Papillon-Lefévre syndrome: a highly-suspected case.

Papillon-Lefévre syndrome (PLS) is characterized by severe periodontal disease extending to destruction of the alveolar bone surrounding deciduous teeth and palmoplantar hyperkeratosis of the skin. Increased susceptibility to infection has been reported among individuals with the cathepsin C (CTSC) gene mutation. This article reports a 7-year-old Japanese girl who presented with deciduous tooth mobility and was diagnosed as having PLS. Radiographic examination revealed loosening of deciduous incisors and the right second molar of the maxilla, and destruction of the alveolar bone around the residual deciduous dentition. However, there was no destruction of the alveolar bone around the permanent molars. The patient did not show the typical signs of CTSC polymorphism, which almost always negatively impacts certain activating enzymes. With respect to immune function, analysis of the patient's leukocytes indicated that H(2)O(2), chemotactic and phagocytotic functions were within the normal range. However, the special precautions normally applied to prevent infections in PLS patients undergoing dental treatment were taken.

Case report of intra-osseous fibroma: a study on odontogenic and desmoplastic fibromas with a review of the literature.

Intra-osseous fibromas of the jaw are classified by origin. Intra-osseous odontogenic fibromas have odontogenic epithelia, while desmoplastic fibromas do not. However, it is often difficult to determine the odontogenic origin for central fibromas. Three subjects with a diagnosis of intra-osseous fibroma were examined. Case 1 was a 35-year-old man found to have a panoramic radiograph from the right premolar to the mandibular ramus in the mandible that exhibited multilocular radiolucency. Within the radiolucency, small-radioopaque bodies were observed. Case 2 was a 13-year-old female, in whom a panoramic radiograph from the left premolar to the molar in the mandible showed multilocular radiolucency. Case 3 was a 51-year-old female who exhibited a heart-shaped radiolucency in the panoramic radiograph of the left first molar area in the mandible. We also reviewed the literature for previously reported cases of intra-osseous odontogenic and desmoplastic fibroma. In 64 cases of intra-osseous odontogenic fibroma and 68 cases of desmoplastic fibroma we extracted data on age, sex, location, and radiographic findings. Based on the analysis of the reported literature cases, re-evaluation of the patients in our study revealed that case 1 could be classified as desmoplastic fibroma, while cases 2 and 3 were intra-osseous odontogenic fibromas.

Differential diagnosis between dentigerous cyst and benign tumor with an embedded tooth.

It has been generally recognized that the radiological appearances of cysts and tumors related to an embedded tooth are similar. However, based on their clinical experience, Abrams et al. pointed out that there was a difference between the two lesions at the attachment point to the embedded tooth. To investigate this difference, we conducted a study employing the radiographs of patients who visited Nihon University Dental Hospital at Matsudo and were pathologically defined as having a cyst or tumor. Using radiographs of these patients, we investigated the attachment point to the embedded tooth, and expressed the results as the proportion of the attachment point to the embedded tooth root length. The study was carried out in 100 patients with cysts (87 dentigerous cysts and 13 odontogenic keratocysts), and 27 patients with benign tumors (24 ameloblastomas and three adenomatoid odontogenic tumors). Prior to treatment based on the numerical results, the distribution of the results was examined. Thus, we evaluated several methods of examining the distributions, and found the best method to be discriminant analysis. The results showed that the discriminated boundary value (from the cemento-enamel junction) was 0.38 for the embedded tooth root length. The cases showing a boundary value of less than 0.4 for the cemento-enamel junction were judged to be cysts, and those showing a value of 0.4 or more were judged to be benign tumors. The rate of misjudgement was 28% in the cyst group and 33.3% in the benign tumor group.