Combined Mechanical and Antibiotic Periodontal Therapy in a Case of Papillon‐Lefèvre Syndrome

Eickholz, Peter; Kugel, Birgit; Pohl, Sabine; Näher, Helmut; Staehle, Hans Jörg

doi:10.1902/jop.2001.72.4.542

Cited by 38 publications

(56 citation statements)

References 17 publications

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“…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.…”

Section: Discussionsupporting

confidence: 89%

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

et al. 2006

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Periodontitis is a chronic destructive infection of the tooth-supportive tissues, which is caused by pathogenic bacteria such as Actinobacillus actinomycetemcomitans. A severe form of periodontitis is found in PapillonLefèvre syndrome (PLS), an inheritable disease caused by loss-of-function mutations in the cathepsin C gene. Recently, we demonstrated that these patients lack the activity of the polymorphonuclear leukocyte (PMN)-derived serine proteinases elastase, cathepsin G, and proteinase 3. In the present study we identified possible pathways along which serine proteinases may be involved in the defense against A. actinomycetemcomitans. Serine proteinases are capable to convert the PMN-derived hCAP-18 into LL-37, an antimicrobial peptide with activity against A. actinomycetemcomitans. We found that the PMNs of PLS patients released lower levels of LL-37. Furthermore, because of their deficiency in serine proteases, the PMNs of PLS patients were incapable of neutralizing the leukotoxin produced by this pathogen, which resulted in increased cell damage. Finally, the capacity of PMNs from PLS patients to kill A. actinomycetemcomitans in an anaerobic environment, such as that found in the periodontal pocket, seemed to be reduced. Our report demonstrates a mechanism that suggests a direct link between an inheritable defect in PMN functioning and difficulty in coping with a periodontitisassociated pathogen.

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Section: Discussionsupporting

confidence: 89%

“…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).…”

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

et al. 2006

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“…All adult patients (#1, 2, and 7) have reported premature tooth loss of deciduous teeth caused by severe periodontitis. Patient #1 was treated successfully and has retained 26 permanent teeth up to now [7,32]. Patients 2 and 7 were edentulous or had left only very few remaining permanent teeth at the time point of examination.…”

Section: Resultsmentioning

confidence: 99%

“…Some case reports described favorable results after early extraction of all erupted teeth, followed by an edentulous period, to prevent subsequent infection of the non-erupted teeth [10,11,[35][36][37]. However, successful cases in which A. actinomycetemcomitans had either not been detected before treatment or had been eliminated by treatment have also been described that did not include large-scale extractions [7,9,32,[38][39][40]. Success of periodontal therapy in PLS may be related to infection with A. actinomycetemcomitans and its elimination, respectively.…”

Section: Discussionmentioning

confidence: 99%

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Cytokine production by leukocytes of Papillon–Lefèvre syndrome patients in whole blood cultures

et al. 2011

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Papillon-Lefèvre syndrome (PLS) is characterised by aggressively progressive periodontitis combined with palmo-plantar hyperkeratosis. It is caused by "loss of function" mutations in the cathepsin C gene. The hypothesis behind this study is that PLS patients' polymorphonuclear leukocytes (PMNs) produce more proinflammatory cytokines to compensate for their reduced capacity to neutralize leukotoxin and to eliminate Aggregatibacter actinomycetemcomitans. Production of more interleukin (IL)-8 would result in the attraction of more PMNs. The aim of this study was to evaluate the cytokine profile in PLS patients' blood cultures. Blood was sampled from eight PLS patients (one female) from six families (antiinfective therapy completed: six; edentulous: two) with confirmed cathepsin C mutations and deficient enzyme activity. Nine healthy males served as controls. Whole blood cultures were stimulated with highly pure lipopolysaccharide (LPS) from Escherichia coli R515 and IL-1β plus tumor necrosis factor (TNF)-α. Thereafter, release of IL-1β (stimulation: LPS and LPS plus adenosine triphosphate), IL-6, IL-8, interferon-inducible protein (IP)-10, and interferon (IFN)-γ (stimulation: LPS, IL-1β/TNFα) were detected by ELISA. Medians of cytokine release were, with the exception of IP-10, slightly higher for PLS than for controls' cultures. None of these differences reached statistical significance. Increased production of IL-1β, IL-6, IL-8, IP-10, or IFNγ as a significant means to compensate for diminished activity and stability of polymorphonuclear leukocyte-derived proteases could not be confirmed in this study. Cytokine profiles in blood cultures may not be used to identify PLS patients.

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Disorders of Keratinization

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McLean²,

Munro³

2004

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Combined Mechanical and Antibiotic Periodontal Therapy in a Case of Papillon‐Lefèvre Syndrome

Abstract: Eradication (suppression beneath detection levels) of A. actinomycetemcomitans seems to play a significant role in the successful treatment of localized prepubertal periodontitis in PLS.

Cited by 38 publications

References 17 publications

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

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

Cytokine production by leukocytes of Papillon–Lefèvre syndrome patients in whole blood cultures

Disorders of Keratinization

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