Atypical familial Papillon-Lefevre syndrome

Inaloz H., Harman M., Akdeniz S., Inaloz S., Isik A.

JOURNAL OF THE EUROPEAN ACADEMY OF DERMATOLOGY AND VENEREOLOGY, vol.15, no.1, pp.48-50, 2001 (Journal Indexed in SCI) identifier identifier identifier

  • Publication Type: Article / Editorial Material
  • Volume: 15 Issue: 1
  • Publication Date: 2001
  • Doi Number: 10.1046/j.1468-3083.2001.00121.x
  • Title of Journal : JOURNAL OF THE EUROPEAN ACADEMY OF DERMATOLOGY AND VENEREOLOGY
  • Page Numbers: pp.48-50

Abstract

The Papillon-Lefevre syndrome is a rare autosomal recessive disorder. Consanguinity seems a notable prerequisite. Papillon-Lefevre syndrome manifests in the first 6 months of life with rapidly progressive periodontitis and severe alveolar bone destruction leading to early loss of both the deciduous and permanent teeth in association with palmo-plantar hyperkeratosis. We present two unusual cases of familial Papillon-Lefevre syndrome, one of whom has only late onset of mild skin lesions and the other has severe skin lesions and relatively mild periodontal disease. A number of other cases recently described have also had atypical features.