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.[1, 2, 3, 4, 5]
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.[6] 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.[7]
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."[8] 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,[9, 10] 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.
Epidemiology
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.[11]
Mortality/Morbidity
Patients with map-dot-fingerprint dystrophy may be asymptomatic. Others experience painful recurrent erosions, decreased vision, or both.[12]
Sex
This condition is slightly more common in females than in males.
Age
This condition is uncommon in children.
Cogan DG, Donaldson DD, Kuwabara T, et al. Microcystic dystrophy of the corneal epithelium. Trans Am Ophthalmol Soc. 1964;62:213-25. [Medline].
Cogan DG, Kuwabara T, Donaldson DD, et al. Microcystic dystrophy of the cornea. A partial explanation for its pathogenesis. Arch Ophthalmol. Dec 1974;92(6):470-4. [Medline].
Guerry D 3rd. Observations on Cogan's microcystic dystrophy of the corneal epithelium. Trans Am Ophthalmol Soc. 1965;63:320-34. [Medline].
Waring GO 3rd, Rodrigues MM, Laibson PR. Corneal dystrophies. I. Dystrophies of the epithelium, Bowman's layer and stroma. Surv Ophthalmol. Sep-Oct 1978;23(2):71-122. [Medline].
Trobe JD, Laibson PR. Dystrophic changes in the anterior cornea. Arch Ophthalmol. Apr 1972;87(4):378-82. [Medline].
Laibson PR, Krachmer JH. Familial occurrence of dot (microcystic), map, fingerprint dystrophy of the cornea. Invest Ophthalmol. May 1975;14(5):397-9. [Medline].
Rodrigues MM, Fine BS, Laibson PR, et al. Disorders of the corneal epithelium. A clinicopathologic study of dot, geographic, and fingerprint patterns. Arch Ophthalmol. Dec 1974;92(6):475-82. [Medline].
Weiss JS, Moller H, Lisch W, Kinoshita S, et al. The IC3D classification of corneal dystrophies. Cornea. In press.
Boutboul S, Black GC, Moore JE, et al. A subset of patients with epithelial basement membrane corneal dystrophy have mutations in TGFBI/BIGH3. Hum Mutat. Jun 2006;27(6):553-7. [Medline].
Paliwal P, Sharma A, Tandon R, Sharma N, Titiyal JS, Sen S, et al. TGFBI mutation screening and genotype-phenotype correlation in north Indian patients with corneal dystrophies. Mol Vis. Jul 29 2010;16:1429-38. [Medline]. [Full Text].
Werblin TP, Hirst LW, Stark WJ, et al. Prevalence of map-dot-fingerprint changes in the cornea. Br J Ophthalmol. Jun 1981;65(6):401-9. [Medline].
Reidy JJ, Paulus MP, Gona S. Recurrent erosions of the cornea: epidemiology and treatment. Cornea. Nov 2000;19(6):767-71. [Medline].
Guerry D III. Fingerprint-like lines in the cornea. Am J Ophthalmol. 1950;33:724-726.
Nelson JD, Williams P, Lindstrom RL, et al. Map-fingerprint-dot changes in the corneal epithelial basement membrane following radial keratotomy. Ophthalmology. Feb 1985;92(2):199-205. [Medline].
Seitz B, Lisch W. Stage-related therapy of corneal dystrophies. Dev Ophthalmol. 2011;48:116-53. [Medline].
Buxton JN, Constad WH. Superficial epithelial keratectomy in the treatment of epithelial basement membrane dystrophy. Cornea. 1987;6(4):292-7. [Medline].
Itty S, Hamilton SS, Baratz KH, et al. Outcomes of epithelial debridement for anterior basement membrane dystrophy. Am J Ophthalmol. Aug 2007;144(2):217-221. [Medline].
Forstot SL, et al. Diamond burr keratectomy for the treatment of recurrent corneal erosion syndrome. Ophthalmol Suppl: Poster. 1994.
Soong HK, Farjo Q, Meyer RF, et al. Diamond burr superficial keratectomy for recurrent corneal erosions. Br J Ophthalmol. Mar 2002;86(3):296-8. [Medline].
Ohman L, Fagerholm P, Tengroth B. Treatment of recurrent corneal erosions with the excimer laser. Acta Ophthalmol (Copenh). Aug 1994;72(4):461-3. [Medline].
Orndahl MJ, Fagerholm PP. Phototherapeutic keratectomy for map-dot-fingerprint corneal dystrophy. Cornea. Nov 1998;17(6):595-9. [Medline].
Sridhar MS, Rapuano CJ, Cosar CB, et al. Phototherapeutic keratectomy versus diamond burr polishing of Bowman's membrane in the treatment of recurrent corneal erosions associated with anterior basement membrane dystrophy. Ophthalmology. Apr 2002;109(4):674-9. [Medline].
McLean EN, MacRae SM, Rich LF. Recurrent erosion. Treatment by anterior stromal puncture. Ophthalmology. Jun 1986;93(6):784-8. [Medline].
Dastgheib KA, Clinch TE, Manche EE, et al. Sloughing of corneal epithelium and wound healing complications associated with laser in situ keratomileusis in patients with epithelial basement membrane dystrophy. Am J Ophthalmol. Sep 2000;130(3):297-303. [Medline].
Rezende RA, Uchoa UC, Cohen EJ, et al. Complications associated with anterior basement membrane dystrophy after laser in situ keratomileusis. J Cataract Refract Surg. Nov 2004;30(11):2328-31. [Medline].
Kenyon KR, Paz H, Greiner JV, et al. Corneal epithelial adhesion abnormalities associated with LASIK. Ophthalmology. Jan 2004;111(1):11-7. [Medline].
Akhtar S, Bron AJ, Meek KM, et al. Clinical and ultrastructural findings in mare's tail lines of the corneal epithelium. Br J Ophthalmol. Jul 2004;88(7):864-7. [Medline].

