Cases reported "Papillon-Lefevre Disease"

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1/9. 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.
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2/9. 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.
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3/9. Successful periodontal maintenance of a case with Papillon-Lefevre syndrome: 12-year follow-up and review of the literature.

    BACKGROUND: Various approaches to treating the periodontal condition associated with Papillon-Lefevre syndrome have been reported. These include oral hygiene instruction, use of mouthrinses, frequent debridement, multiple antibiotic regimens, periodontal surgery, extraction of hopeless teeth, and extraction of all deciduous teeth. Because Papillon-Lefevre syndrome is rare, most publications are case reports, and very few document long-term successful treatment of the periodontal condition. methods: In 1986, a 3.5-year-old Indo-Canadian male was diagnosed with Papillon-Lefevre syndrome and began periodontal treatment. Initial therapy consisted of debridement every 3 weeks, a 0.12% chlorhexidine mouthrinse, 2 regimens of metronidazole, and oral hygiene instruction for his parents. After 10 months it became apparent that the treatment was having little beneficial effect, since the periodontal destruction continued and teeth 51 and 61 exfoliated. At age 4, all remaining deciduous teeth were extracted and complete dentures inserted for the following 2-year edentulous period; then a 3-month maintenance schedule was maintained. RESULTS: The patient is now 17 years old and all his adult teeth are present with the exception of the third molars. His oral hygiene varies between moderate and good, with his most recent plaque score at 80% effectiveness. There are no probing depths greater than 4 mm, with the exception of the distal of the lower second molars where opercula are present. CONCLUSIONS: Extraction of all the deciduous teeth followed by a period of edentulousness may partially explain the fact that there has been no recurrent attachment loss in the permanent teeth up to age 17. Other explanations are discussed as part of the literature review of Papillon-Lefevre syndrome.
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4/9. Papillon-Lefevre syndrome: a case report.

    We report the first diagnosed case of Papillon-Lefevre syndrome in thailand. The patient is the youngest child of consanguinous parents, and she has had symmetrical hyperkeratotic plaques on both plantar surfaces since birth with a history of chronic gingivitis, periodontitis, and premature loss of primary dentition. The histologic study revealed compact hyperkeratosis with epidermal acanthosis. Radiologic studies of the skull were normal. The radiographic panoramic view of the oral cavity revealed generalized severe vertical and horizontal alveolar bone loss. The immunologic analysis of polymorphonuclear leukocyte phagocytic function by nitrobluetetrazolium test (NBT test) showed decreasing response to latex stimulation. serum parathyroid hormone, calcium, phosphate, and alkaline phosphatase levels were within normal limits. The skin lesions were temporary relieved with topical keratolytic agents. The oral lesions were improved by the extraction of hopeless teeth and conventional periodontal treatments.
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5/9. Treatment of prepubertal periodontitis. A case report and discussion.

    This paper reports the treatment of prepubertal periodontitis in a 3-year-old girl with Papillon-Lefevre syndrome. Initially, the patient was found to have a myeloperoxidase deficiency and microbiological tests have identified bacteroides and Fusobacterium, in 60% and 25%, respectively of the total number of microbial flora cultivated. The initial treatment was extraction of all the primary teeth with grade 3 mobility, scaling, root planing and daily subgingival irrigation with a 0.2% solution of chlorhexidine. Several months before the eruption of the first permanent molars, the rest of the primary teeth were extracted. The patient was treated with daily subgingival irrigation of chlorhexidine and weekly professional oral hygiene. At the age 6 1/2 years, the permanent teeth have normal gingiva and crevice depths; microbiological investigation reveals a prevalence of the coccoid forms, and radiographs show no evidence of periodontal pathology.
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6/9. Periodontal therapy in siblings with Papillon-Lefevre syndrome and tinea capitis: a report of two cases.

    OBJECTIVE: Report of clinical and microbiological periodontal findings before and 6 months after treatment of two siblings with Papillon-Lefevre syndrome (PLS) and tinea capitis. methods: Two brothers, RG 3 years and NG 5 years of age, were referred for treatment due to premature mobility of their deciduous teeth. Probing depths (PPD), attachment levels (PAL-V), and furcation involvements were examined clinically. Panoramic radiographs were taken. Subgingival plaque samples within the deepest pocket of each tooth were taken and analysed by real-time polymerase chain reaction (PCR) for actinobacillus actinomycetemcomitans (AA), porphyromonas gingivalis, Tannerella forsythensis, treponema denticola, fusobacterium nucleatum, and prevotella intermedia. One-stage full-mouth scaling and extraction of hopeless teeth were performed under general anaesthesia, followed by systemic amoxicillin and metronidazole for 7 days. Clinical and microbiological analyses were performed 6 months after treatment. RESULTS: Before treatment, both siblings had exhibited PPD of up to 13 mm, Class III furcation defects at four teeth, and marginal suppuration. AA was detected in both patients and at all teeth at levels ranging from 3.0 x 10(2) to 5.1 x 10(6). Both patients exhibited palmar and plantar hyperkeratosis. Seven teeth were extracted from RG, and nine from NG. Six months after treatment, PPD had been reduced to patients can be treated successfully. Suppression of AA to below detection level seems to be of high significance.
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7/9. Treatment of rapidly destructive periodontitis in Papillon-Lefevre syndrome. Laboratory and clinical observations.

    This paper reports the successful treatment of the periodontal component of the Papillon-Lefevre syndrome in 2 siblings. Treatment consisted of extractions of periodontally-involved teeth under antibiotic therapy. The exogenous source of the suspected pathogenic A. actinomycetemcomitans in this family, identified as a pet dog, was also treated with antibiotics. Assessments of monocyte function and levels of serum antibodies against A. actinomycetemcomitans were performed prior to an immediately after treatment. Microbiological screening of subgingival dental plaque of the PLS patients as well as the rest of the household members, including the dog, was performed every month during the study period. Remission of the rapidly destructive periodontitis, as well as an earlier-described monocyte dysfunction in these two PLS patients occurred concomitantly with the eradication of A. actinomycetemcomitans from the family. 15 months after this treatment, the children still had no signs of periodontitis or of A. actinomycetemcomitans infection. The legitimacy with which PLS is defined as a disease entity is discussed and questioned.
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8/9. Papillon-Lefevre syndrome associated early onset periodontitis: a review and case study.

    A case of Papillon-Lefevre syndrome that has been managed successfully for six years is reported. Papillon-Lefevre syndrome is a rare form of early onset periodontitis that occurs at a rate of 1-3 per million. Diagnostic features include palmar-plantar hyperkeratosis and rapid periodontal destruction. Although the etiology of this syndrome is unknown, current theories on the nature of the underlying defect fall into three main categories: anatomical, bacterial and host response. Historically, Papillon-Lefevre syndrome was thought to lead to the inevitable loss of both the primary and permanent dentitions. However, a recently proposed treatment involving antibiotic coverage, extraction of the primary dentition and a period of edentulism has been shown to be effective in maintaining the permanent dentition. Since treatment may begin prior to the eruption of the permanent dentition, early recognition of Papillon-Lefevre syndrome is critical. Any young patient who exhibits palmar hyperkeratosis should be examined carefully for periodontal breakdown. Since the number of cases available for study is limited, referral of such individuals to University dental clinics may allow for a more specific analysis of immune or bacterial factors that may lead to a better understanding of this disease.
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9/9. Dental treatment of Papillon-Lefevre syndrome: 15-year follow-up.

    A 9-year-old girl was initially treated for the periodontal component of Papillon-Lefevre syndrome by extraction of all patient's erupted teeth, after unsuccessful clinical treatment with two different antibiotics. Follow-up dental records at age 24 showed the patient to have generalized gingivitis and poor oral hygiene; however, no additional teeth were lost or mobile. Radiographically, the alveolar crests, lamina dura, and periodontal ligament spaces appeared normal for a subject with missing teeth. Initially, the patient had depressed polymorphonuclear leukocyte (PMN) chemotaxis and adherence, as well as evidence of periodontal infection with actinobacillus actinomycetemcomitans, (A.a.). The 6 and 15-year follow-ups showed normal PMN function and no detectable A.a. The improvement of the patient's PMN function was coincident with lack of detection of certain periodontopathic bacteria. If the PMN dysfunction of PLS is secondary to the infection, the reasons for the initiation of the disease still need to be clarified.
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