Fuchs' dystrophy reduces the number of specific cells (called endothelial cells) that make up the inner layer of the cornea. Endothelial cells are key to processing water that makes up the corneal cell structure. When the endothelial cells diminish, the cells stop processing water properly and fluid starts to build up. The corneal tissue gradually thickens, causing the cornea to become swollen and cloudy, losing its crystal-clear transparency.

Also seen in Fuchs' dystrophy, are dewdrop-shaped outgrowths called guttata in the layer just underneath the endothelial cell layer (called Descemet's membrane).

Because Fuchs' dystrophy is a progressive disease, over time, changes to the corneal cells may interfere with vision. The condition can result in corneal scar tissue, which may have to be removed surgically, and can even lead to blindness.

Fuchs' dystrophy is somewhat more common in women and usually affects people in their 50s or 60s, although occasionally it appears earlier in one's adulthood.

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