Treatment of the periodontal component of Papillon-Lefèvre syndrome

J Clin Periodontol. 1986 Jan;13(1):6-10. doi: 10.1111/j.1600-051x.1986.tb01407.x.


A 9-year-old girl was treated for the periodontal component of Papillon-Lefèvre syndrome, an autosomal recessive disease characterized by palmarplantar hyperkeratosis and premature loss of teeth. Initially, the patient was found to have a polymorphonuclear leukocyte chemotactic dysfunction, defective leukocyte adherence, and deep periodontal pockets harboring presumptive periodontopathic bacteria. After unsuccessful treatment with combined mechanical therapy and 2 different antibiotics, all of the patient's erupted teeth were extracted in an attempt to minimize the chance of infection of teeth yet to erupt. At age 16 years, the now-erupted teeth have normal gingiva and crevice depths, radiographs show no evidence of periodontal pathology, no periodontopathic bacteria are detected in gingival crevices, and leukocyte function is normal.

Publication types

  • Case Reports

MeSH terms

  • Bacteria / isolation & purification
  • Child
  • Dental Plaque / microbiology
  • Erythromycin / therapeutic use
  • Female
  • Humans
  • Keratoderma, Palmoplantar* / physiopathology
  • Papillon-Lefevre Disease* / physiopathology
  • Periodontal Diseases / drug therapy
  • Periodontal Diseases / microbiology
  • Periodontal Diseases / therapy*
  • Tetracycline / therapeutic use
  • Tooth Extraction


  • Erythromycin
  • Tetracycline