Atypical familial Papillon-Lefevre syndrome

The Papillon-Lefevre syndrome is a rare autosomal recessive disorder. Consa nguinity seems a notable prerequisite. Papillon-Lefevre syndrome manifests in the first 6 months of life with rapidly progressive periodontitis and se vere alveolar bone destruction leading to early loss of both the deciduous and permanent teeth in association with palmo-plantar hyperkeratosis. We pr esent 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 lesi ons and relatively mild periodontal disease. A number of other cases recent ly described have also had atypical features.