title: Initial expression of the Papillon-Lefevre syndrome in consanguine family.
abstract: A rare case of Papillon-Lefevre syndrome is discussed with clinicoradiological presentation. The purpose of the case report is to make the medical community aware of this rare syndrome and its association with consanguinity. Papillon-Lefevre syndrome is an extremely rare genodermatosis of autosomal-recessive inheritance which usually manifests itself between the ages of 6 months to 4 years characterised by diffuse palmoplanter hyperkeratosis (keratoderma), and rapidly progressive and devastating periodontitis, affecting the primary as well as permanent dentition. Papillon-Lefevre syndrome results from a combination of host and bacterial factors, including recessive gene, consanguinity, specific periodontal pathogens and lack of thorough oral hygiene. The present case report describes Papillon-Lefevre syndrome and its association with consanguinity in a 3-year-old girl.
authors.affiliation: Department of Oral Medicine and Radiology, Rungta College of Dental Sciences, Bhilai, Chhattisgarh, India.
authors.forename: Anand Pratap
authors.initials: AP
authors.lastname: Singh
authors.forename: T R
authors.initials: TR
authors.lastname: Chaitra
authors.forename: Surendra Pratap
authors.initials: SP
authors.lastname: Singh
authors.forename: Adwait Uday
authors.initials: AU
authors.lastname: Kulkarni
mesh_terms: D002675:Child, Preschool; D003241:Consanguinity; D003475:Curettage; D003773:Dental Plaque / Q000209:etiology / Q000628:therapy; D012534:Dental Scaling; D005260:Female; D005888:Gingival Pocket / Q000209:etiology / Q000628:therapy; D005889:Gingival Recession / Q000209:etiology / Q000628:therapy; D006801:Humans; D010214:Papillon-Lefevre Disease / Q000150:complications* / Q000175:diagnosis*; D011862:Radiography, Panoramic; D016388:Tooth Loss / Q000209:etiology*