Papillon-Lefèvre syndrome: a study of the long-term clinical course of recurrent pyogenic infections and the effects of etretinate treatment.

A family with a clinical variant of Papillon-Lefèvre syndrome (PLS), associated with recurrent pyogenic infections, has been followed up for more than 20 years. Of the five living siblings, four were treated with etretinate for a period of at least 21 consecutive months. Clinical follow-up showed that the course of recurrent infections in susceptible PLS patients, although usually more severe in childhood, can be variable and unpredictable. The etretinate therapy resulted in marked improvement of the keratodermas, and was associated with complete remission of the pyodermas on both keratotic and non-keratotic skin. It is, therefore, suggested that etretinate may have a primary role in the prevention of recurrent pyogenic infections in susceptible PLS patients.