Ustekinumab as an Effective Therapeutic Option in Papillon-Lefèvre Syndrome: Long-Term Follow-Up.

Papillon-Lefèvre syndrome (PLS) is a rare autosomal recessive genodermatosis characterized by palmoplantar hyperkeratosis and severe periodontitis, leading to premature loss of both primary and permanent teeth, and treatment responses are often unsatisfactory. We report a 20-year-old female patient with PLS who presented with extensive palmoplantar hyperkeratosis and widespread psoriasiform plaques refractory to multiple conventional therapies. Treatment with ustekinumab resulted in marked clinical improvement by the sixth month after three subcutaneous injections. The patient has remained in sustained clinical remission during 6 years of continuous therapy, with only mild and intermittent recurrent plaques on the knees, suggesting that biologic therapy may represent a sustainable option in selected cases of PLS.