1/43. 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.- - - - - - - - - - ranking = 1keywords = palm (Clic here for more details about this article) |
2/43. 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.- - - - - - - - - - ranking = 1keywords = palm (Clic here for more details about this article) |
3/43. 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.- - - - - - - - - - ranking = 1keywords = palm (Clic here for more details about this article) |
4/43. 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.- - - - - - - - - - ranking = 1keywords = palm (Clic here for more details about this article) |
5/43. Atypical familial Papillon-Lefevre syndrome.The Papillon-Lefevre syndrome is a rare autosomal recessive disorder. consanguinity seems a notable prerequisite. Papillon-Lefevre syndrome manifests in the first 6 months of life with rapidly progressive periodontitis and severe alveolar bone destruction leading to early loss of both the deciduous and permanent teeth in association with palmo-plantar hyperkeratosis. We present two unusual cases of familial Papillon-Lefevre syndrome, one of whom has only late onset of mild skin lesions and the other has severe skin lesions and relatively mild periodontal disease. A number of other cases recently described have also had atypical features.- - - - - - - - - - ranking = 1keywords = palm (Clic here for more details about this article) |
6/43. Papillon Lefevre syndrome: management of a case.Papillon Lefevre syndrome is a rare syndrome autosomal recessive in nature, manifesting as keratosis palmoplantaris with periodontitis. A case of Papillon Lefevre syndrome present in a 12 year old boy is presented. Only the permanent maxillary canines were present in the oral cavity when the patient was first seen, and despite efforts towards preservation, they had to be extracted. (complete dentures were made and the patient was followed up for 26 months.- - - - - - - - - - ranking = 1keywords = palm (Clic here for more details about this article) |
7/43. Papillon-Lefevre syndrome: a case report of four affected siblings.Four siblings, products of a consanguineous marriage, had palmoplantar keratoderma and loss of teeth. In addition, 3 of the siblings had psoriasiform plaques on their elbows and/or knees and 1 had intracranial calcifications. To our knowledge, this is the second largest reported number of affected members in a family with Papillon-Lefevre syndrome, a rare genodermatosis.- - - - - - - - - - ranking = 1keywords = palm (Clic here for more details about this article) |
8/43. Papillon-Lefevre syndrome: serum immunoglobulin g (IgG) subclass antibody response to periodontopathic bacteria. A case report.BACKGROUND: Papillon-Lefevre syndrome (PLS) is a rare autosomal recessive disorder which is characterized by palmar-plantar hyperkeratosis and rapid periodontal destruction of both primary and permanent dentitions. In this case report, we present clinical features, and microbiological and immunological findings of 40 month-old Thai male PLS patient. methods: Microbiological examinations consisted of bacterial culture methods utilizing selective media, morphological identification, and biochemical tests. In addition, the specific serum IgG subclass antibody titers reactive with etiologic periodontal bacteria were determined by the dot-blot immunological analysis and enzyme linked immunosorbent assay (ELISA). RESULTS: The examinations revealed that the patient harbored 3 major suspected periodontopathic microorganisms, A. actinomycetemcomitans, P. gingivalis, and P. intermedia. The patient's serum IgG1, IgG2, and IgG3, but not IgG4, titers against A. actinomycetemcomitans were dramatically increased. The predominant IgG subclass was IgG1. In contrast, the IgG titers against other tested bacteria, P. gingivalis, P. intermedia, and F. nucleatum, appeared to be similar to those of a healthy control. CONCLUSIONS: A. actinomycetemcomitans seems to play a pivotal role in the bacteria-host interaction in PLS periodontal pathogenesis. Response of the specific serum IgG subclass antibody titers against the A. actinomycetemcomitans antigen has been demonstrated. This association warrants further investigation.- - - - - - - - - - ranking = 1keywords = palm (Clic here for more details about this article) |
9/43. Papillon-Lefevre syndrome: the response to acitretin.Papillon-Lefevre syndrome is a rare autosomal recessive disease comprising palmoplantar keratoderma and periodontitis. Palmoplantar keratoderma can be severe, necessitating systemic treatment. Different systemic retinoids were found to be highly efficacious. We describe the successful use of acitretin in one patient who had severe palmoplantar keratoderma, with maintenance of the improvement using topical treatment.- - - - - - - - - - ranking = 2keywords = palm (Clic here for more details about this article) |
10/43. Pyogenic liver abscess and Papillon-Lefevre syndrome: not a rare association.Papillon-Lefevre syndrome is a rare, autosomal recessive disease comprising palmoplantar keratoderma and periodontitis. Pyogenic liver abscess is an increasingly recognized complication. We report a new case of this association and review the current literature.- - - - - - - - - - ranking = 1keywords = palm (Clic here for more details about this article) |
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