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Dystrophia Smolandiensis: a novel morphological picture of recurrent corneal erosions

Hammar, Björn LU ; Lagali, Neil; Ek, Stefan; Seregard, Stefan; Dellby, Anette and Fagerholm, Per (2010) In Acta Ophthalmologica 88(4). p.394-400
Abstract
Purpose: The aim of this study was to describe morphological changes in Dystrophia Smolandiensis, a corneal disease that is characterized by recurrent corneal erosive episodes and the formation of central corneal keloid-like opacities in approximately half of those affected. Methods: The corneas of seven affected individuals were examined using in-vivo confocal microscopy. Specimens of one primary corneal graft, one regraft and one biopsied keloid-like region - all obtained from members of a large family with the disease - were re-examined with a light microscope. Sections were stained with Congo red and analysed immunohistochemically for fibronectin and S100A4. Results: Light microscopic examination revealed epithelial hyperplasia,... (More)
Purpose: The aim of this study was to describe morphological changes in Dystrophia Smolandiensis, a corneal disease that is characterized by recurrent corneal erosive episodes and the formation of central corneal keloid-like opacities in approximately half of those affected. Methods: The corneas of seven affected individuals were examined using in-vivo confocal microscopy. Specimens of one primary corneal graft, one regraft and one biopsied keloid-like region - all obtained from members of a large family with the disease - were re-examined with a light microscope. Sections were stained with Congo red and analysed immunohistochemically for fibronectin and S100A4. Results: Light microscopic examination revealed epithelial hyperplasia, absence of Bowman's layer and subepithelial fibrosis. Fibronectin was expressed in the area of subepithelial fibrosis, and the keratocytes in this area generally expressed S100A4. The biopsy specimen stained positive for Congo red, suggesting an amyloid deposit. In-vivo confocal microscopy confirmed epithelial abnormalities, loss of Bowman's layer and significant alterations of the subbasal nerve plexus in affected individuals. Conclusion: The morphological picture in Dystrophia Smolandiensis is novel for a condition dominated by recurrent corneal erosions at the clinical level. Although no single morphological feature unique to the disease could be found, the general morphological pattern of pathology (true keloid formation, absence of Bowman's layer, subepithelial fibrosis and abnormal subbasal nerves) probably reflects a novel phenotypic expression of the healing response to recurrent erosion of the corneal epithelium. However, the pathogenesis of Dystrophia Smolandiensis remains to be elucidated fully. (Less)
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author
organization
publishing date
type
Contribution to journal
publication status
published
subject
keywords
cornea, corneal dystrophies, confocal microscopy, morphology
in
Acta Ophthalmologica
volume
88
issue
4
pages
394 - 400
publisher
Wiley-Blackwell
external identifiers
  • wos:000278182000003
  • scopus:77952814992
ISSN
1755-3768
DOI
10.1111/j.1755-3768.2009.01548.x
language
English
LU publication?
yes
id
4628a978-cb79-4904-af30-473473b62227 (old id 1632557)
date added to LUP
2010-07-21 14:51:15
date last changed
2018-05-29 11:45:24
@article{4628a978-cb79-4904-af30-473473b62227,
  abstract     = {Purpose: The aim of this study was to describe morphological changes in Dystrophia Smolandiensis, a corneal disease that is characterized by recurrent corneal erosive episodes and the formation of central corneal keloid-like opacities in approximately half of those affected. Methods: The corneas of seven affected individuals were examined using in-vivo confocal microscopy. Specimens of one primary corneal graft, one regraft and one biopsied keloid-like region - all obtained from members of a large family with the disease - were re-examined with a light microscope. Sections were stained with Congo red and analysed immunohistochemically for fibronectin and S100A4. Results: Light microscopic examination revealed epithelial hyperplasia, absence of Bowman's layer and subepithelial fibrosis. Fibronectin was expressed in the area of subepithelial fibrosis, and the keratocytes in this area generally expressed S100A4. The biopsy specimen stained positive for Congo red, suggesting an amyloid deposit. In-vivo confocal microscopy confirmed epithelial abnormalities, loss of Bowman's layer and significant alterations of the subbasal nerve plexus in affected individuals. Conclusion: The morphological picture in Dystrophia Smolandiensis is novel for a condition dominated by recurrent corneal erosions at the clinical level. Although no single morphological feature unique to the disease could be found, the general morphological pattern of pathology (true keloid formation, absence of Bowman's layer, subepithelial fibrosis and abnormal subbasal nerves) probably reflects a novel phenotypic expression of the healing response to recurrent erosion of the corneal epithelium. However, the pathogenesis of Dystrophia Smolandiensis remains to be elucidated fully.},
  author       = {Hammar, Björn and Lagali, Neil and Ek, Stefan and Seregard, Stefan and Dellby, Anette and Fagerholm, Per},
  issn         = {1755-3768},
  keyword      = {cornea,corneal dystrophies,confocal microscopy,morphology},
  language     = {eng},
  number       = {4},
  pages        = {394--400},
  publisher    = {Wiley-Blackwell},
  series       = {Acta Ophthalmologica},
  title        = {Dystrophia Smolandiensis: a novel morphological picture of recurrent corneal erosions},
  url          = {http://dx.doi.org/10.1111/j.1755-3768.2009.01548.x},
  volume       = {88},
  year         = {2010},
}