Papillon-Lefevre syndrome is a rare disease, the principal symptom being hyperkeratosis palmo-plantaris accompanied by severe destruction of the alveolar bone in deciduous or permanent teeth.
We encountered a case of Papillon-Lefevre syndrome showing extremely severe periodontal disease of the entire row of deciduous teeth accompanied by hyperkeratosis palmo-plantaris. This patient was a 2 year,8 months old female with unstability of the deciduous teeth and halitosis as the chief complaints. At the age of about 4 years some deciduous teeth dropped out and because of difficulty in mastication, the remaining teeth were all extracted and dentures were set. Thereafter permanent teeth began eruption and now at the age of 10 years and 7 months 61|6 have already dropped out.
Additionally we discussed 34 cases of this syndrome reported in our country. They involved 17 males and 17 females. It was very interesting that 17 cases out of 34 cases were concerned with consanguineous.