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CASE PRESENTATION Patient:A 7 years old child was referred to oral medician departmant of mashhad dental school by pediatric dentist (Dr Saraf) Chief complaint  Mobility of permanent dentition history  Mobility of primary tooth since he was 4 years old  early missing the primary tooth  Pataint has dry scaly patches on the skin of the palms and the soles (palmar-plantar hyperkeratosis) Oral lesion periodontit Pyogenic granuloma Diagnosis…..??? papillon lefevre syndrom 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 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 Treatment Treatment  SRP and OHI and Follow up  Retinoeid  Antibiotics  Genetic consultation THANKS FOR ATTENTION
 
									 
									 
									 
									 
									 
									 
									 
									 
									 
									 
									 
									 
									 
									 
									 
									 
                                             
                                             
                                             
                                             
                                             
                                             
                                             
                                             
                                             
                                             
                                            