Cogan-guerry syndrome (microcystic corneal dystrophy; map-dot fingerprint dystrophy)   264

General: Etiology obscure; condition benign and asymptomatic; females predomin 212d31c antly affected; ultrastructural studies show discontinuous multilaminar thickened basement membrane under abnormal epithelium; the primary defect appears to be synthesis of abnormal basement membrane and adhesion complexes by the dystrophic epithelium.

Ocular: Reduced vision mainly with involvement of center of cornea; very fine wavy lines resembling fingerprints within or very close to corneal epithelium and best seen on biomicroscopy with retroillumination; fine grayish spheres (0.1- to O.5-mm diameter) in superficial corneal epithelium; maplike irregular border-lined slightly grayish area.

Clinical: None.

Cogan DG, et al. Microcystic dystrophy of the corneal epithelium. Trans Am Ophthalmol Soc 1964; 62:213.

Guerry D III. Fingerprint-like lines in the cornea. Am J Ophthalmol 1950; 33:724.

Luxenberg MN, et al. Superficial microcystic corneal dystrophy. Arch Ophthalmol 1975; 93:107.

Starck T, Hersh PS, Kenyon KR. Corneal dysgeneses, dystrophies and degenerations. In: Albert DM, Jakobiec FA, eds. Principles and Practice of Ophthalmology, vol. I. Philadelphia: WB Saunders, 1994:26-30.