EARLY-ONSET PERIODONTITIS ASSOCIATED WITH WEARY-KINDLER-SYNDROME - A CASE-REPORT

Weary-Kindler syndrome is a rare and poorly understood genetic disorde r that has manifestations of both epidermolysis bullosa and poikiloder ma congenitale. There are approximately 70 cases documented in the pas t 40 years but no cases appear in the dental literature, although dent al findings have been discussed superficially in dermatological and pe diatric publications. This case reports on the periodontal findings an d treatment for a 16-year-old female diagnosed with the syndrome. Earl y exfoliation of deciduous teeth, severe periodontal bone loss around many permanent teeth, and fragile bleeding gingiva were key features. Microbiological testing revealed an absence of Actinobacillus actinomy cetemcomitans and low levels of other commonly accepted periodontal pa thogens. Tests for inflammation, including AST and elastase, were posi tive prior to therapy and greatly decreased after mechanical root inst rumentation. A beneficial effect of non-surgical periodontal therapy w as observed in the short-term follow-up.