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

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

THANKS FOR ATTENTION