Papillon-Lefèvre syndrome

Indian Pediatr. 2003 Dec;40(12):1197-200.


Papillon- Lefèvre syndrome (PLS) is a rare autosomal recessive disorder of keratinization characterized by palmoplantar hyperkeratosis, periodontopathy and precocious loss of dentition. The exact pathomechanism of these clinical events mainly remains speculative. This paper describes two cases of PLS with classic clinical features and briefly review the relevant literature.

Publication types

  • Case Reports

MeSH terms

  • Abnormalities, Multiple / diagnosis
  • Adolescent
  • Child
  • Female
  • Genetic Predisposition to Disease
  • Humans
  • India
  • Papillon-Lefevre Disease / diagnosis*
  • Papillon-Lefevre Disease / genetics*
  • Physical Examination
  • Prognosis
  • Risk Assessment
  • Severity of Illness Index