Periodontal health in relation to systemic retinoid medication was evaluated retrospectively in patients with Papillon-Lefèvre Syndrome (PLS). The material consisted of 18 children/young adults ranging from 8 to 28 years of age, all with a confirmed diagnosis of PLS. 9 participants, comprising a medication group, had been on long-term (range 1.5-9 years) retinoid medication for their cutaneous lesions. The remaining 9 served as controls. Regardless of whether or not retinoid medication was received, every patient experienced an early and devastating periodontitis, with atypical edematous and erythematous gingiva, suppuration from deep gingival pockets and premature loss of teeth. No correlation could be found between the severity of skin involvement and the severity of periodontal involvement. An improvement with age could be seen for the cutaneous lesions but not for the periodontal condition. Systemic medication with retinoids had a favorable therapeutic effect on cutaneous lesions, and no severe complication/side effect could be seen after several years of continuous use. However, from the results of this study it can be concluded that, at least in a situation with poor compliance of daily oral home-care, no positive effect on the periodontal health in patients with PLS could be seen by the retinoid medication.

BACKGROUND/AIM: Conventional mechanical periodontal treatment of Papillon-Lefevre syndrome (PLS) has often been reported to fail. This study describes the outcome of a non-surgical periodontal therapy including antimicrobial treatment of nine patients diagnosed with PLS. The patients originate from a total of 15 children and adolescents with PLS for which clinical characteristics are presented.

METHODS: Clinical examination including conventional periodontal measurements. Initial treatment including oral hygiene instruction, scaling and root planing and systemic amoxicillin-metronidazole therapy for 6 weeks. After that the patients were enrolled in a 3-month recall maintenance program. In addition to this mechanical supportive maintenance treatment, tetracycline was prescribed and used continuously for 1.5 years.

RESULTS/CONCLUSION: On five patients who were showing acceptable standard of oral hygiene and also compliance with the antibiotic medication, development of periodontitis on erupting teeth was prevented and disease activity on the previously periodontally involved teeth controlled during a 3-year period. Poor results of treatment were observed for three patients, all siblings. These patients failed to comply with the medication and also failed to improve their oral hygiene.

The prevalence of 18 selected bacterial species was assessed by means of "checkerboard" DNA-DNA hybridisation in a group of 12 Saudi-Arabian adolescents with Papillon-Lefèvre syndrome. A total of 36 tooth sites were investigated. The patients exhibited severe periodontal disease with deep pockets. All 12 patients harboured the putative bacterial pathogens P. intermedia, F. nucleatum, P. micros and S. intermedius while T. denticola, B. forsythus, P. nigrescens, E. corrodens, S. noxia and C. rectus were recovered from 11 patients. P. gingivalis was recovered from 9 patients and 18 sites while corresponding figures for A. actinomycetemcomitans were 8 and 19, respectively. A number of the investigated species (B. forsythus, T. denticola, P. intermedia, C rectus) reached high levels (> or =10(6) cells) in more than 1/2 of the patients. On the other hand, bacteria such as A. actinomycetemcomitans and P. gingivalis were infrequently encountered at high levels in these subgingival samples. In conclusion, the analysis failed to demonstrate a PLS-specific profile of the subgingival infection, since the bacterial composition of the sampled sites closely resembled that characterising deep pockets in adult periodontitis patients.

Papillon-Lefèvre syndrome (PLS), palmoplantar hyperkeratosis with periodontitis, has been genetically characterized. However, suspected associated immune dysfunctions remain elusive. The purpose of this study was to evaluate peripheral blood lymphocyte levels and natural killer (NK) cell cytotoxicity in PLS. Twenty patients and 20 healthy controls were examined. Peripheral blood lymphocytes were analyzed by flow cytometry for surface markers. NK cell cytotoxicity against K562 cells was determined by means of a 51Cr release assay. White blood cell differential and proportions of B-, T-, T-helper, T-suppressor, and NK cells revealed only sporadic borderline variations from control values. In contrast, NK cell cytotoxicity was consistently and severely depressed (32-53% of control values) in all patients. To the best of our knowledge, this newly described impairment of NK cell cytotoxic function is the first consistent immune dysfunction reported in PLS. This suggests that the impaired NK cell cytotoxicity might contribute to the pathogenesis of PLS-associated periodontitis.

The aim of the present study was to evaluate the salivary secretion rate and composition in a group of 16 children and young adults (6-27 years) with Papillon-Lefèvre Syndrome (PLS), and to compare the findings with a group (n = 16) of healthy controls. Unstimulated and stimulated whole saliva was collected at least 2 h after meals and the secretion rate determined. The stimulated saliva was assessed for buffer capacity, total protein, peroxidase and hexosamine, while the unstimulated samples were evaluated for total protein, lysozyme, thiocyanate, lactoferrin and salivary IgA. Both the unstimulated (p < 0.01) and stimulated (p < 0.05) saliva secretion rates were significantly lower among the PLS patients compared with the controls. Furthermore salivary buffer capacity was significantly (p < 0.01) lower in the PLS patients. The total protein content in saliva was comparatively high in the study group, while the concentrations of immunoglobulins and non-immunoglobulins were within normal ranges. When calculating the output of the assessed antimicrobial factors, the mean peroxidase level in stimulated whole saliva was found to be significantly (p < 0.01) lower in the PLS patients than in the healthy controls. In conclusion, the present study indicates an impaired water secretion and a somewhat altered saliva gland function in children and young adults with PLS.