Transcript Case presentation

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