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