Papillon-Lefevre syndrome: A case report with review of literature

Abstract

Papillon-Lefevre syndrome is an extremely rare genodermatosis inherited as an autosomal recessive trait, affecting children between the ages of 1-4 years. It has a prevalence of 1-4 cases per million persons. Males and females are equally affected and there is no racial predominance. The disorder is characterized by diffuse palmoplantar keratoderma and premature loss of both deciduous and permanent teeth. Often, there is associated hyperhidrosis of the palms and soles resulting in a foul-smelling odor. Well-demarcated psoriasiform plaques occur on the elbows and knees. The second major feature of PLS is severe periodontitis, which starts at age 3 or 4 years. We report a case of a 14-year-old patient with Papillon-Lefevre syndrome.