Papillon-Lefèvre syndrome: Oral aspects and treatment

Dermatol Ther. 2020 May;33(3):e13336. doi: 10.1111/dth.13336. Epub 2020 Apr 9.


Papillon-Lefèvre syndrome (PLS) is a rare disorder characterized by diffuse palmoplantar erythematous, fissured hyperkeratosis, and aggressive periodontal disease that starts in the early periods of childhood. Periodontal disease occurs with the early loss of deciduous teeth at the age of 2 to 4 years, followed by the loss of permanent teeth during adolescence. Prosthodontics management of PLS patients is very complex and sometimes requires invasive therapeutic treatments. Early diagnosis is essential for correct treatment management avoiding the possibility that patients are early edentulous. Management could be a conventional periodontal treatment and pharmacological therapy but in severe cases, digital techniques, could be help the clinician for increased patient comfort and minimized tissue damage.

Keywords: Papillon-Lefèvre syndrome; management; periodontitis; primary teeth; prosthodontics management; treatment approaches.

Publication types

  • Review

MeSH terms

  • Adolescent
  • Child, Preschool
  • Humans
  • Keratosis*
  • Papillon-Lefevre Disease* / diagnosis
  • Papillon-Lefevre Disease* / genetics
  • Papillon-Lefevre Disease* / therapy