title: Reis-Bücklers' corneal dystrophy. Immunofluorescent and electron microscopic studies.
abstract: The anterior stroma, epithelium, and Bowman's layer have been proposed as the site of primary pathology in Reis-Bücklers' corneal dystrophy (RBCD). Immunofluorescent localization of laminin and bullous pemphigoid antigen (BPA) was compared with the ultrastructure of RBCD. As previously reported, patchy deposition of characteristic "peculiar curly" filaments was found in the supra-Bowman's, subepithelial fibrous tissue. We also recognized areas of early involvement with deposition of this "peculiar curly" material between a distorted epithelial basal lamina and a normal undisturbed Bowman's layer. In normal cornea, laminin and BPA localized to the epithelial basal lamina. In RBCD, laminin and BPA were in a piebald mosaic distribution throughout the aberrant subepithelial fibrous tissue between the basal lamina and the buried Bowman's layer. This indicates that RBCD is an epithelial disease, with the "peculiar curly" material paralleling the distribution of the attachment proteins.
authors.affiliation: Department of Ophthalmology, Northwestern University Medical School, Chicago, Illinois 60611.
authors.forename: E
authors.initials: E
authors.lastname: Lohse
authors.forename: E L
authors.initials: EL
authors.lastname: Stock
authors.forename: J C
authors.initials: JC
authors.lastname: Jones
authors.forename: L S
authors.initials: LS
authors.lastname: Braude
authors.forename: R B
authors.initials: RB
authors.lastname: O'Grady
authors.forename: S I
authors.initials: SI
authors.lastname: Roth
mesh_terms: D000368:Aged; D001324:Autoantigens / Q000032:analysis; D001485:Basement Membrane; D002352:Carrier Proteins; D002387:Cataract Extraction; D003094:Collagen; D003317:Corneal Dystrophies, Hereditary / Q000276:immunology* / Q000473:pathology; D016039:Corneal Transplantation; D003598:Cytoskeletal Proteins; D000071821:Dystonin; D004847:Epithelial Cells; D005404:Fixatives; D005455:Fluorescent Antibody Technique; D006801:Humans; D007797:Laminin / Q000032:analysis; D008297:Male; D008854:Microscopy, Electron; D009419:Nerve Tissue Proteins; D024041:Non-Fibrillar Collagens; D010375:Pedigree; D014792:Visual Acuity; D000097805:Collagen Type XVII
chemical_list: D001324:Autoantigens; D002352:Carrier Proteins; D003094:Collagen; D003598:Cytoskeletal Proteins; D000071821:Dystonin; D005404:Fixatives; D007797:Laminin; D009419:Nerve Tissue Proteins; D024041:Non-Fibrillar Collagens; D000097805:Collagen Type XVII; C486268:DST protein, human