Papillon-Lefèvre syndrome: a report of two cases.

Abstract

Papillon-Lefèvre syndrome is a rare disease characterized by skin lesions caused by palmar-plantar hyperkeratosis, and severe periodontal destruction involving both the primary and permanent dentitions. It is transmitted as an autosomal recessive condition and consanguinity of parents is evident in about one-third of cases. This paper describes two preschool children who presented at the Paediatric Dentistry Department, Sheffield, UK, with progressively loosening teeth and discomfort during eating. The medical history revealed scaling on the hands and feet, which had been medically diagnosed as eczema. Papillon-Lefèvre syndrome was diagnosed in both cases.