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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