Microbiological features of Papillon‐Lefèvre syndrome periodontitis

Abstract

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-Lefèvre 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-Lefèvre 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 rôles in the development of Papillon-Lefèvre syndrome periodontitis.

Role of Polymorphonuclear Leukocyte-Derived Serine Proteinases in Defense againstActinobacillus actinomycetemcomitans

“…The microbiological status of PLS patients suggests that a majority of them are infected with A. actinomycetemcomitans (1,5,7,13,17,18,20,22,33,41,42,46,53,60,67,70,71,74,75,76). This is in line with our finding that their resistance to this pathogen may be compromised.…”

“…As in other forms of periodontitis, PLS patients seem to have increased susceptibility to infections with periodontitisassociated pathogens such as A. actinomycetemcomitans (1,5,7,13,17,18,20,22,33,41,42,46,53,59,60,67,70,74,75,76). This facultative anaerobic gram-negative pathogen produces virulence factors to promote its colonization and survival (61).…”

Papillon Lefevre Syndrome: a case report with review

“…Beside genetic predisposition, certain environmental factors like viral and bacterial infections of actinobacillus actinomycetemcomitans, capnocytophaga, cytomegalo and Epstein Barr virus etc also play an important role in pre pubertal periodontitis of PLS patients [23][24][25].…”

Syndromes That Include Both Palmoplantar Keratoderma And Severe Periodontitis: A Review

“…Several patients, however, were positive for other periodontal pathogens [19][20][21]. PLS-periodontitis was also reported to be associated with human herpesviruses [22].…”