title: Atypical familial Papillon-Lefèvre syndrome.
abstract: The Papillon-Lefèvre syndrome is a rare autosomal recessive disorder. Consanguinity seems a notable prerequisite. Papillon-Lefèvre 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-Lefèvre 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.
authors.affiliation: Department of Dermatology, University of Wales College of Medicine, Cardiff, UK. serhatinaloz@hotmail.com
authors.forename: H S
authors.initials: HS
authors.lastname: Inalöz
authors.forename: M
authors.initials: M
authors.lastname: Harman
authors.forename: S
authors.initials: S
authors.lastname: Akdeniz
authors.forename: S S
authors.initials: SS
authors.lastname: Inalöz
authors.forename: A G
authors.initials: AG
authors.lastname: Isik
mesh_terms: D000293:Adolescent; D006801:Humans; D008297:Male; D010214:Papillon-Lefevre Disease / Q000175:diagnosis / Q000235:genetics*