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Fleck corneal dystrophy
Fleck corneal dystrophy (FCD) is a rare generally asymptomatic form of stromal corneal dystrophy (see this term) characterized by multiple asymptomatic, non-progressive opacities disseminated throughout the corneal stroma with no effect on visual acuity.
ORPHA:98970Classification level: Disorder
- François-Neetens speckled corneal dystrophy
- Prevalence: <1 / 1 000 000
- Inheritance: Autosomal dominant
- Age of onset: All ages
- ICD-10: H18.5
- OMIM: 121850
- UMLS: C1562113
- MeSH: -
- GARD: -
- MedDRA: -
Prevalence of this form of corneal dystrophy is not known. FCD affects males and females equally.
The condition may occur throughout life and has been reported in children as young as 2 years of age. Corneal sensation is usually normal. Vision is not affected but mild photophobia may occur.
Fleck corneal dystrophy is caused by a mutation in the PIKFYVE gene (2q34).
Lesions are mostly minute and symmetrical. Opacities are sometimes small, oval, round, or semi-circular with distinct borders resembling flecks in the central and peripheral cornea. Opacities may also resemble snowflakes or clouds and consist of small grayish aggregations with ill-defined margins, occurring particularly in the central third of the cornea.
An autosomal dominant pattern of inheritance has been reported.
Management and treatment
Fleck dystrophy does not require specific treatment.