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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.
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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)
PapillonLefevre 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
