Long-term preservation of permanent teeth in a patient with Papillon-Lefèvre syndrome treated with etretinate.

Papillon-Lefèvre syndrome (PLS) is a rare autosomal recessive genodermatosis characterized by the association of palmoplantar keratoderma with severe periodontopathy resulting in premature loss of both deciduous and permanent dentition. We treated a patient with PLS with etretinate and followed him for 10 years. Treatment was started at age 9 years, soon after the eruption of secondary dentition. Healing of cutaneous and periodontal lesions was obtained in a few months and maintained during the four years of treatment. Six years after etretinate withdrawal the teeth are still free of periodontal disease and firmly anchored to the alveolar bone. Our case confirms that retinoid therapy can positively influence the development of normal dentition in PLS when it is started during the eruption of the permanent teeth, and suggests that this result can be maintained for a long time even after stopping therapy.