Papillon-Lefèvre syndrome. Successful treatment with oral retinoids in three patients.

Papillon-Lefèvre syndrome is a rare autosomal recessive disorder of palmoplantar keratinization in which there is a unique association of the palmoplantar hyperkeratosis and premature loss of both deciduous and permanent teeth. We report three patients with papillon-lefèvre syndrome who showed a remarkable degree of improvement after treatment with an oral retinoid.