University of Iowa Health Care

Ophthalmology and Visual Sciences

EyeRounds.org

Fleck Corneal Dystrophy

Contributor: T.J. Clark, MD

Photographer: Brice Critser, CRA

Category: Cornea

Fleck Corneal Dystrophy (a.k.a. Francois-Neetens speckled corneal dystrophy) is an autosomal dominant disorder caused by a mutation in phosphoinositide kinase (gene locus 2q34) that results in the accumulation of glycosaminoglycans and complex lipids in swollen vacuolated keratocytes. Clinically, subtle, yet distinctive, small, translucent, gray-white, dandruff-like flakes are seen scattered throughout all levels of an otherwise clear corneal stroma, extending to the periphery. Signs of the disease are typically seen at birth or within the first few years of life. Affected patients are typically asymptomatic, but may have slight photophobia or decreased corneal sensitivity. The disease is non-progressive with a good prognosis.

Clinically, subtle, yet distinctive, small, translucent, gray-white, dandruff-like flakes are seen scattered throughout all levels of an otherwise clear corneal stroma, extending to the periphery
Clinically, subtle, yet distinctive, small, translucent, gray-white, dandruff-like flakes are seen scattered throughout all levels of an otherwise clear corneal stroma, extending to the periphery

Please see the EyeRounds review for more information about other corneal stromal dystrophies.

Reference

Weiss JS, Moller HU, Aldave AJ, et al. IC3D Classification of Corneal Dystrophies—Edition 2. Cornea. 2015;34:117-159.


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last updated: 09/14/2015