Vestibular involvement of PFAPA syndrome.

OBJECTIVE: PFAPA syndrome is derived from the initials of the English words of the findings that make up the syndrome ("Periodic Fever", "Aphthous Stomatitis", "Pharyngitis", "Adenitis"). This study aims to evaluate the vestibular system in patients with PFAPA syndrome by the cVEMP test and to give a general review of PFAPA syndrome in light of current literature. METHODS: In this prospective study, 30 patients aged 4-6 who were diagnosed with PFAPA in a tertiary pediatrics clinic, between January 2016 and February 2020 and 30 children of the same age group who applied to a tertiary otorhinolaryngology clinic for other reasons and proven to have no hearing or vestibular problems were included and in addition to routine physical examination, electromyographic activity of the sternocleidomastoid muscle surface was measured. RESULTS: We found that the amplitude difference between cVEMP p1-n1 in patients with PFAPA syndrome in both ears decreased compared to the healthy control group. CONCLUSION: Our study proves there is a vestibular system involvement of PFAPA syndrome. This study is the first in the literature to search the relationship between PFAPA and the vestibular system.

Effect of different treatments on recurrent aphthous stomatitis: laser versus medication.

Recurrent aphthous stomatitis (RAS) is a common disease with ulcers in oral cavity which may trigger chewing, speaking, and swallowing difficulties to patients. Treatment of RAS is primarily aimed at pain relief and the promotion of wound healing. However, few agents have been found to have definite effect in the management of RAS and most of the medicinal products may cause adverse reactions or other disadvantages, which makes their clinical usage questionable. The purpose of this randomized controlled clinical trial (RCT) was to assess the clinical effect of diode laser and traditional medication treatment on RAS. In this study, 56 patients were randomly assigned to two groups (n = 28). Laser group was treated using diode laser (810 nm, 1.0 W, CW, irradiation time 20 s for 3 applications) once daily for continuous 3 days. Medication group was treated with triamcinolone acetonide 0.1% three times a day until the lesion was healed. Spontaneous and functional pain level on the third day of treatment was significantly less in the laser group. Significant difference was observed with respect to healing time; however, the order of difference is small albeit of statistical significance. Diode laser with the chosen parameters had better effects on pain relief and no distinct advantage on wound healing comparing with medication. Trial registration number: ChiCTR2000030298; date of registration: 26 February 2020 (retrospectively registered).

Common benign and malignant oral mucosal disease.

BACKGROUND: Mucosal diseases of the oral cavity are relatively common, and patients often seek initial assessment from their general practitioner. OBJECTIVE: The aim of this article is to provide an overview of common oral mucosal diseases to help with formulating a differential diagnosis and stratifying the urgency of referral. DISCUSSION: Pathological mucosal conditions of the oral cavity and jaws commonly present as a mucosal ulcer or a white, red or pigmented lesion. In this review, the authors outline the most common conditions organised according to their clinical presentation and describe their typical appearance and management.

Consensus treatment plans for periodic fever, aphthous stomatitis, pharyngitis and adenitis syndrome (PFAPA): a framework to evaluate treatment responses from the childhood arthritis and rheumatology research alliance (CARRA) PFAPA work group.

BACKGROUND: Periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome is the most common periodic fever syndrome in children. There is considerable heterogeneity in management strategies and a lack of evidence-based treatment guidelines. Consensus treatment plans (CTPs) are standardized treatment regimens that are derived based upon best available evidence and current treatment practices that are a way to enable comparative effectiveness studies to identify optimal therapy and are less costly to execute than randomized, double blind placebo controlled trials. The purpose of this project was to develop CTPs and response criteria for PFAPA. METHODS: The CARRA PFAPA Working Group is composed of pediatric rheumatologists, infectious disease specialists, allergists/immunologists and otolaryngologists. An extensive literature review was conducted followed by a survey to assess physician practice patterns. This was followed by virtual and in-person meetings between 2014 and 2018. Nominal group technique (NGT) was employed to develop CTPs, as well as inclusion criteria for entry into future treatment studies, and response criteria. Consensus required 80% agreement. RESULTS: The PFAPA working group developed CTPs resulting in 4 different treatment arms: 1. Antipyretic, 2. Abortive (corticosteroids), 3. Prophylaxis (colchicine or cimetidine) and 4. Surgical (tonsillectomy). Consensus was obtained among CARRA members for those defining patient characteristics who qualify for participation in the CTP PFAPA study. CONCLUSION: The goal is for the CTPs developed by our group to lead to future comparative effectiveness studies that will generate evidence-driven therapeutic guidelines for this periodic inflammatory disease.

Performance of recently proposed periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome criteria in a region endemic for familial Mediterranean fever.

The periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome is an auto-inflammatory condition characterized by recurrent episodes of fever accompanied by aphthosis, cervical adenitis, and pharyngitis. Diagnosis of PFAPA could be challenging due to clinic overlap with familial Mediterranean fever (FMF). An international consensus has been established recently, to define a new set of classification criteria for PFAPA syndrome. We aimed to evaluate the performance of recently proposed PFAPA criteria, to assess their utility in FMF regions. Patients diagnosed with PFAPA syndrome, FMF, and juvenile idiopathic arthritis (JIA) were included. Two investigators blindly evaluated all of patients for the newly proposed PFAPA criteria. A total of 542 patients (322 with PFAPA syndrome, 118 FMF and 102 JIA) were evaluated. Mean age of patients was 6.6 ± 2.81, 12.75 ± 3.9, and 12.42 ± 4.8 years for PFAPA, FMF, and JIA, respectively. We found quite high sensitivity (89.7%) but insufficient specificity of newly proposed PFAPA criteria (69.5%). When applied to control patients separately, specificity was found to be 61% and 79.4% for FMF and JIA patients, respectively. Positive predictive value was 81%, while negative predictive value was 82%. Recently proposed PFAPA criteria have satisfactory sensitivity, but its specificity is still under expectation. There is a need for a distinctive criterion between PFAPA syndrome and FMF, in FMF endemic regions, e.g., cryptic tonsillitis rapidly responsive to single dose of glucocorticoids. Further studies with higher patients' number in different regions are needed.

The influence of oral health and psycho-social well-being on clinical outcomes in Behçet's disease.

This study was designed to investigate the association of oral ulceration and oral health factors, together with psycho-social well-being in Behçet's disease (BD), and to clarify the importance of psycho-social support of patients in the overall management of BD. The study comprised of a cohort of 146 BD patients (mean age ± SD = 39.65 ± 13.20) and 20 recurrent aphthous stomatitis (RAS) patients (mean age ± SD = 42.32 ± 11.32). Oral ulcer severity score (OUSS), Behçet's disease current activities form (BDCAF), hospital anxiety and depression scale (HADS), and the work and social adjustment scale (WSAS) were investigated. Oral health risk factors were also included. The analysis of variance, regression, and factor analysis were used to scrutinise the data. Almost 73% of patients were at high caries risk in BD and RAS groups. Thirty-nine percent of BD and forty percent of RAS had a score of BPE3 (probing depth 3.5-5.5 mm). Regression analysis revealed that OUSS and WSAS had a positive impact to increase the BDCAF score in BD patients (ß = 0.395, P = 0.001; ß = 0.240, P = 0.019), respectively. Dental health, periodontal health, anxiety, depression, and WSAS variables had strong loadings by factor analysis based on gender and at the time of present and absent of oral ulceration. The main oral ulcer characteristics that had significant influences on the total of oral health quality of life by 68.6% were: size, duration, ulcer-free period, and pain. The results highlighted the significant influence of oral ulceration, patients' oral health, diet, and psycho-social well-being as multi-factorial causes on increasing disease activity in BD patients.

Oral pathology in inflammatory bowel disease.

The incidence of inflammatory bowel diseases (IBD) - Crohn's disease (CD) and ulcerative colitis (UC) - has been increasing on a global scale, and progressively, more gastroenterologists will be included in the diagnosis and treatment of IBD. Although IBD primarily affects the intestinal tract, extraintestinal manifestations of the disease are often apparent, including in the oral cavity, especially in CD. Specific oral manifestations in patients with CD are as follows: indurate mucosal tags, cobblestoning and mucogingivitis, deep linear ulcerations and lip swelling with vertical fissures. The most common non-specific manifestations, such as aphthous stomatitis and angular cheilitis, occur in both diseases, while pyostomatitis vegetans is more pronounced in patients with UC. Non-specific lesions in the oral cavity can also be the result of malnutrition and drugs. Malnutrition, followed by anemia and mineral and vitamin deficiency, affects the oral cavity and teeth. Furthermore, all of the drug classes that are applied to the treatment of inflammatory bowel diseases can lead to alterations in the oral cavity due to the direct toxic effects of the drugs on oral tissues, as well as indirect immunosuppressive effects with a risk of developing opportunistic infections or bone marrow suppression. There is a higher occurrence of malignant diseases in patients with IBD, which is related to the disease itself and to the IBD-related therapy with a possible oral pathology. Treatment of oral lesions includes treatment of the alterations in the oral cavity according to the etiology together with treatment of the primary intestinal disease, which requires adequate knowledge and a strong cooperation between gastroenterologists and specialists in oral medicine.

Recurrent aphthous stomatitis (RAS): a preliminary within-subject study of quality of life, oral health impacts and personality profiles.

BACKGROUND: RAS may affect quality of life and impacts oral health and daily activities; consequently, psychological factors, dental needs and patients' perceptions. Professionals should understand this relation to adequately manage oral ulcers. This study aimed at investigating the relationship between oral health impacts, oral health-related quality of life and psychological profiles in patients with recurrent aphthous stomatitis (RAS). METHODS: Fifty-three patients (30 men and 23 women, mean age: 31.1 ± 10.1 years) with RAS participated in this study. During ulcer episodes, participants completed Hospital Anxiety and Depression Scale (HAD), Neuroticism-Extraversion-Openness Five Factor Inventory (NEO-FFI), Oral Health Impact Profile (OHIP-14) and United Kingdom Oral Health-Related Quality of Life measure (OHQoL-UK) proformas. Patients completed OHIP-14 and OHQoL-UK again when they were ulcer-free. The statistically significance levels were set at P ≤ 0.05. RESULTS: During ulcer episodes, patients reported worse oral health impacts and an inferior quality of life in comparison with ulcer-free times (P < 0.0001). Higher OHIP scores were significantly associated with higher HAD depression (P = 0.036) and anxiety (P = 0.012) scores. NEO-FFI scores had no significant relations with OHIP and OHQoL-UK scores (P > 0.05). During ulcer-free periods, no relationships were detected between OHIP, OHQoL-UK, HAD and NEO-FFI scores (P > 0.05), except between OHQoL-UK and conscientiousness scores (P = 0.05). CONCLUSIONS: RAS increased the negative oral health impacts on patients and consequently lowered their quality of life. Stressful situations and conditions (including anxiety and depression), rather than inherent personality profiles and stable psychological traits, were related to oral health impacts and quality of life in patients with RAS.

Palatal ulceration.

Palatal ulcers are a common presentation and can be conveniently divided into developmental and acquired causes, the latter of which is subdivided into acute and chronic causes. Most commonly seen dermatologic causes have associated skin manifestations. Acute and multiple ulcers are usually infectious or drug induced in origin. Recurrent ulcers are largely dominated by aphthosis, while chronic ulcers are seen in immunocompromised patients and can occasionally be malignant. It is essential to involve the oral and maxillofacial surgeons early in the therapeutic management to tackle the inevitable complications that may ensue in the chronic cases.

Mean platelet volume in recurrent aphthous stomatitis and Behçet disease.

Behcet disease (BD) and recurrent aphthous stomatitis (RAS) are systemic inflammatory diseases, but the exact pathogenesis of both the diseases is unknown. Mean platelet volume (MPV) is an indicator of platelet activation. The aim of this study was to investigate the MPV levels in patients with BD, RAS, and healthy participants. A total of 61 patients with BD, 60 patients with RAS, and 60 healthy controls were included in this study. The MPV levels and erythrocyte sedimentation rate in patients with BD and RAS groups were significantly higher than the control groups (P < .001). In the BD group as well as in the RAS group, the disease activity does not affect the levels of MPV. The MPV levels may be used as a cheap and feasible diagnostic marker in patients with BD and RAS. Nevertheless, the MPV does not have a predictive value in differentiating the diagnosis of BD and RAS.

Anamnestic findings from patients with recurrent aphthous stomatitis.

Recurrent aphthous stomatitis (RAS) is a common oral disorder with a prevalence varying between 5% and 66%. RAS appears in three forms; minor, major and herpetiform. The aetiology is unknown.The aim of this study was to evaluate associations between specific anamnestic information and different types of recurrent aphthous stomatitis (RAS). A group of 177 patients (mean age = 42.8 years; SD = 14.3; range 17-79 years) participated. Data were collected from a structured interview, consisting of 22 questions. Information about i) health status and medication, ii) predisposing factors, iii) RAS experience, iv) previous treatment methods and v) brand of toothpaste was collected. Sixty-eight per cent of the patients were healthy and 44% of the patients were not taking any medication. Forty-one per cent of the patients did not have any apprehension of the reason for their RAS, while stress (15.8%) was the most common apprehended aetiological factor. Sixty-two per cent had one to three minor ulcers at one time. Forty-eight per cent reported having had a major aphthous ulcer at least once.The most frequent symptom reported was pain (53.7%), followed by a smarting sensation (18.6%) and tenderness (4%). The most common treatment for RAS was Zendium™ toothpaste/mouthrinse (28%), followed by corticosteroids (25%). Fifty-four per cent of the patients experienced no relief from the treatment. When toothpaste habits were investigated, Zendium™ was used by 32% of the patients and toothpaste containing sodium-lauryl-sulfatase was used by 32%.There was no positive correlation between the use of Zendium™ toothpaste and the relief of symptoms or the size, number or frequency of the aphthous ulcers. Sixty-four per cent of the patients had never smoked, while 7% were smokers. No positive correlation was found when age, gender, allergy, medication and smoking were correlated to the frequency, number and size of the aphthous ulcers. In conclusion, we found that the aetiology behind RAS is still unclear and probably multifactorial. Standard treatment methods like Zendium™ should perhaps be questioned and this study did not find any support for smoking as a "protective" factor, i.e. having less likelihood of experiencing major problems from RAS.

Association of aphthous ulcers with self-reported symptoms of depression in a sample of smartphone users.

BACKGROUND: Our goal is to examine the association of recurrent aphthous stomatitis (RAS) with symptoms of depression using a smartphone-based questionnaire survey. METHODS: An electronic survey was administered through a smartphone app asking respondents about current depressive symptoms using the Quick Inventory of Depressive Symptoms (QIDS), and asking whether they had ever or recently experienced RAS. Multivariate logistic regression analysis was used to determine associations. RESULTS: A total of 478 individuals completed the survey, with 64% reporting a lifetime prevalence of RAS, and 21% experiencing an aphthous ulcer within the last month. RAS was significantly associated with increased sleep, decreased appetite, low energy, and feeling sluggish. RAS was not associated with overall depression severity as measured by total QIDS score, or with cardinal features of depression such as sadness, insomnia, impaired concentration, self-blame, thoughts of death, or anhedonia. Prevalence of RAS did not differ by age, sex, or smoking status, but was less likely in blacks and Asians compared with whites. CONCLUSIONS: RAS was a common phenomenon in this sample of mostly depressed individuals, and was associated with some neurovegetative symptoms of depression, but not depression severity.

Is there any relationship between recurrent oral aphthous stomatitis and prediabetes?

Periodontal disease has been associated with glycaemia. Recurrent aphthous stomatitis (RAS) is one of the most common oral lesions and is characterized by painful recurrent oral ulcers. However, the exact cause and treatment of RAS is not yet well-known. Furthermore, there is still unknown the relationship between RAS and glycaemia. Prediabetes has shown to have role on worse metabolic profile. However, there is still no data on the relationship between prediabetes and clinical RAS. Therefore, the purpose of this hypothesis is to assess whether the prediabetes aggravate RAS.

Salivary cortisol determination in patients from the Basque Country with recurrent aphthous stomatitis. A pilot study

Objectives: Stress and anxiety are controversial factors involved in the complex pathogenesis of Recurrent Aphthous Stomatitis (RAS). The determination of salivary cortisol is a useful, simple and safe test to detect states of high stress or anxiety. The aim of this study is to check for changes in salivary cortisol levels in patients with RAS during periods of active disease. Study design: A measurement of cortisol employing Enzyme-Linked Immuno Sorbent Assay (ELISA) was carried out in samples of unstimulated saliva from 20 patients with active lesions of RAS and 10 healthy individuals used as controls. Results: Increased levels of salivary cortisol were detected in 3 cases, all of them within the group of patients with RAS. In none of the control group patients the level of salivary cortisol was increased. The mean level of salivary cortisol was 0.64 mg / dl (range 0.2 to 1.62) for patients with RAS and 0.57 mg / dl (range 0.25 to 1.09) for controls. Conclusion: Salivary cortisol levels are not statistically higher in patients with active lesions of RAS (AU)

Haematological parameters and recurrent aphthous stomatitis.

OBJECTIVE: To find out the relationship between recurrent aphthous stomatitis (RAS) with deficiencies of haemoglobin, haematocrit, serum vitamin B12, serum Ferritin and red blood cells (RBC) Folate level. STUDY DESIGN: An analytical cross-sectional study. PLACE AND DURATION OF STUDY: Department of Oral Health Sciences, Shaikh Zayed Federal Postgraduate Medical Complex, Lahore, from February to July 2008. METHODOLOGY: Sixty consecutive subjects with active RAS were taken as the aphthous group; 60 age and gender matched subjects without RAS were as the Non-Aphthous group. Five milliliter blood was taken from both groups to evaluate the levels of serum B12, and RBC Folate through radio immuno assay and serum ferritin with enzyme linked immuno-sorbent assay tests. Complete blood count was carried out to determine the level of haemoglobin and haematocrit in both groups. Proportion of subjects with lower values was compared using 2 text of proportions with significance at p < 0.05. RESULTS: Serum Ferritin (p = 0.001), haematocrit (p < 0.001), RBC Folate (p < 0.001) and serum B12 (p < 0.001) were significantly lower in the RAS group. Combined deficiency state (haemoglobin, serum Ferritin, haematocrit, RBC Folate and serum B12) was identified in 13% (n = 8) RAS patients. CONCLUSION: Frequency of haematinic deficiencies was high in RAS patients. Serum B12 and RBC Folate were significantly low in aphthous group.

PFAPA with facial swelling- a new association?

PFAPA (periodic fever, apthous stomatitis, pharyngitis, cervical adenitis) is a rare condition of unknown cause affecting children. Although the exact etiology is unknown, inflammatory, immunological or genetic causes have been suggested. The diagnosis is made by exclusion of other causes of periodic fever. Although management is essentially symptomatic, single corticosteroid dose, tonsillectomy and Cimetidine has been shown to be associated with resolution of symptoms. Although abdominal pain and genital ulcers have been reported in association with PFAPA, unilateral transient facial swelling has not been previously reported. The authors present a hitherto unreported association of PFAPA with recurrent episodes of unilateral facial swelling.

Symptomatic changes of oral mucosa during normal hormonal turnover in healthy young menstruating women.

INTRODUCTION: Changes in hormonal levels, such as those that occur during puberty, pregnancy, menstruation and menopause, have varying effects on oral cavity. Many researchers have proposed a direct link between changing hormonal status and oral health among females. OBJECTIVES: To study the various symptoms and clinical manifestations of oral cavity during normal course of menstrual cycle in healthy women. METHOD: Our study comprised of forty healthy young women volunteers with normal menstrual cycle of 28 to 30 days. A proper menstrual history was recorded from the study subjects. The entire cycle was divided into four phases comprising of bleeding, proliferative, ovulation and secretory. All the study subjects had a menstrual cycle of 28 to 30 days. Thorough recording of oral discomforts during various phases of the cycle was done during the study period. RESULTS: 30% of study subjects complained of aphthous ulcers, 5% had herpes labialis, 25% of them complained of depression, 8% showed gingival bleeding. CONCLUSION: Complaints, like oral ulcerations, mood variations, recurrent herpetic lesions, gingival bleeding in females during normal menstrual period, are attributed to the role of female sex hormones. CLINICAL SIGNIFICANCE: Lesions, like oral ulcers, recurrent herpetic lesions and increased gingival bleeding, seen in females during normal menstrual periods, could be related to hormonal turnover and therefore treated accordingly.

Impacts of recurrent aphthous stomatitis on quality of life of 12- and 15-year-old Thai children.

PURPOSE: To assess the prevalence and characteristics of oral impacts attributed to recurrent aphthous stomatitis (RAS) in 12- and 15-year-olds Thais. METHODS: A national oral health survey was conducted. Child-OIDP and OIDP indices were used to collect oral impacts in 1,100 12- and 871 15 year olds. RESULTS: RAS-related impacts were reported in 24.7% of 12 and 36.2% of 15 year olds. Girls were more likely than boys to report RAS-related impacts. Among all perceived causes of oral impacts, RAS ranked second for 12 and first for 15 year olds. Among 12 and 15 years olds, 79.8 and 86.8% respectively had impacts on eating, 81.0 and 84.4% on cleaning teeth and 51.7 and 60.3% on emotional stability. For individual children, impacts affected between 1-6 daily performances. Impacts were of 'little' and 'moderate' intensity for 12 and 15 year olds, respectively. RAS-related impacts occurred mostly in combination with impacts from other oral conditions. Combined with other oral conditions, the impacts were worse, in terms of score, intensity and extent, than when RAS occurred alone. CONCLUSIONS: RAS-related impacts were common in 12- and 15-year-old Thai children and mostly affected eating, cleaning teeth and emotional stability. RAS tended to occur with other conditions leading to more severe, more extensive impacts on quality of life.