2. Patient:A 7 years old child was referred to oral medician departmant of mashhad dental school by pediatric dentist (Dr Saraf)

3. Chief complaint  Mobility of permanent dentition

4. history  Mobility of primary tooth since he was 4 years old  early missing the primary tooth

5.  Pataint has dry scaly patches on the skin of the palms and the soles (palmar-plantar hyperkeratosis)

6. Oral lesion

7. periodontit

8. Pyogenic granuloma

9. Diagnosis…..???

10. papillon lefevre syndrom

11. Papillon Lefevre Syndrome  an extremely rare genetic disorder that typically becomes apparent from approximately one to five years  development of dry scaly patches on the skin of the palms and the soles (palmar-plantar hyperkeratosis)  severe inflammation and d papillon lefevre syndrom egeneration of periodontium

13. Etiology  Papillon-Lefevre Syndrome is transmitted as an autosomal recessive trait  Genetic analysis of several affected families (kindreds) suggests that the disorder may result from changes (mutations) of a gene that regulates production of an enzyme known as cathespin

14. Treatment

15.  SRP and OHI and Follow up  Retinoeid  Antibiotics  Genetic consultation

16. THANKS FOR ATTENTION