Intraoral findings of Papillon-LeFevre syndrome.
Papillon-Lefevre syndrome (PLS) is a rare autosomal, recessive condition characterized by hyperkeratosis of palms and soles of the feet and elbows and by rapid formation of periodontitis and hypermobility, migration and exfoliation of the teeth of primary and permanent dentition. The purpose of this report was to describe the case of an 8-year-old boy who presented to the Department of Oral Diagnosis and Radiology of Faculty of Dentistry of Ondokuz Mayis University with a chief complaint of mobility and rapid loss of teeth. Hyperkeratosis of palms and soles were realized. His gingivae were hyperemic and edematous, and the teeth were mobile. Histopathological examination of the specimen taken from the thickened skin was reported to be consistent with PLS. All teeth with poor prognosis were extracted and extensive periodontal therapy was administered, and a special denture was constructed.
Canger
Emin Murat
EM
Department of Oral Diagnosis and Radiology, Faculty of Dentistry, Ondokuz Mayis University, Samsun, Turkey. mcanger@gmail.com
Celenk
Peruze
P
Devrim
Inci
I
Yenisey
Murat
M
Gunhan
Omer
O
D016301:Alveolar Bone Loss / Q000209:etiology*; D002648:Child; D003241:Consanguinity; D017742:Dental Care for Chronically Ill; D003829:Denture, Partial; D006801:Humans; D008297:Male; D010214:Papillon-Lefevre Disease / Q000150:complications*; D010518:Periodontitis / Q000209:etiology* / Q000628:therapy; D014080:Tooth Exfoliation / Q000209:etiology; D014081:Tooth Extraction; D014086:Tooth Mobility / Q000209:etiology / Q000601:surgery