[The Papillon-Lefevre syndrome (keratosis palmoplantaris with periodontopathy). Treatment with etretinate].

The Papillon-Lefèvre syndrome is an autosomal recessive gene defect characterized by transgredient palmoplantar hyperkeratosis and periodontopathia leading to loss of the teeth. The syndrome is described in a 28-year-old man who had lost all of his teeth at the age of 10 years. Treatment with etretinate resulted in a marked improvement of the palmar and plantar skin lesions.