Posterior polymorphous corneal dystrophy 1
Encyclopedia
Posterior Polymorphous Corneal Dystrophy 1 (PPCD1; sometimes also Schlichting dystrophy) is a rare disease of the cornea
, characterised by changes in Descemet's membrane
and endothelial layer
. Symptoms mainly consist of decreased vision due to corneal edema
. In some cases they are present from birth, other patients are asymptomatic. Histopathological analysis shows that the cells of endothelium have some characteristics of epithelial cells, and endothelium itself sometimes consists of more than one cell layer. The disease was first described in 1916 by Koeppe as keratitis bullosa interna.
Cornea
The cornea is the transparent front part of the eye that covers the iris, pupil, and anterior chamber. Together with the lens, the cornea refracts light, with the cornea accounting for approximately two-thirds of the eye's total optical power. In humans, the refractive power of the cornea is...
, characterised by changes in Descemet's membrane
Descemet's membrane
Descemet's membrane is the basement membrane that lies between the corneal proper substance, also called stroma, and the endothelial layer of the cornea. It is composed of a different kind of collagen than the stroma. The endothelial layer is located at the posterior of the cornea...
and endothelial layer
Corneal endothelium
The corneal endothelium is a single layer of cells on the inner surface of the cornea. It faces the chamber formed between the cornea and the iris....
. Symptoms mainly consist of decreased vision due to corneal edema
Edema
Edema or oedema ; both words from the Greek , oídēma "swelling"), formerly known as dropsy or hydropsy, is an abnormal accumulation of fluid beneath the skin or in one or more cavities of the body that produces swelling...
. In some cases they are present from birth, other patients are asymptomatic. Histopathological analysis shows that the cells of endothelium have some characteristics of epithelial cells, and endothelium itself sometimes consists of more than one cell layer. The disease was first described in 1916 by Koeppe as keratitis bullosa interna.
See also
- Posterior polymorphous corneal dystrophy 2Posterior polymorphous corneal dystrophy 2Posterior Polymorphous Corneal Dystrophy 2 is a rare disease of the cornea. It is linked to the mutations in COL8A2.-See also:*Posterior polymorphous corneal dystrophy 1*Posterior polymorphous corneal dystrophy 3-External links:...
- Posterior polymorphous corneal dystrophy 3Posterior polymorphous corneal dystrophy 3Posterior Polymorphous Corneal Dystrophy 3 is a rare disease of the cornea. It is linked to the mutations in ZEB1 gene.-See also:*Posterior polymorphous corneal dystrophy 1*Posterior polymorphous corneal dystrophy 2-External links:...