Early-onset periodontitis associated with Weary-Kindler syndrome: a case report.
Weary-Kindler syndrome is a rare and poorly understood genetic disorder that has manifestations of both epidermolysis bullosa and poikiloderma congenitale. There are approximately 70 cases documented in the past 40 years but no cases appear in the dental literature, although dental findings have been discussed superficially in dermatological and pediatric publications. This case reports on the periodontal findings and treatment for a 16-year-old female diagnosed with the syndrome. Early 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 actinomycetemcomitans and low levels of other commonly accepted periodontal pathogens. Tests for inflammation, including AST and elastase, were positive prior to therapy and greatly decreased after mechanical root instrumentation. A beneficial effect of non-surgical periodontal therapy was observed in the short-term follow-up.
Department of Clinical Dental Sciences, University of British Columbia, Vancouver.
C B
CB
Wiebe
J G
JG
Silver
H S
HS
Larjava
D000293:Adolescent; D016976:Aggregatibacter actinomycetemcomitans / Q000302:isolation & purification; D010520:Aggressive Periodontitis / Q000382:microbiology / Q000473:pathology* / Q000628:therapy; D016301:Alveolar Bone Loss / Q000473:pathology; D001219:Aspartate Aminotransferases / Q000032:analysis; D004820:Epidermolysis Bullosa / Q000235:genetics* / Q000473:pathology; D005260:Female; D005500:Follow-Up Studies; D005884:Gingival Hemorrhage / Q000473:pathology; D006801:Humans; D010196:Pancreatic Elastase / Q000032:analysis; D010787:Photosensitivity Disorders / Q000235:genetics* / Q000473:pathology; D016745:Root Planing; D011038:Rothmund-Thomson Syndrome / Q000235:genetics* / Q000473:pathology; D013577:Syndrome; D014080:Tooth Exfoliation / Q000473:pathology; D014094:Tooth, Deciduous
D001219:Aspartate Aminotransferases; D010196:Pancreatic Elastase