Inalöz, HSHarman, MAkdeniz, SInalöz, SSIsik, AG2024-04-242024-04-2420010926-9959https://doi.org/10.1046/j.1468-3083.2001.00121.xhttps://hdl.handle.net/11468/16496The 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.eninfo:eu-repo/semantics/closedAccessHyperkeratosisPapillon-Lefevre SyndromePeriodontitisPsoriasiform LesionsRetinoidsEditorial1514850WOS:0001695600000172-s2.0-00348485231145132310.1046/j.1468-3083.2001.00121.xQ1Q3