1/95. Microbiological features of Papillon-Lefevre syndrome periodontitis. Papillon-Lefevre syndrome patients exhibit hyperkeratosis palmo-plantaris and severe periodontitis. The syndrome is an autosomal recessive trait, but the mechanism of periodontal destruction is not known. This report presents the clinical and microbiological features of an 11-year old girl with Papillon-Lefevre syndrome. Clinical examination included conventional periodontal measurements and radiographic analysis. In samples from 3 deep periodontal lesions, the occurrence of major suspected periodontopathic bacteria was determined by selective and non-selective culture and polymerase chain reaction (PCR) identification, and the presence of cytomegalovirus and Epstein-Barr type 1 virus by a nested-PCR detection method. 10 of 22 available teeth demonstrated severe periodontal breakdown. Major cultivable bacteria included actinobacillus actinomycetemcomitans (3.4% of total isolates), prevotella nigrescens (16.4%), fusobacterium nucleatum (14.3%) and peptostreptococcus micros (10.6%). A. actinomycetemcomitans, P. nigrescens, porphyromonas gingivalis and eikenella corrodens were identified by PCR analysis. The patient's non-affected parents and older brother revealed several periodontal pathogens but not A. actinomycetemcomitans. The viral examination demonstrated cytomegalovirus and Epstein-Barr type 1 virus in the subgingival sample of the Papillon-Lefevre syndrome patient. The father and brother yielded subgingival cytomegalovirus but not Epstein-Barr type 1 virus. We hypothesize that human herpesviruses in concert with A. actinomycetemcomitans play important roles in the development of Papillon-Lefevre syndrome periodontitis. ( info) |
2/95. root resorption and signs of repair in Papillon-Lefevre syndrome. A case study. The aim of this investigation was to describe some tooth-related histological features of prepubertal periodontitis. Teeth extracted during treatment of two Papillon-Lefevre syndrome patients were processed by means of the sawing and grinding technique. light microscopy examination revealed little or no cementum in the coronal parts of the roots. Resorptions of various depths (0.02 to 1.5 mm) and to various extents (affecting up to 1/3 of the root surface) were observed in the 5 investigated teeth. Some resorptive defects on 1 of the examined incisors showed signs of spontaneous repair. Extrinsic fibers were inserted into the new cellular intrinsic fiber cementum which had formed directly on the bottom of the defect. Intact acellular extrinsic fiber cementum was found where fibers were still attached. Here, the characteristic of pristine cementum, a hyaline layer of peripheral dentin, could be identified. If resorption was not present, the cementum did not show any signs of hypoplasia. Thus, histological features of prepubertal periodontitis in the current material were (i) areas of extensive resorption, (ii) signs of spontaneous repair, and (iii) healthy cementum. ( info) |
3/95. association of inflammatory pseudotumor of the liver and Papillon-Lefevre syndrome--case report. A case of hepatic inflammatory pseudotumor mimicking malignancy in a 4-year-old girl with the Papillon-Lefevre syndrome (PLS) is reported. Only recently, an association between this inherited syndrome and liver abscesses has been found. Its possible pathogenesis is discussed and immunologic defects resulting from the Papillon-Lefevre syndrome are presented. The development of inflammatory pseudotumor of the liver might be caused by immunologic disturbances and staphylococcal infection. The picture of the hepatic tumor on imaging in patients with PLS should be attributed rather to inflammatory than neoplastic process. ( info) |
4/95. Combined systemic and local antimicrobial therapy of periodontal disease in Papillon-Lefevre syndrome. A report of 4 cases. 4 patients, 2 pairs of siblings, suffering from Papillon-Lefevre syndrome were treated for periodontal disease. Following extraction of hopeless teeth, the children received scaling and adjunctive systemic antibiotics (metronidazole and amoxicillin for 7 to 10 days). In addition, they performed supragingival pulsated jet irrigation with 0.06% chlorhexidine digluconate 1 x daily. In 2 siblings, A. actinomycetemcomitans was suppressed subgingivally below detectable levels, pocket probing depths were reduced to 4 mm or less, and plaque and bleeding indices were low. No further disease progression was seen over a 3-year-period. Another female patient also showed clinical improvement and suppression of subgingival A. actinomycetemcomitans and B. forsythus up to the 9-month-follow-up, while her sister showed further attachment loss over the course of 4 years. The present case reports indicated that in some patients suffering from Papillon-Lefevre syndrome periodontal disease may be arrested by means of (i) oral hygiene instruction, (ii) extraction of severely diseased teeth, (iii) scaling, (iv) systemic antibiotics and (v) long-term antimicrobial irrigation. ( info) |
5/95. Leukocyte functions in 2 cases of Papillon-Lefevre syndrome. AIM: To investigate the role of leukocytes in the pathogenesis of Papillon-Lefevre syndrome (PLS). methods: Peripheral blood polymorphonuclear neutrophils (PMNs), monocytes (MNs) and gingival crevicular fluid (GCF) were obtained from 2 cases of PLS with typical features. The chemotaxis of PMNs and MNs were evaluated using a modified Boyden chamber. The adherence of PMNs was determined by adherence of PMNs to petri dishes. interleukin-8 (IL-8) in GCF was detected by sandwich ELISA. Elastase activity in GCF was measured with a low molecular weight substrate (S-2484) specific for granulocyte elastase. RESULTS: PMNs from both patients showed depressed chemotactic response to FMLP and IL-8. Total amounts of IL-8 in GCF from the 2 patients were much higher than those of the normal controls. Elastase activity was not significantly different from that of the controls. The adherence of PMN and the chemotaxis of MN in the 2 patients were normal. CONCLUSION: The depressed chemotactic response of PMN leads to decreased recruitment of PMN and/or release of lysozyme from PMN in the diseased gingival tissue, increasing the susceptibility of PLS patients to periodontal infection. ( info) |
6/95. A new family with Papillon-Lefevre syndrome: effectiveness of etretinate treatment. Papillon-Lefevre syndrome is characterized by the association of palmoplantar hyperkeratosis, severe periodontitis, and early loss of deciduous and permanent teeth. We report two patients from the same family, aged 21 and 30 years, who were unaware of their pathology; one was successfully treated with etretinate. ( info) |
7/95. Periodontal treatment of rapid progressive periodontitis in 2 siblings with Papillon-Lefevre syndrome: 15-year follow-up. AIMS: This paper reports the treatment of the periodontal component of the Papillon-Lefevre syndrome in 2 siblings (case A, born 1974; case B, born 1976). METHOD: The initial treatment, in 1982, consisted of extraction of all primary teeth, scaling and rootplaning of the erupted permanent teeth and systemic antibiotic therapy. During 15 years, continuous and intensive periodontal treatment consisted of chlorhexidine 0.2% rinses, bi-weekly professional prophylaxis, scaling and rootplaning or surgery if indicated. Systemic antibiotics often accompanied mechanical therapy after bacteriological analysis. RESULTS: In case A, a favourable number of permanent teeth could be maintained, but in case B, all permanent teeth were lost in spite of the intensive treatment. Darkfield microscopy at different intervals revealed high numbers of spirochetes and motile rods in both siblings. Only in case A were they temporarily reduced to zero after scaling and rootplaning combined with metronidazole. Anaerobic cultering revealed high numbers of actinobacillus actinomycetemcomitans (A.a) in both patients. In 1994, 2 years after combined amoxicillin/metronidazole therapy, no A.a could be detected in case A. In case B, A.a could still be detected and was found to be resistant to metronidazole. One year after extraction of all permanent teeth, could no A.a be detected in case B. CONCLUSION: Intensive periodontal treatment combined with antibiotic therapy was not able to prevent complete tooth loss in case B. In case A, the treatment was more effective, resulting in preserving a number of permanent teeth in a stable clinical situation. In these 2 cases, no attempt was made to create an edentulous period between the periodontally-diseased mixed dentition and the eruption of the remaining teeth, which may have contributed to treatment failure. ( info) |
8/95. Osseointegrated implants in a patient with Papillon-Lefevre syndrome. A 4 1/2-year follow up. BACKGROUND: The Saudi female reported in this study was diagnosed with Papillon-Lefevre syndrome. She had lost all her teeth, including third molars, at the age of 18 years. METHOD: She was wearing full dentures for 7 years, before 5 titanium implants were installed in the mandible. RESULTS: 4 1/2 years after the implant installation, the treatment was clinically and radiographically successful. Neither A. actinomycetemcomitans nor P. gingivalis were detected at any of the implants. This very encouraging result does not differ from what is generally reported in edentulous medically healthy patients. CONCLUSION: The use of implants could considerably enhance future therapeutic options for the severely dentally compromised patients with Papillon-Lefevre syndrome. ( info) |
9/95. Papillon-Lefevre syndrome with pyogenic hepatic abscess: a rare association. Papillon-Lefevre syndrome in a 14-year-old boy presenting with palmoplantar keratoderma, periodontosis, recurrent pyogenic infections of the skin, and hepatic abscesses is reported for its rarity and unusual manifestations. The patient showed a dramatic response to acitretin therapy. ( info) |
10/95. Combined mechanical and antibiotic periodontal therapy in a case of Papillon-Lefevre syndrome. BACKGROUND: Papillon Lefevre syndrome (PLS) is a rare entity and, as such, it is almost impossible to evaluate an effective therapy in a randomized controlled study. The amount of success reported after therapy for prepubertal periodontitis (PP) in PLS is highly variable from case to case. The goal of this case report is to evaluate the effects of a combined mechanical and antibiotic periodontal therapy regimen in the management of PLS. methods: A male patient was diagnosed as suffering from PP associated with PLS at the age of 7 years. He showed hyperkeratosis of the palms and soles, as well as advanced periodontal disease already affecting permanent teeth with maximal probing depth and vertical attachment loss of 12 mm and 11 mm, respectively. Subgingival debridement was performed with simultaneous administration of oral 250 mg amoxicillin 3 times daily and 250 mg metronidazole twice daily for one week. Clinical parameters were assessed and subgingival plaque was collected from all teeth prior to therapy and 7 and 26 months after treatment. Selective cultures for A. actinomycetemcomitans were incubated for each individual tooth and DNA probe analysis was performed for various periodontal pathogens. RESULTS: Prior to combined mechanical and antibiotic treatment, all teeth but one harbored actinobacillus actinomycetemcomitans subgingivally. However, at 7 and 26 months after therapy A. actinomycetemcomitans could be detected neither by culture nor by dna probes. Clinical parameters improved markedly and teeth erupting after therapy did not exhibit attachment loss of more than 1.5 mm during the observation period. CONCLUSIONS: Eradication (suppression beneath detection levels) of A. actinomycetemcomitans seems to play a significant role in the successful treatment of localized prepubertal periodontitis in PLS. ( info) |