Systemic retinoid medication and periodontal health in patients with Papillon-Lefèvre syndrome

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.</p

Saliva composition in children and young adults with Papillon-Lefèvre syndrome

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 &lt; 0.01) and stimulated (p &lt; 0.05) saliva secretion rates were significantly lower among the PLS patients compared with the controls. Furthermore salivary buffer capacity was significantly (p &lt; 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 &lt; 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.</p