Epithelial Basement Membrane Dystrophy
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Disease Entity
Epithelial basement membrane dystrophy (also known as map-dot-fingerprint dystrophy, Cogan microcystic dystrophy, or anterior basement membrane dystrophy)
Disease
Epithelial basement membrane dystrophy (EBMD) is a disease that affects the anterior cornea, causing characteristic "map," "dot," and "fingerprint" changes (visible on slit lamp) that may result in decreased vision and/or recurrent corneal erosions. There is some debate as to whether EBMD is a true dystrophy (a disease which occurs more commonly within affected families than in the general population) or a corneal degeneration (a disease which more commonly occurs randomly than in affected families).
Etiology
Although most patients do not seem to be aware of a family history EBMD, autosomal dominant pedigrees have been described. Certain point mutations in the TGFBI gene on chromosome 5 may be responsible for some EBMD cases.
Risk Factors
Known family history and age are the most notable risk factors for EBMD; there are no known controllable risk factors. Risk factors for progression or exacerbation of the disease include trauma such as corneal abrasion, LASIK, or other intraocular surgery.
General Pathology
In EBMD, extra sheets of basement membrane extend abnormally into the corneal epithelium ("maps"). Maturing epithelial cells migrating towards the anterior surface of the epithelium become entrapped in these extra sheets and form cysts ("dots"). Parallel or concentric lines of thickened basement membrane present as "fingerprints." These abnormalities within the epithelium may cause blurred vision, and abnormalities of the basement membrane may interfere with adherence of the epithelial cells to the basement membrane and lead to painful recurrent corneal erosions.
Primary Prevention
There is no prevention for EBMD. However, if recurrent corneal erosions are present, they may be managed or prevented using nighttime lubricating or hypertonic saline ointments or with various surgical procedures (described below).
Diagnosis
Diagnosis is clinical and based on characteristic "map-dot-fingerprint" slit lamp findings.
History
Patients could be asked about mild fluctuations in vision, mild foreign body sensation, or a history of recurrent corneal erosion. Recurrent corneal erosions usually present as pain (sometimes severe) in one eye in the middle of the night or immediately upon opening the eyes in the morning, but there is great variability in the severity of the pain and the frequency of recurrence.
Physical Examination
The characteristic slit lamp findings for EBMD are best described by the name "map-dot-fingerprint." The "map" changes are most commonly seen and appear as amorphous or geographic sharply demarcated clear zones within light grayish areas. The "dots" appear as small, irregular, putty-like grayish-white opacities. The "fingerprints" consist of small clusters of curved, parallel lines. Other findings include areas of irregular circles of negative staining when the cornea is viewed through blue light after the instillation of topical fluorescein.
Symptoms
Many patients are asymptomatic, although symptoms may include blurry vision, variable vision, foreign body sensation, and pain associated with recurrent corneal erosions.
Clinical Diagnosis
The clinical diagnosis is based on history and physical exam. No diagnostic procedures are required and there are no laboratory tests currently available.
Differential diagnosis
The differential diagnosis may include other anterior corneal dystrophies, such as Meesman Juvenile Epithelial Dystrophy or Reis-Bucklers Dystrophy. The differential cause of recurrent corneal erosions includes corneal abrasion. Herpes simplex virus or herpes zoster virus keratitis might cause subepithelial/anterior stromal scarring or epithelial defects that could be confused with EBMD-associated scarring or erosions.
Management
The goal of EBMD management is to improve vision and reduce the rate of recurrence of corneal erosions. Sometimes, surgical management may be recommended in order to obtain more reliable keratometry readings before cataract surgery and improve refractive outcomes after cataract surgery.[1] First line treatment options usually involve the use of nighttime lubricating ointments or hypertonic saline ointments. Symptomatic erosions may be treated with bandage contact lenses, antibiotic ointments, or patching, and there is some evidence that topical steroids or oral doxycycline may provide some benefit for patients with frequently occurring erosions.
Various procedures can be of benefit in reducing the rate of recurrent erosions, including anterior stromal puncture (outside the visual axis), YAG laser micropuncture, cautery, epithelial debridement and/or diamond-burr polishing, phototherapeutic keratectomy, and extended bandage contact lens wear. More recently, some ophthalmologists have touted the use of autologous serum tears or amniotic membranes to help treat recurrent corneal erosions and prevent new ones from occurring. A 2022 study by Yeu et al found that the placement of amniotic membranes after corneal debridement for EBMD resulted in slightly quicker re-epithelialization; however, differences were not statistically significant, and no other benefits were noted.[2]
Complications
Scarring from recurrent corneal erosions can result in loss of best corrected visual acuity.
Prognosis
The prognosis is very variable. The disease tends to progress with age, although many patients can experience a waxing and waning of symptoms throughout their lives and some experience improvement over time.
References
Krachmer, Mannis, and Holland, CORNEA, 2nd ed, vol 1, Elsevier Mosby, 2005, pp 898-902.
