Papillon-Lefèvre syndrome: a review of the literature and report of 4 cases

J Periodontol. 1995 May;66(5):413-20. doi: 10.1902/jop.1995.66.5.413.

Abstract

This report describes 4 cases of Papillon-Lefèvre syndrome (PLS) affecting 2 Jordanian families with a total of 8 children. In family A, 3 girls out of 6 siblings were affected; and in family B, the eldest of 2 brothers. The patients were between 4 1/2 and 12 years old, and their parents, who are first cousins, were not affected. Diagnosis was determined by history, and clinical, laboratory, and radiographic examinations. All present cases exhibited the typical clinical features of PLS: hyperkeratosis of the palms and soles and the presence of advanced destructive periodontitis resulting in early loss of both the primary and permanent dentitions. In Case 4, cutaneous lesions were noticed at age 6 months and the child was edentulous by the age of 12 years. In all cases, there was a relationship between increased severity of skin lesions and seasonal variations and intensified periodontal destruction. There was an early eruption of the permanent teeth. The teeth were caries-free with no sign of root resorption. Dentists play a significant role in the diagnosis and management of PLS patients.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Child
  • Child, Preschool
  • Consanguinity
  • Female
  • Humans
  • Jordan
  • Male
  • Papillon-Lefevre Disease / complications
  • Papillon-Lefevre Disease / pathology*
  • Periodontitis / etiology*
  • Periodontitis / pathology