Papillon-lefevre syndrome: Review of literature and report of three cases in the same family

Abstract

The objective of this paper is to give a review of the literature on the aetiopathogenesis, clinical presentation, differential diagnosis and treatment of Papillon-Lefevre Syndrome, and present three cases of PLS in siblings of the same parents demonstrating typical clinical and radiological features of the condition. Three cases of Papillon-Lefevre syndrome affecting three girls among six siblings of normal parents who are first-degree cousins. The three siblings aged 14, 6 and 4 ⅇ years all had severe gingivitis, periodontitis with grossly mobile teeth and tooth migration. Other findings included premature exfoliation of deciduous teeth (2/3), premature loss of permanent teeth in one sibling. Plantar hyperkeratosis was detected in all of the cases, leading to a difficult and painful walking in two cases due to lesions on the soles. Palmar hyperkeratosis was present in the oldest sibling as well. Radiologic study confirmed alveolar bone destruction in two cases. Early recognition of this entity as well as multidisciplinary management may help in the prognosis of these cases.