ADULT syndrome: dental features of a very rare condition.

The Acro-Dermato-Ungual-Lacrimal-Tooth syndrome (ADULT syndrome) is one of the rarest ectodermal dysplasias and it is associated with several malformations involving especially the limbs. The most clinical features are the presence of ectrodactyly, syndactyly, hypermelanosis or multiple lentigines, onhycodysplasia, abnormalities in the lacrimal duct, recurrent conjuntivitis, photophobia, mammarian hypoplasia, hypotrichosis and frontal alopecia, hypohydrosis, cutaneous photosensitivity, nasal bridge prominence, exfoliative dermatitis and xerosis. The ectodermal dysfunction expresses itself with conoid teeth, enamel hypoplasia, dentinal dysplasia and especially hypodontia, with following functional and aesthetic defects. We report the case of an 11-year-old Caucasian girl affected by ADULT syndrome.

Genetic correlation between rheumatoid arthritis and periodontal disease: the role of sex and IL-10.

The chronic stimulation of the immune system due to the presence of bacterial antigens within periodontal tissues has been associated with several autoimmune diseases, like diabetes mellitus, infective endocarditis or cardiovascular atherosclerosis. The current study aims at evaluating the correlation between Rheumatoid Arthritis (RA) and Periodontal Disease (PD) with special attention to genetic polymorphisms in cytokine expression. A total number of 34 patients affected by RA were recruited. Each of them underwent haematochemical analysis and data were collected for Rheumatoid Factor (RF), Anti-Citrullinated Protein’s Antibody (CCP) and HLA-BDR1. DAS-28 questionnaire for disease activity was fulfilled by the rheumatologist, while a periodontal examination was carried out by the dental clinician and crevicular fluid samples were collected to evaluate the IL-6, IL-10 and VDR polymorphysms. A connection between CCP and IL-10 polymorphisms was found, with IL-10 expressing protecting tendency against periodontal disease when CCP are found in the bloodstream (p=0.0017). Finally, males mainly expressed IL-10 predisposing genes (p=0.046), while females showed a greater tendency to express RF (p=0.014) and CCP (p=0.050). This paper corroborates the idea of a correlation between sex, IL-10 polymorphisms and RA, which should be studied in depth, since recent papers have shown that IL-10 injected into joints seems to decrease inflammation.

Human ß2-defensin in oral lichen planus expresses the degree of inflammation.

Syndrome (BMS). Seventeen OLP patients, with a positive histopathologic diagnosis of the disease, were recruited into this study in order to measure the relative quantity of HBD-2 in their saliva and crevicular fluid. The values were compared with those collected from a group of 9 patients affected by the Burning Mouth Syndrome (BMS) and with a control group (CTRL) of 9 patients. There was no statistically significant difference between the groups (p=0.523; p=0.897). However, patients affected by OLP showed a dycotomic distribution of values: while 10 of them showed similar values to those found out in the other two groups, 7 patients expressed high levels of HBD-2 and 3500 pg/ml was the threshold to distinguish the subgroups. During the dental visit the clinician classified OLP patients into two groups according to the clinical presentation of the disease: reticular and hyperplastic (white OLP), atrophic and erosive forms (red OLP). There was a statistical significant correlation between the clinical and numeric classification of the patients (p=0.004; p=0.001), and the expression of HBD-2 was higher in the red OLP group than in the white OLP group (p=0.000; p=0.000). In conclusion, this study shows that HBD-2 represents an index to assess active inflammation and it is probably linked to the presence of the typical band-like CD8+ infiltrate in Oral Lichen Planus.

A “burning” therapy for burning mouth syndrome: preliminary results with the administration of topical capsaicin.

Burning mouth syndrome is defined as an intraoral burning sensation for which no medical or dental cause can be found. Recently, researchers have demonstrated an altered trophism of the small nerve fibres and alterations in the numbers of TRPV-1 vanilloid receptors. Capsaicin is a molecule that is contained in hot peppers and is specifically detected by TRPV-1 vanilloid receptors that are distributed in the oral mucosae. We aimed at verifying if topical capsaicin could prove to be an effective treatment of Burning Mouth Syndrome. A group of 99 BMS patients were recruited. We subdivided the BMS patients into two groups: the collaborative patients, who expressed a predominantly neuropathic pattern of symptoms, and the non-collaborative patients, who were characterised by stronger psychogenic patterns of the syndrome. Both groups underwent topical therapy with capsaicin in the form of a mouth rinse 3 times a day for a long period. After 1 year of treatment, the final overall success rate was approximately 78%, but with a significant difference in the success rates of the two groups of patients (87% and 20% among the collaborative and non-collaborative patients, respectively; p=0.000). The use of topical capsaicin can improve the oral discomfort of BMS patients, especially during the first month of therapy, but it is more effective for those patients in which the neuropathic component of the syndrome is predominant. Our hypothesis is that chronic stimulation with capsaicin leads to decreases in burning symptoms. This phenomenon is called desensitisation and is accompanied by substantial improvements in oral symptoms.

Periodontal microbioma and rheumatoid arthritis: The role of Porhyromonas gingivalis.

Rheumatoid Arthritis is a disease, which can be described as an autoimmune response after molecular mimicry caused by infective agents. The current study aims at evaluating the correlation between Rhematoid Arthritis (RA) and Periodontal Disease (PD), with special attention to the microbioma detected in the gums. Thirty-four patients with RD were recruited into the current study. Among rheumatic parameters, Rheumatoid Factor (RF), anti-citrullinated protein antibody (CCP), HLA-BDR1 and DAS28 were collected. A dental clinician evaluated the periodontal screening record (PSR). Afterwards, 1 paper cone was inserted for 30 seconds into the gingival sulcus then sent to the laboratory for evaluation. Quantitative PCR of 16S rRNA genes was performed with the hydrolysis probes method to identify and evaluate the amount Porphyromonas gingivalis, Tannerella forsythensis, Treponema denticola, Fusobacterium nucleatum and Campylobacter rectus. There were no statistical differences in the composition of oral microbioma between PSR groups. There were no statistical significant differences between bacterial loads and serum values. On the contrary, a positive correlation was found between the presence of Porphyromonas gingivalis in periodontal pockets on one side and RF and CCP on the other. Therefore, the presence of Porhyromonas gingivalis in periodontal pockets is associated to RA inflammatory indices.

Oral manifestations of selective IgA-deficiency: review and case-report.

Immunoglobulin A deficiency is the most common primary immunodeficiency defined as decreased serum level of IgA (less than 7 mg/dl) in the presence of normal levels of other immunoglobulin isotypes. Most individuals with IgA deficiency are asymptomatic and identified coincidentally. However, some patients may present with recurrent infections, allergic disorders and autoimmune manifestations, such as diabetes mellitus, Graves disease and celiac disease. The international literature has not produced any kind of review yet about intra-oral manifestations of selective IgA-deficiency. L.S., a 7-year-old Caucasian girl, was examined at our hospital. After she had undergone a professional dental cleaning, a symmetric, bilateral ulcerative gingivitis developed nearby the upper second primary molars. The gingival ulcers were persistent and did not disappear in the following 3 weeks. In the meantime, the young patient reported the presence of gastrointestinal symptoms. IgA serum level was 4.5 mg/dl, while the other isotypes levels were in the common range. The diagnosis of selective IgA-deficiency was formulated and the girl underwent further examination for the specific IgG autoantibodies in celiac disease, which were not present. Consequently, a full prevention program was planned. This case report emphasizes the role of the paediatric dentist in the early detection of systemic disorder, such as the immunological diseases. The oral cavity often reveals to be the first site of manifestation of important systemic diseases. Immunoglobulin A (IgA) deficiency is the most common primary immunodeficiency and is defined as a decrease in serum IgA levels in the presence of normal levels of other immunoglobulin isotypes (1). Serum IgA deficiency was first described in children with ataxia-telangiectasia (2) and has since been identified in other patients, including normal patients. The prevalence of IgA deficiency ranges from 1:223 to 1:1000 in community studies and from 1:400 to 1:3000 in healthy blood donors (3).

The “elfin face”: craniofacial and dental aspects of the Williams-Beuren syndrome.

Williams Syndrome is a rare congenital disorder characterized by supravalvular aortic stenosis, peripheral pulmonary artery stenosis, mental retard and dysmorfic facial features. As regards the dental aspects of the syndrome, the deletion of the elastin gene induced clinicians to suspect periodontal alterations with a greater frequency of gingivo-periodontitis, but on the contrary no association between the syndrome and periodontal diseases have been found. Furthermore, patients show a higher frequency of teeth hypoplasia, an abnormal tooth morphology during primary dentition (12.5%) and during permanent dentition. We present a case report of a 12-year-old Caucasian boy affected by Williams-Beuren Syndrome who visited our hospital for a dental and orthodontic evaluation.

Helicobacter pylori in periodontal pockets and saliva: a possible role in gastric infection relapses.

It has been a long time since the scientific community started to speculate upon the presence of Helicobacter pylori (HP) in periodontal pockets as an extra-gastric reservoir responsible for gastric relapses after eradication therapy. The aim of this study is to evaluate the presence of oral HP in a group of patients who underwent examination for gastric infection. Sixty patients were enrolled in the current study, subdivided into two groups: 30 patients with a positive result for HP gastric infection with C-Breath Test Urea examination, and 30 patients with a negative result for HP gastric infection. Crevicular fluid and salivary samples were collected in a sterile tube and then sent to the laboratory for evaluation. Specimens were processed to quantify the levels of HP and bacterial load by real time PCR technique. Even though there was no statistically significant difference among the two groups (A vs B) with regard to the total amount of HP in saliva or in periodontal tissues, this study demonstrates that the oral cavity is an extra-gastric reservoir of HP when it is affected by periodontal disease, and that periodontal disease is correlated to gastric HP infection.