Dystrophy of the Bowman’s membrane type I , better known as Reis – Buckler’s corneal dystrophy, is a rare form of dystrophy of the human cornea that affects the layer called the Bowman’s membrane . The disease is associated with mutations in the TGFBI gene encoding keratoepithelium .
| Bowman membrane dystrophy type I | |
|---|---|
 Reis - Bucklers dystrophy: reticular opacities of the outer part of the cornea | |
| ICD-10-KM | |
| ICD-9-KM | |
| Omim | 608470 |
| Mesh | and |
Symmetric reticular opacities, usually occurring in the cornea of both eyes by 4–5 years, lead to convexity of the corneal epithelium , causing erosion, causing bouts of hyperemia , pain, photophobia .
The disease was first described in 1917 by V. Reis [2] , and in 1949 by M. Bucklers [3] .
Optical microscopy , which shows characteristic red-colored (three-color Masson stain) deposits of mutated keratoepithelin in the surface layer of the corneal stroma [4]
See also
- Granular dystrophy of the cornea type I
- Granular dystrophy of the cornea type II
- Dystrophy of the Bowman membrane type II
- Lattice dystrophy of the cornea type I
Notes
- ↑ Monarch Disease Ontology release 2018-06-29sonu - 2018-06-29 - 2018.
- ↑ Reis W. Fämiliare, fleckige Hornhautetartung (German) // Deutsche Medizinische Wochenschrift . - 1917. - Vol. 43. - P. 575.
- ↑ Bücklers M. Über eine weitere familiare Hornhautdystrophie (Reis) (German) // Klinische Monatsblätter für Augenheilkunde . - Stuttgart , 1949. - Vol. 114. - P. 386-397.
- ↑ Gordon K. Klintworth. Corneal dystrophies (English) // Orphanet Journal of Rare Diseases . - 2009. - Vol. 4. - DOI : 10.1186 / 1750-1172-4-7 . . PMID 19236704 .
Links
- Reis – Bucklers dystrophy at Digital Reference of Ophthalmology.