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Purpose:The ectodermal dysplasia s yndromes are undere- stimated although precise inclusio n criteria have been formulated.The purpose is to establ ish easily detectable ophthalmologic symptoms and signs a s reliable criteria for ectodermal dysplasia syndromes.Me thods:Thirty-six pa-tients with confirmed ectodermal dysplasia syndromes were included in an observational case se ries:hypohidrotic ec-todermal dysplasia(30),EEC syndrome (3),AEC syn-drome (2),Gorlin-Goltz syndrome (1).Each patient was examined ophthalmologically.The p rincipal outcome measures were ocular symptoms and signs in patients with different ectodermal dysplasia syn dromes of varying severity.Result:Some 94.4%of the p atients suffered from dry eye symptoms.Reduction of e yebrows was seen in 94.4%;the lashes were altered in 91.6%.Changes of the meibomian glands were detecte d in 95.45%.Corneal changes such as pannus occur red later in life.Conclusions:Alterations of the meibomian glands,which were detected by meibomianoscopy,a re the most reliable ocular sign of ectodermal dysplasia syndromes.
Purpose: The ectodermal dysplasia s syndromes are undere- stigant and precise inclusio n criteria have been formulated. The purpose is to establ ish easily detectable ophthalmologic symptoms and signs as reliable criteria for ectodermal dysplasia syndromes.Me thods: Thirty-six pa-tients with confirmed ectodermal dysplasia syndromes were included in an observational case of se ries: hypohidrotic ec-todermal dysplasia (30), EEC syndrome (3), AEC syn-drome (2), Gorlin-Goltz syndrome The p rincipal outcome measures were ocular symptoms and signs in patients with different ectodermal dysplasia syn dromes of varying severity. Result: Some 94.4% of the p atients suffered from dry eye symptoms. Reduction of e yebrows was seen in 94.4%; the lashes were altered in 91.6% .Changes of the meibomian glands were detecte d in 95.45% .Corneal changes such as pannus occur red later in life.Conclusions: Alterations of the meibomian glands, which were detected by meibomi anoscopy, a re the most reliable ocular sign of ectodermal dysplasia syndromes.