Francois (1) dystrophy (francois-neetens syndrome; central cloudy dystrophy; cloudy central corneal dystrophy)

General: Autosomal dominant; etiology unknown; not progressive; isolated keratocytes contain elevated amounts of glycosaminoglycans and lipids.

Ocular: Bilateral dystrophy of central third of cornea; snowflake patches covering the pupil; lesions show no definite structure or limits; more dense near Descemet membrane and becoming less toward the anterior surface toward the periphery; associated with central cloudy dystrophy; keratoconus; limbal dermoid; pseudoxanthoma elasticum; lenticular opacities; reduced corneal sensation.

Francois J. Une Novelle Dystrophie Heredo-familiale de la Cornee. J Genet Hum 1956; 5:189-l96.

Jablonsk S. Eponymic syndromes and diseases. Philadelphia: WB Saunders, 1969:110.

Merin S. Corneal dystrophies affecting the stroma. Cloudy central cornea dystrophy. In: Merin S, ed. Inherited eye diseases. New York: Marcel Dekker, 1991:22-41.

Pavan-Langston D. Cornea and external diseases. Central cloudy and parenchymatous dystrophy. In: Pavan-Langston D, ed. Manual of ocular diagnosis and therapy, 4th ed. Boston: Little, Brown and Company, 1995:108.

Smolin G. Corneal dystrophies and degenerations. In: Smolin G, Thoft RA, eds. The Cornea. Boston: Little, Brown and Company, 1994:516.