Papillon- Lefèvre Syndrome: Report of a case and its management.

[papillon-lefèvre syndrome]

Papillon-Lefèvre Syndrome (PLS ) is a rare autosomal recessive disorder first described by two French physicians , Papillon and Lef èvre in 1924 . The disorder is characterized by diffuse palmoplantar keratoderma and precocious aggressively progressing periodontitis , leading to the premature loss of deciduous and permanent teeth at a very young age . The cutaneous lesions are usually manifested simultaneously with the intra-oral presentations and include keratotic plaques on the palms and soles varying from mild psoriasiform scaly skin to overt hyperkeratosis . The etiopathogenesis of the syndrome is relatively obscure and immunologic , genetic or possible bacterial etiologies have been proposed . Due to the vast degree of periodontal breakdown involved at such an early age , the dental surgeon is often the first to diagnose the syndrome . This paper presents a clinical presentation a 15 year old male diagnosed with Papillon- Lef èvre Syndrome . Key words :Papillon-Lefèvre Syndrome , palmoplantar keratoderma , rapidly progressing periodontitis .