Dental treatment of Papillon-Lefèvre syndrome: 15-year follow-up

A 9-year-old girl was initially treated for the periodontal component of Papillon-Lefèvre syndrome by extraction of all patient's erupted teeth, after unsuccessful clinical treatment with two different antibiotics. Follow-up dental records at age 24 showed the patient to have generalized gingivitis and poor oral hygiene; however, no additional teeth were lost or mobile. Radiographically, the alveolar crests, lamina dura, and periodontal ligament spaces appeared normal for a subject with missing teeth. Initially, the patient had depressed polymorphonuclear leukocyte (PMN) chemotaxis and adherence, as well as evidence of periodontal infection with Actinobacillus actinomycetemcomitans, (A.a.). The 6 and 15-year follow-ups showed normal PMN function and no detectable A.a. The improvement of the patient's PMN function was coincident with lack of detection of certain periodontopathic bacteria. If the PMN dysfunction of PLS is secondary to the infection, the reasons for the initiation of the disease still need to be clarified.