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  1. Case presentation

  2. Patient:A 7 years old child was referred to oral medician departmant of mashhad dental school by pediatric dentist (Dr Saraf)

  3. Chief complaint • Mobility of permanent dentition

  4. history • Mobility of primary tooth since he was 4 years old • early missing the primary tooth

  5. Pataint has dry scaly patches on the skin of the palms and the soles (palmar-plantar hyperkeratosis)

  6. Oral lesion

  7. periodontit

  8. Pyogenicgranuloma

  9. Diagnosis…..???

  10. papillon lefevre syndrom

  11. 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

  12. 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

  13. Treatment

  14. Treatment • SRP and OHI and Follow up • Retinoeid • Antibiotics • Genetic consultation

  15. THANKS FOR ATTENTION

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