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: Anand Pratap Singh, T R Chaitra, Surendra Pratap Singh, Adwait Uday Kulkarni
• Journal: BMJ case reports (BMJ Case Rep) Vol. 2012 (Aug 24 2012) ISSN: 1757-790X [Electronic] England
• PMID: 22922917 (Publication Type: Case Reports, Journal Article)

Topics

• Child, Preschool
• Consanguinity
• Curettage
• Dental Plaque (etiology, therapy)
• Dental Scaling
• Female
• Gingival Pocket (etiology, therapy)
• Gingival Recession (etiology, therapy)
• Humans
• Papillon-Lefevre Disease (complications, diagnosis)
• Radiography, Panoramic
• Tooth Loss (etiology)