Papillon-Lefèvre syndrome. Report of two patients treated successfully with isotretinoin.

The Papillon-Lefèvre syndrome is an autosomal recessive disorder characterized by diffuse palmoplantar keratoderma and premature loss of both the deciduous and the permanent teeth. We report two female patients with Papillon-Lefèvre syndrome in whom treatment with oral isotretinoin produced remarkable improvement of the keratoderma within 2 weeks.