Microbiological and immunohistological findings in a patient with Papillon‐Lefére syndrome

Kleinfelder, J. W.; Topoll, Heinz; Preus, Hans R.; Müller, R. F.; Lange, Dieter; Böcker, W.

doi:10.1111/j.1600-051x.1996.tb00533.x

Cited by 35 publications

(36 citation statements)

References 31 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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confidence: 99%

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

et al. 2006

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“…Bu araşt›rmalarda, periodontal ceplerden elde edilen subgingival plak örneklerinde Actinobacillus actinomycetamcomitans'a ek olarak (11,14,(29)(30)(31)(32)(33), Porphyromonas gingivalis, Fusobacterium nucleatum, Bacteroides forsythus, Treponema denticola ve Prevotella intermedia gibi diğer putatif periodontopatojenlerin de PLS ile ilişkili periodontitis patogenezinde rol oynad›ğ› ortaya konmuştur (11,34,35). Ancak spesifik bakteriyel profile ilişkin bulgular, Lundgren ve ark.…”

Section: Discussionunclassified

“…Önceki dönemlerde yap›lm›ş vaka raporlar›, bu vakalarda oral hijyen eğitimi, diş yüzey temizliği, kök yüzey düzleştirmesi ile küretaj gibi cerrahi olmayan tedavinin ve hatta buna ek sistemik antibiyotik uygulamas› ve tüm dişlerin çekiminin de yer ald›ğ› cerrahi işlemleri kapsayan periodontal tedavinin başar›s›z olabildiğini ve sendromun bir parças› olan tam dişsizlikle sonuçlanabildiğini öne sürmüşlerdir (12)(13)(14)(15). Araşt›rmalar›n sonuçlar› periodontal tedavideki başar›n›n çok değişken olduğuna dikkati çekmekte ve prognozun bireye özgü farkl›l›klar sergilediğini desteklemektedir (3).…”

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Papillon-Lefevre Sendromu İle İli̇şki̇li̇ Peri̇odonti̇ti̇s Olgusu: 1 Yillik Taki̇p

Özçelik¹,

Haytaç²

2008

Ankara Üniversitesi Diş Hekimliği Fakültesi Dergisi

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Papillon-Lefevre Syndrome (PLS) is a rare autosomal recessive disease with a very low prevalence. Characteristic symptoms are diffuse palmar and plantar hyperkeratosis combined with a rapidly progressive periodontal breakdown affecting both dentitions, resulting in premature loss of deciduous and permanent teeth. This paper reports the treatment planning of the severely affected periodontal component of a patient with Papillon-Lefevre Syndrome.Clinical examination which included conventional periodontal measurements and radiographic analysis showed severe inflamation and bone loss. Total blood count, neutrophil function tests and alkaline phosphatase, CD 2, 3, 4, 5, 8, 11b, 16, HLA-DR and immunoglobulin analysis were made. Occurence of major suspected periodontopathic bacteria which included Porphyromonas gingivalis, Bacteriodes forsythus and Trepenoma denticola was determined by BANA (N-benzoyl-DL-argininenaphthylamide) test. As the result of clinical, immunological, microbiological and hematological findings, the patient was diagnosed as having PLS. The treatment consisted of extraction of all primary teeth and systemic antibiotic therapy. After an edentulous period complete dentures were inserted 3 months after the extractions. The patient is still under control after one year.

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“…Furthermore, a "massive occurrence of A actinomycetemcomitans" has been noted in periodontal pockets in PLS, although the same species was present also in the mouths of siblings and a parent without PLS. 42 Similarly, in a group of 12 Saudi-Arabian adolescents with PLS, there was no PLS specific profile of the subgingival infection because the bacterial composition resembled that characterising deep pockets in adult patients with periodontitis. 43 It is possible that recognised periodontal pathogens might be involved in PLS.…”

Section: Discussionmentioning

confidence: 99%

A microbiological study of Papillon-Lefevre syndrome in two patients

Robertson

Drucker²,

James³

et al. 2001

Journal of Clinical Pathology

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Aim-To analyse the microflora of subgingival plaque from patients with PapillonLefèvre syndrome (PLS), which is a very rare disease characterised by palmarplantar hyperkeratosis with precocious periodontal destruction. Methods-Bacterial isolates were identified using a combination of commercial identification kits, traditional laboratory tests, and gas liquid chromatography. Some isolates were also subjected to partial 16S rDNA sequencing. Plaque samples were also assayed for the presence of Porphyromonas gingivalis, Prevotella intermedia, and Actinobacillus actinomycetemcomitans in a quantitative enzyme linked immunosorbent assay (ELISA) using monoclonal antibodies. Results-The culture results showed that most isolates were capnophilic and facultatively anaerobic species-mainly Capnocytophaga spp and Streptococcus spp. The latter included S constellatus, S oralis, and S sanguis. Other facultative bacteria belonged to the genera gemella, kingella, leuconostoc, and stomatococcus. The aerobic bacteria isolated were species of neisseria and bacillus. Anaerobic species included Prevotella intermedia, P melaninogenica, and P nigrescens, as well as Peptostreptococcus spp. ELISA detected P gingivalis in one patient in all sites sampled, whereas A actinomycetemcomitans was detected in only one site from the other patient. Prevotella intermedia was present in low numbers. Conclusions-Patients with PLS have a very complex subgingival flora including recognised periodontal pathogens. However, no particular periodontopathogen is invariably associated with PLS.

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Microbiological and immunohistological findings in a patient with Papillon‐Lefére syndrome

Cited by 35 publications

References 31 publications

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

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

Papillon-Lefevre Sendromu İle İli̇şki̇li̇ Peri̇odonti̇ti̇s Olgusu: 1 Yillik Taki̇p

A microbiological study of Papillon-Lefevre syndrome in two patients

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