Dystrophia Smolandiensis: a novel morphological picture of recurrent corneal erosions.

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.

• Authors: Björn Hammar, Neil Lagali, Stefan Ek, Stefan Seregard, Anette Dellby, Per Fagerholm
• Journal: Acta ophthalmologica (Acta Ophthalmol) Vol. 88 Issue 4 Pg. 394-400 (Jun 2010) ISSN: 1755-3768 [Electronic] England
• PMID: 19681763 (Publication Type: Journal Article, Research Support, Non-U.S. Gov't)

Chemical References

• Amyloid
• Fibronectins
• S100 Calcium-Binding Protein A4
• S100 Proteins
• S100A4 protein, human

Topics

• Adult
• Aged
• Amyloid (metabolism)
• Corneal Dystrophies, Hereditary (metabolism, pathology, surgery)
• Corneal Opacity (metabolism, pathology, surgery)
• Female
• Fibronectins (metabolism)
• Humans
• Immunoenzyme Techniques
• Keratoplasty, Penetrating
• Male
• Microscopy, Confocal
• Recurrence
• S100 Calcium-Binding Protein A4
• S100 Proteins (metabolism)
• Young Adult