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