ABSTRACT
Papillon-Lefèvre syndrome (PLS) is a rare autosomal recessive disorder of keratinization associated with palmoplantar keratoderma and severe periodontitis resulting in complete edentulism in late adolescence. The pathognomonic dental features of PLS are pathologic migration, hypermobility, and exfoliation of the teeth without any signs of root resorption. It has been suggested that an effective way to treat PLS patients presenting early in the disease progression is extraction of the erupted primary dentition or hopeless permanent teeth followed by antibiotic coverage with periodontal therapy for the remaining teeth. Unfortunately, studies have shown that this regimen only temporarily delays the progression of periodontal disease and does not prevent further tooth loss and bone destruction in the long term. Post-tooth loss, atrophic ridges make conventional prosthodontic rehabilitation quite challenging, and more recently, implant-supported prostheses have been considered as a viable alternative. In a PLS patient, implant placement is complicated by inadequate bone volume; thus, bone augmentation techniques or the use of short implants is often considered. When large volumes of bone are required, parietal calvarium bone can be used to predictably reconstruct severe defects. A PLS patient aged 21 years presented a chief complaint of ill-fitting conventional complete dentures. The patient had severely atrophic ridges, requiring significant bone augmentation for an implant-supported prosthesis. The present case is the first example of bone augmentation using autogenous calvarium parietal graft followed by endosseous implant placement and prosthetic restoration in a PLS patient.
