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This report details the successful prosthetic rehabilitation of a 25-year-old male patient with Papillon Lefèvre Syndrome (PLS) using an implant-supported hybrid prosthesis. Six implants were placed in the maxilla, and four were placed in the mandibular arch. All implants were inserted axially (non-tilted) and were planned to be loaded after a healing period of 6 months. One implant failed due to graft loss during the healing phase, which was removed and the remaining implants were restored with a hybrid prosthesis after 6 months, using the delayed loading protocol. The patient was followed-up for four years and all the remaining implants successfully integrated and remained fully functional during this period. The prosthesis significantly improved the functional, aesthetic, and psychological well-being of the patient. This case report is the first of its kind to use only four axially placed implants for rehabilitation of a PLS patient with a successful four-year follow-up.

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Background: Papillon-Lefèvre syndrome (PLS) is considered a rare syndrome, which is characterized by the presence of palmar-plantar hyperkeratosis and aggressively progressing periodontitis that finally leads to premature loss of both deciduous and permanent teeth. Case report: A four-year-old Egyptian boy presented with a maternal complaint that her child suffers from early loss of many teeth, presence of loose teeth along with an asymptomatic swelling related to the upper anterior area. The patient was diagnosed with PLS. A symptomatic management and prevention program was followed and the swelling was excised; afterwards diagnosed as peripheral ossifying fibroma. Conclusion: Early recognition and intervention for patients with PLS is essential to avoid the threat of being edentulous if left unmanaged.

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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.

Papillon-Lefèvre syndrome (PLS) is a rare primary immunodeficiency, which combines severe periodontal disease with edentulism and palmoplantar keratosis (PPK). PLS is inherited as an autosomal recessive trait and is due to mutations in the cathepsin C gene. The biological properties of the neutrophils (PN) are altered, leading to a gingival dysbiosis and bacterial overgrowth, with intense inflammation of the periodontium. We report the observation of a 4-year-old girl who presented to the clinic with gingivitis, partial edentulism, and PPK, whose diagnosis, raised after a long delay, was suggested by null cathepsin C activity and confirmed by the presence of heterozygous mutations in exon 4: c.628C>T, pArg210* and in exon 7: c.1286G>A, p.Trp429*. A multidisciplinary approach transformed the functional and esthetic prognosis and psychological behavior of this child. This classical observation describes this poorly known phenotype.

BACKGROUND: Papillon-Lefèvre Syndrome is a rare autosomal recessive disorder characterized by diffuse, transgradient palmoplantar keratoderma, destructive periodontitis beginning in childhood, premature loss of primary teeth, and frequent cutaneous and systemic pyogenic infections. Pyogenic liver abscess is an uncommon presentation of the disease present in this case.
METHODS: A 16-year-old Punjabi, Pakistani boy presented to the outpatient department of a tertiary-care hospital of Lahore with high-grade fever of 2 months duration. He had been treated for a pyogenic liver abscess 2 years back with antibiotics followed by incision and drainage. He had poor orodental hygiene, palmoplantar keratoderma and periodontitis. His parents had history of consanguinity. His brother and two cousins had similar skin lesions and were edentulous. An orthopentogram showed atrophy of the alveolar bone. He was treated with broad-spectrum antibiotics, and antipyretics for systemic infection. Multivitamins, topical steroids, topical keratolytics and emollients were used for his dermatological issues.
CONCLUSIONS: Our patient was successfully treated. His fever settled and his skin lesions improved with antibiotics, topical steroids and keratolytics. He was sent home and was asked to return for follow-up on a monthly basis.

Papillon-Lefevre syndrome (PLS) is a rare autosomal recessive disorder characterized by diffuse palmoplantar keratoderma and precocious aggressively progressing periodontitis, leading to the premature loss of deciduous and permanent teeth. Full mouth rehabilitation with implant supported fixed prosthesis provides numerous advantages over the conventional denture in terms of esthetics, function, longevity of the prosthesis, and patient satisfaction. We report a case of PLS in a 19-year-old female who underwent extraction of all the teeth followed by full mouth rehabilitation with implant supported fixed prostheses. A one-year follow up of the case showed functionally and esthetically stable dental implant with no signs of infection or bone loss.