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Öğe Atypical familial Papillon-Lefevre syndrome(European Acad Dermatology & Venereology, 2001) Inalöz, HS; Harman, M; Akdeniz, S; Inalöz, SS; Isik, AGThe 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.
