Atypical familial Papillon-Lefèvre syndrome.
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.
Inalöz
H S
HS
Department of Dermatology, University of Wales College of Medicine, Cardiff, UK. serhatinaloz@hotmail.com
Harman
M
M
Akdeniz
S
S
Inalöz
S S
SS
Isik
A G
AG
D000293:Adolescent; D006801:Humans; D008297:Male; D010214:Papillon-Lefevre Disease / Q000175:diagnosis / Q000235:genetics*