Papillon lefevre syndrome: review and report of three cases in one family

ABSTRACT

Papillon LefŠvre syndrome is a rare disease believed to be inherited as an autosomal recessive trait. This report describes 3 cases of Papillon LefŠvre syndrome affecting one Jordanian family. One girl [4.5 years] her two albino brothers [12 and 21 years] and their parents, who are first cousins were examined. The parents were not affected, but their 3 children exhibited the typical clinical features of Papillon LefŠvre syndrome palmar and plantar hyperkeratosis with severe destruction of the periodontal structure resulting in early loss of both primary and permanent dentition. Traditional treatment involving professional oral hygiene care [scaling, root planning, curettage] and prolonged systemic antibiotic therapy has proved to be ineffective