title: Successful periodontal maintenance of a case with Papillon-Lefèvre syndrome: 12-year follow-up and review of the literature.
abstract: BACKGROUND
  Various approaches to treating the periodontal condition associated with Papillon-Lefèvre syndrome have been reported. These include oral hygiene instruction, use of mouthrinses, frequent debridement, multiple antibiotic regimens, periodontal surgery, extraction of hopeless teeth, and extraction of all deciduous teeth. Because Papillon-Lefèvre syndrome is rare, most publications are case reports, and very few document long-term successful treatment of the periodontal condition.
  
  
  METHODS
  In 1986, a 3.5-year-old Indo-Canadian male was diagnosed with Papillon-Lefèvre syndrome and began periodontal treatment. Initial therapy consisted of debridement every 3 weeks, a 0.12% chlorhexidine mouthrinse, 2 regimens of metronidazole, and oral hygiene instruction for his parents. After 10 months it became apparent that the treatment was having little beneficial effect, since the periodontal destruction continued and teeth 51 and 61 exfoliated. At age 4, all remaining deciduous teeth were extracted and complete dentures inserted for the following 2-year edentulous period; then a 3-month maintenance schedule was maintained.
  
  
  RESULTS
  The patient is now 17 years old and all his adult teeth are present with the exception of the third molars. His oral hygiene varies between moderate and good, with his most recent plaque score at 80% effectiveness. There are no probing depths greater than 4 mm, with the exception of the distal of the lower second molars where opercula are present.
  
  
  CONCLUSIONS
  Extraction of all the deciduous teeth followed by a period of edentulousness may partially explain the fact that there has been no recurrent attachment loss in the permanent teeth up to age 17. Other explanations are discussed as part of the literature review of Papillon-Lefèvre syndrome.
authors.affiliation: Department of Oral Biological and Medical Sciences, Faculty of Dentistry, The University of British Columbia, Vancouver. cwiebe@unixg.ubc.ca
authors.forename: C B
authors.initials: CB
authors.lastname: Wiebe
authors.forename: L
authors.initials: L
authors.lastname: Häkkinen
authors.forename: E E
authors.initials: EE
authors.lastname: Putnins
authors.forename: P
authors.initials: P
authors.lastname: Walsh
authors.forename: H S
authors.initials: HS
authors.lastname: Larjava
mesh_terms: D000293:Adolescent; D000900:Anti-Bacterial Agents / Q000627:therapeutic use; D003773:Dental Plaque / Q000517:prevention & control; D005500:Follow-Up Studies; D006801:Humans; D008297:Male; D009067:Mouthwashes / Q000627:therapeutic use; D009910:Oral Hygiene; D010214:Papillon-Lefevre Disease / Q000150:complications*; D010353:Patient Education as Topic; D010510:Periodontal Diseases / Q000517:prevention & control*; D010514:Periodontal Pocket / Q000517:prevention & control; D014078:Tooth Eruption; D014081:Tooth Extraction; D014094:Tooth, Deciduous / Q000601:surgery
chemical_list: D000900:Anti-Bacterial Agents; D009067:Mouthwashes