Aggressive periodontitis associated with Papillon-Lefèvre syndrome: report of a 14-year follow-up.
[papillon-lefèvre syndrome]
This case report describes the periodontal management, therapeutic approach, and 14-year follow-up of a patient diagnosed with Papillon-Lefevre syndrome (PLS). A female child, diagnosed with PLS-associated periodontitis at the age of 9 years and 11 months, presented with hyperkeratosis of the palms and soles, as well as generalized aggressive periodontitis. The dental treatment comprised standard periodontal debridement, scaling and root planing, instructions on oral hygiene, restorations, extraction of hopelessly affected teeth and a therapeutic use of antibiotics. The concomitant supportive periodontal therapy and antibiotic coverage could not stop the loss of periodontal attachment and destruction of the alveolar bone. Four years after treatment was initiated, the last remaining teeth were extracted and complete dentures were constructed. The dentures have been periodically replaced and the patient continues to return for follow-up once a year. The combination of intensive periodontal treatment and antibiotic regimen only temporarily delayed periodontal disease progression and did not prevent loss of both primary and permanent teeth. The outcome of this long-term follow-up case report shows that management of PLS-associated periodontitis is further complicated when the patient is first seen in the mixed dentition stage or later. In these situations, the chances of controlling the progression of periodontal breakdown and minimizing tooth loss are greatly reduced.
