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Researchers describe new hereditary erosive corneal disease
Acta Ophthalmol. 2008;86(7):758-763.
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Dystrophia smolandiensis is the proposed name of a newly described hereditary corneal disease that has recurrent erosions as its main clinical component. A second feature is development of central superficial keloid-like structures.
Researchers in Sweden studied 28 individuals in a six-generational family in which recurrent corneal erosions were reported. The pedigree included 171 individuals. Twenty-one of the 28 subjects studied were affected with attacks that lasted 1 day to 7 days. Frequency and intensity of the attacks declined with age, with symptoms described as beginning as early as 8 months of age and sometimes being precipitated by a common cold, exposure to intense sunlight or exposure to draft.
No other eye diseases were identified in either affected or unaffected family members, except those due to aging, and no relationship was found between this disease and genes causing other clinically similar autosomal-dominant inherited corneal dystrophies.
"Furthermore, no clinical features associated with epithelial, Bowman's layer and stromal dystrophies associated with recurrent corneal erosions were found," the researchers said.