • Visual acuity ranges from 20/15 to 20/200.
  • Refraction may have an uncertain endpoint due to irregular astigmatism.
  • On slit lamp examination, pathology is at the epithelial and basement membrane levels. Areas of pathology often are identified best by broad-beam illumination, fluorescein with cobalt blue light (to identify areas of negative staining), or retroillumination following dilation. Slit lamp findings include the following:
    • The corneal maps in map-dot-fingerprint dystrophy are irregular geographic shaped, faint gray-white patches that may contain clear oval areas. They vary greatly in size (usually 1 mm to several mm) and are seen best with broad oblique illumination.
    • The corneal dots in map-dot-fingerprint dystrophy are gray-white, puttylike opacities, which can be round, comma-shaped, or irregular. They are usually 0.05-1 mm in size.
    • The corneal fingerprints in map-dot-fingerprint dystrophy are clusters of contoured concentric lines, 0.25-4 mm in length. They are seen best with retroillumination.
    • Corneal blebs are clear, round, bubblelike defects, 0.05-0.2 mm in diameter. They are seen best with retroillumination. 
  • Keratometry or computerized topography can be used to check for irregular astigmatism. A Placido disk or keratometer often demonstrates irregularity better than computerized topography.

Causes

More on Dystrophy, Map-dot-fingerprint

Overview: Dystrophy, Map-dot-fingerprint
Differential Diagnoses & Workup: Dystrophy, Map-dot-fingerprint
Treatment & Medication: Dystrophy, Map-dot-fingerprint
Follow-up: Dystrophy, Map-dot-fingerprint
Multimedia: Dystrophy, Map-dot-fingerprint
References
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References

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Further Reading

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Keywords

map-dot-fingerprint dystrophy, corneal map-dot-fingerprint dystrophy, Cogan’s dystrophy, Cogan's microcystic dystrophy, anterior basement membrane dystrophy, epithelial basement membrane dystrophy, corneal dystrophy, corneal dystrophies, corneal degeneration, corneal erosion

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Contributor Information and Disclosures

Author

David Verdier, MD, Clinical Professor, Department of Surgery, Division of Ophthalmology, Michigan State University College of Human Medicine
David Verdier, MD is a member of the following medical societies: American Academy of Ophthalmology, American Medical Association, Cornea Society, Eye Bank Association of America, Michigan Society of Eye Physicians & Surgeons, and Michigan State Medical Society
Disclosure: Nothing to disclose.

Medical Editor

Fernando H Murillo-Lopez, MD, Senior Surgeon, Unidad Privada de Oftalmologia CEMES
Fernando H Murillo-Lopez, MD is a member of the following medical societies: American Academy of Ophthalmology
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: Nothing to disclose.

Managing Editor

Christopher J Rapuano, MD, Professor, Department of Ophthalmology, Jefferson Medical College of Thomas Jefferson University; Co-Chairman of the Cornea Service, Co-Chairman of Refractive Surgery Department, Wills Eye Institute
Christopher J Rapuano, MD is a member of the following medical societies: American Academy of Ophthalmology, American Society of Cataract and Refractive Surgery, Contact Lens Association of Ophthalmologists, Cornea Society, Eye Bank Association of America, International Society of Refractive Surgery, and Pan-American Association of Ophthalmology
Disclosure: Allergan Honoraria Speaking and teaching; Allergan Consulting fee Consulting; Alcon Honoraria Speaking and teaching; Inspire Honoraria Speaking and teaching; RPS Ownership interest Other; Vistakon Honoraria Speaking and teaching

CME Editor

Lance L Brown, OD, MD, Ophthalmologist, Affiliated With Freeman Hospital and St John's Hospital, Regional Eye Center, Joplin, Missouri
Disclosure: Nothing to disclose.

Chief Editor

Hampton Roy Sr, MD, Associate Clinical Professor, Department of Ophthalmology, University of Arkansas for Medical Sciences
Hampton Roy Sr, MD is a member of the following medical societies: American Academy of Ophthalmology, American College of Surgeons, and Pan-American Association of Ophthalmology
Disclosure: Nothing to disclose.

 
 
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