Validation of an Oral Disease Severity Score (ODSS) tool for use in oral mucous membrane pemphigoid.

BACKGROUND: Mucous membrane pemphigoid (MMP) is a rare autoimmune bullous disease predominantly affecting the oral mucosa. Optimal management relies upon thorough clinical assessment and documentation at each visit. OBJECTIVES: The primary aim of this study was to validate the Oral Disease Severity Score (ODSS) for the assessment of oral involvement in MMP. We also compared its inter- and intraobserver reliability with those of the oral parts of the Mucous Membrane Pemphigoid Disease Area Index (MMPDAI), Autoimmune Bullous Skin Disorder Intensity Score (ABSIS) and Physician's Global Assessment (PGA). METHODS: Fifteen patients with mild-to-moderately severe oral MMP were scored for disease severity by 10 oral medicine clinicians from four U.K. centres using the ODSS, the oral sections of MMPDAI and ABSIS, and PGA. Two clinicians rescored all patients after 2 h. RESULTS: In terms of reliability, the interobserver ODSS total score intraclass correlation coefficient (ICC) was 0·97, MMPDAI activity 0·59 and damage 0·15, ABSIS total 0·84, and PGA 0·72. The intraobserver ICCs (two observers) for ODSS total were 0·97 and 0·93; for MMPDAI activity 0·93 and 0·70 and damage 0·93 and 0·79; for ABSIS total 0·99 and 0·94; and for PGA 0·92 and 0·94. Convergent validity between ODSS and MMPDAI was good (correlation coefficient 0·88). The mean ± SD time for completion of ODSS was 93 ± 31 s, with MMPDAI 102 ± 24 s and ABSIS involvement 71 ± 18 s. The PGA took < 5 s. CONCLUSIONS: This study has validated the ODSS for the assessment of oral MMP. It has shown superior interobserver agreement over MMPDAI, ABSIS and PGA, and superior intraobserver reliability to MMPDAI. It is quick and easy to perform. What's already known about this topic? There are no validated scoring methodologies for oral mucous membrane pemphigoid (MMP). Proposed disease activity scoring tools for MMP include the Mucous Membrane Disease Area Index (MMPDAI) and the Autoimmune Bullous Skin Disorder Intensity Score (ABSIS). The Oral Disease Severity Score (ODSS) has been validated for use in oral pemphigus vulgaris (PV). It has been shown to be reliable and sensitive in both lichen planus (LP) and MMP. What does this study add? The ODSS has been shown to be a thorough, sensitive and reproducible, yet quick scoring tool for the assessment of oral involvement in MMP. Its versatility for use in oral PV, MMP and LP is an added advantage over other scoring methodologies. What are the clinical implications of this work? We propose that the ODSS be used as a clinical scoring tool for monitoring activity in oral MMP in clinical practice as well as for use in multicentre studies.

An Oral Disease Severity Score validated for use in oral pemphigus vulgaris.

BACKGROUND: Pemphigus vulgaris (PV) is a rare autoimmune bullous disease, which can present with recalcitrant oral mucosal lesions. Optimal management of PV relies upon careful clinical assessment and documentation. OBJECTIVES: The primary aim of this study was to validate the Oral Disease Severity Score (ODSS) for the assessment of oral involvement in PV. A secondary aim was to compare its inter- and intraobserver variability and ease of use with the Physician's Global Assessment (PGA) and the oral scoring methods used in the Autoimmune Bullous Skin Disorder Intensity Score (ABSIS) and the Pemphigus Disease Area Index (PDAI). METHODS: Fifteen patients with mild-to-moderately severe oral PV were scored for disease severity by 10 oral medicine clinicians using the ODSS, the PGA and the oral sections of ABSIS and PDAI. Two clinicians rescored all patients after a minimum 2-h interval. RESULTS: Interobserver reliability was assessed using an intraclass correlation coefficient (ICC). For the ODSS total score the ICC was 0·83, for PDAI (oral total activity) 0·79, ABSIS (oral total) 0·71 and PGA 0·7. Intraobserver agreement between initial scoring and rescoring of the same patient by two clinicians demonstrated an ICC for each of 0·97 and 0·96 for ODSS total score; 0·99 and 0·82 for PDAI oral activity; 0·86 and 0·45 for ABSIS total; and 0·99 and 0·64 for PGA. Convergent validity was good, with a correlation coefficient > 0·5 (P < 0·001). The mean ± SD times taken to complete each scoring method were ODSS 76 ± 37 s, PDAI 117 ± 16 s and ABSIS 75 ± 19 s. CONCLUSIONS: This study has validated the ODSS for the assessment of oral PV. It has shown superior inter- and intraobserver reliability to PDAI, ABSIS and PGA and is quick to perform.

Efficacy of mycophenolate mofetil in severe mucocutaneous lichen planus: a retrospective review of 10 patients.

BACKGROUND: Ulcerative lichen planus is an uncommon and severe subtype of lichen planus primarily affecting the oral mucosal surfaces. It may be associated with significant morbidity and often requires immunosuppressive therapy to achieve disease control. There have been no previous reports in which objective outcome measures have been used to assess the efficacy of mycophenolate mofetil (MMF) in severe ulcerative lichen planus. OBJECTIVE: To evaluate the clinical responses of patients with severe ulcerative oral lichen planus who were treated with MMF at a tertiary oral medicine/dermatology centre. METHODS: This was a retrospective review of oral disease severity scores performed in 10 patients with recalcitrant ulcerative oral lichen planus (vulvovaginal-gingival, n = 8; penogingival, n = 1; oral, n = 1) before and after treatment with MMF therapy. The results were analysed using the Wilcoxon matched pairs signed-rank test. RESULTS: The mean duration of MMF treatment was 3·7 (SD ± 2·4) years with a mean follow-up of 4·2 (SD ± 2·7) years. The mean baseline oral disease severity scores (39·1 ± 11·9) improved by 40% after 12-15 months (24·3 ± 11·9, n = 8, P = 0·01) and by 43% after 21-24 months of MMF treatment (22·2 ± 10·4, n = 9, P = 0·01). Of the 10 patients, six achieved remission, one had well-controlled disease and three had partially controlled disease. Two patients who achieved remission successfully discontinued treatment. MMF was well tolerated in all patients. CONCLUSION: Our case series demonstrates the efficacy and favourable side-effect profile of MMF in the treatment of severe ulcerative lichen planus.

An international survey of oral medicine practice: proceedings from the 5th World Workshop in Oral Medicine.

OBJECTIVES: (i) To define the current state of oral medicine clinical practice internationally, and (ii) to make recommendations for future modeling of the practice of oral medicine. MATERIALS AND METHODS: A survey was designed by an international panel of oral medicine experts to assess the current state of oral medicine practice internationally. The survey was sent to oral medicine experts across the world, and responses were electronically stored and analyzed using descriptive statistics. RESULTS: Two hundred respondents completed the survey representing 40 countries from six continents. The two most common settings for an oral medicine practice were in a hospital and a dental school. More than 88% of respondents considered management of oral mucosal disease, salivary dysfunction, oral manifestations of systemic diseases, and facial pain in the definition of oral medicine. CONCLUSIONS: (i) Oral medicine clinicians diagnose and manage a wide variety of orofacial conditions; (ii) There are significant differences in the definition of oral medicine clinical practice from country to country; (iii) India has the largest expansion of oral medicine services as defined by escalating numbers of clinicians within the specialty as compared with other countries; (iv) oral medicine practitioners have a wide range of professional responsibilities.

DNA ploidy in proliferative verrucous leukoplakia.

Proliferative verrucous leukoplakia (PVL) is a clinicopathologically distinctive form of oral leukoplakia presenting with multifocal flat, nodular and verrucous lesions that progress inexorably to squamous carcinoma. The aims of this investigation were to describe the clinical and histopathological features of six cases of PVL and to determine whether lesional epithelium demonstrates DNA ploidy anomalies prior to malignant transformation. The clinical and pathological features of six patients were reviewed and all biopsy specimens were subjected to image-based DNA ploidy analysis. The female:male ratio was 5:1 and the average age on first biopsy was 66 years. Only one patient reported both tobacco smoking and alcohol intake. The most frequently affected sites were alveolar ridge and/or gingiva (6/6), buccal mucosa (3/6), palate (3/6), tongue (2/6), buccal sulcus (2/6), and lip (1/6). Three patients developed multiple primary carcinomas, either invasive or verrucous. A ploidy anomaly at any oral site would have predicted malignant transformation in four cases and probably in a fifth for whom DNA ploidy failed to meet diagnostic criteria but was suspicious of aneuploidy. The site of transformation was predicted by ploidy and histopathology for three carcinomas and a further carcinoma showed severe dysplasia and a suspicious ploidy result in adjacent tissue. Both conventional histopathology and DNA ploidy proved effective in predicting the site of transformation in this limited series.

Oral dryness and Sjögren's: an update.

Oral dryness is a very common condition presenting to a general dental practitioner or hospital specialist. The most common cause of oral dryness is drug related, however, patients with Sjögren's syndrome, a multisystem autoimmune condition, may present to their dentist rather than their GP complaining of dry mouth and dry eyes. This update article explores the causes of oral dryness and how to manage it. The update on Sjögren's syndrome explains the latest relevant diagnostic criteria, presenting signs, symptoms, investigations and management principles.

Changes in Saliva Rheological Properties and Mucin Glycosylation in Dry Mouth.

Saliva is vital for the maintenance of normal oral physiology and mucosal health. The loss of salivary function can have far-reaching consequences, as observed with dry mouth, which is associated with increased orodental disease, speech impairment, dysphagia, and a significant negative effect on quality of life. The timely diagnosis of oral dryness is vital for the management of orodental disease and any associated often-undiagnosed systemic disease (e.g., Sjögren syndrome). Our aim was to investigate differences in mucin glycoproteins and saliva rheological properties between sufferers and nonsufferers of dry mouth in order to understand the relationship between saliva composition, rheological properties, and dryness perception and provide additional potential diagnostic markers. All patients exhibited objective and subjective oral dryness, irrespective of etiology. Over half of the patients (n = 20, 58.8%) had a saliva secretion rate above the gland dysfunction cutoff of 0.1 mL/min. Mucin (MUC5B and MUC7) concentrations were generally similar or higher in patients. Despite the abundance of these moisture-retaining proteins, patients exhibited reduced mucosal hydration (wetness) and significantly lower saliva spinnbarkeit (stringiness), suggesting a loss of the lubricating and retention/adhesion properties of saliva, which, at least partially, are associated with mucin glycoproteins. Over 90% of patients with dry mouth (DMPs) consistently had unstimulated whole mouth saliva (UWMS) spinnbarkeit below the proposed normal cutoff (10 mm). Further analysis of mucins revealed the reduced glycosylation of mucins in DMPs compared to healthy controls. Our data indicate that UWMS mucin concentrations are not reduced in dry mouth but that the mucin structure (glycosylation) is altered. UWMS from DMPs had reduced spinnbarkeit, the assessment of which, in conjunction with sialometry, could improve sensitivity for the diagnosis of dry mouth. Additionally, it may be useful to take into consideration the altered mucin glycosylation and saliva rheological properties when designing synthetic or purified mucins for saliva substitutes and dry mouth therapy.

Human junctional epithelium: demonstration of a new marker, its growth in vitro and characterization by lectin reactivity and keratin expression.

We have studied lectin reactivity in normal human junctional, sulcular, and attached gingival epithelia with 15 lectins and identified the epithelia by parallel staining with monoclonal anti-keratin antibodies. Dolichos biflorus agglutinin reacted uniquely with junctional epithelium, not staining other gingival cells of non-blood group A1 donors. We have demonstrated that the moiety recognized in junctional epithelium is not blood group A1 antigen or Tn antigen. Using a panning technique with this lectin to isolate the cells, we have grown keratinocytes from human junctional epithelium, and compared their phenotype in vitro to that of cells grown from the sulcular and attached gingival epithelium. Colonies established from each epithelial type were examined in frozen section with the anti-keratin antibodies. All expressed keratin 14 (keratinocyte marker), keratins 4 and 13 (suprabasal non-cornification markers), and keratins 7, 18, and 19 (simple epithelia keratins). Keratins 1, 10, and 8 were not expressed. Vimentin, the intermediate filament of mesenchymal cells, was also expressed by all types of cells in culture. Thus we have shown that when cells from the three areas of the gingiva were grown in culture they revert to one phenotype, at least with respect to their keratin expression. These results support the hypothesis that the epithelial phenotype is influenced by the sub-epithelial mesenchyme, and it is this that is responsible for the unique phenotype of the junctional epithelium.

Sialographic changes in Sjögren's and SOX syndromes.

OBJECTIVES: The purpose of this study was to examine the sialographic presentations of Sjögren's syndrome and SOX (sialadenitis, osteoarthritis, and xerostomia) syndrome and to establish whether changes in duct dimensions were a feature of these conditions. STUDY DESIGN: The sialograms of 17 patients with Sjögren's syndrome, 18 patients with SOX syndrome, and 19 patients with no salivary gland disease were examined. Through the use of an A010 Solitaire Image Analysis System, ductal diameters were measured. The films were then assessed subjectively. RESULTS: There was more sialectasis seen in the patients with Sjögren's syndrome than in the SOX group (chi-squared test: p < 0.01). There were no differences in duct diameter between the three groups. Control subjects with normal glands were shown to have narrower main ducts than has been previously reported, with an age-related widening of 1 degree and 2 degrees ducts. CONCLUSIONS: Sialectasis is not a feature of SOX syndrome. Ductal narrowing should not be thought of as a feature of either SOX or Sjögren's syndrome.

Pyostomatitis vegetans associated with asymptomatic ulcerative colitis: a case report.

Pyostomatitis vegetans, a rare pustular disorder of the oral mucosa, is a highly specific marker for inflammatory bowel disease and may be difficult to treat. A case of pyostomatitis vegetans in a patient with long-standing asymptomatic ulcerative colitis is presented. Complete remission was achieved with topical steroids; no systemic treatment was required.

Patient preferences in a preliminary study comparing an intra-oral lubricating device with the usual dry mouth lubricating methods.

OBJECTIVE: To compare an intra-oral device to relieve oral dryness with the other methods of lubricating the mouth at night. DESIGN: Multidisciplinary single blind randomised cross over study. SETTING: The subjects were drawn from patients attending a dry mouth clinic. MATERIALS AND METHODS: Thirty-four dentate subjects attended on five occasions at intervals of 4 weeks. At the first visit the teeth were scaled and impressions were recorded. The device was fitted either on the second or the fourth visit. At all visits samples were taken of the resting and stimulated saliva for volumetric analysis and the dry mouth score recorded. Data were collected from the lubrication timings and the questionnaire. RESULTS: Ten water, nine saliva substitute and ten sugar-free chewing gum lubricators completed the study. There were 27 female and two male subjects with an average age of 62 years. Nine out of 10 of those lubricating with chewing gum preferred wearing the device (P = 0.037). After the device wearing period the subjects' self assessment of mouth dryness (P = 0.056), speech (P = 0.009) and swallowing (P = 0.031) were more favourable when compared with the alternative lubrication with 66% preferring the intra-oral device to their alternative method of lubrication. CONCLUSIONS: The majority of the subjects preferred wearing the device at night compared with their normal method of lubrication. Subjects' perception of dryness, speech and swallowing became closer to the clinician's assessment after wearing the device.

Clinical assessment of oral dryness: development of a scoring system related to salivary flow and mucosal wetness.

OBJECTIVE: The aim of this study was to develop a clinical oral dryness score (CODS) for routine use in assessment of xerostomia patients and determine its relationship with salivary flow rates and mucosal wetness. STUDY DESIGN: CODS was determined from 10 features of oral dryness, each scoring as 1 point for a total score of 0-10. CODS, salivary flow rates, and mucosal wetness were measured in 100 patients and 50 healthy control subjects. The reproducibility of CODS was 0.89-0.96 (intraclass correlation coefficient). RESULTS: The mean ± SD CODS in patients was 6.0 ± 1.6 compared with 1.0 ± 0.9 for control subjects (P < .001), and the highest mean value was in the primary Sjögren syndrome group. There was a general inverse relationship in patients between mean CODS and salivary flow rate (P < .01) and mean CODS and mucosal wetness (P < .01). CONCLUSIONS: The CODS was found to be useful, easy to use, and reliable for routine assessment of the severity of dry mouth.

American College of Rheumatology classification criteria for Sjögren's syndrome: a data-driven, expert consensus approach in the Sjögren's International Collaborative Clinical Alliance cohort.

OBJECTIVE: We propose new classification criteria for Sjögren's syndrome (SS), which are needed considering the emergence of biologic agents as potential treatments and their associated comorbidity. These criteria target individuals with signs/symptoms suggestive of SS. METHODS: Criteria are based on expert opinion elicited using the nominal group technique and analyses of data from the Sjögren's International Collaborative Clinical Alliance. Preliminary criteria validation included comparisons with classifications based on the American­European Consensus Group (AECG) criteria, a model-based "gold standard"obtained from latent class analysis (LCA) of data from a range of diagnostic tests, and a comparison with cases and controls collected from sources external to the population used for criteria development. RESULTS: Validation results indicate high levels of sensitivity and specificity for the criteria. Case definition requires at least 2 of the following 3: 1) positive serum anti-SSA and/or anti-SSB or (positive rheumatoid factor and antinuclear antibody titer >1:320), 2) ocular staining score >3, or 3) presence of focal lymphocytic sialadenitis with a focus score >1 focus/4 mm2 in labial salivary gland biopsy samples. Observed agreement with the AECG criteria is high when these are applied using all objective tests. However, AECG classification based on allowable substitutions of symptoms for objective tests results in poor agreement with the proposed and LCA-derived classifications. CONCLUSION: These classification criteria developed from registry data collected using standardized measures are based on objective tests. Validation indicates improved classification performance relative to existing alternatives, making them more suitable for application in situations where misclassification may present health risks.

A scoring system for mucosal disease severity with special reference to oral lichen planus.

BACKGROUND: To date, there is only weak evidence for the superiority of any interventions over placebo for the palliation of symptomatic oral lichen planus (LP). Further research involving large placebo-controlled, randomized clinical trials is needed. These will require carefully selected and standardized outcome measures. OBJECTIVES: To formulate a scoring system for intraoral LP. METHODS: One hundred and fifty-six patients with biopsy-confirmed LP were scored at the first and subsequent visits according to (i) extent of site involvement, (ii) disease activity at each site and (iii) an overall pain score as reported by the patient. Overall differences between clinical variants of LP were analysed using the Kruskal-Wallis test and pairwise differences by the Mann-Whitney U-test. Clinical sensitivity (Wilcoxon signed-rank test) was assessed by scoring patients before and after treatment (n = 23). RESULTS: Reticular LP (n = 48) was the commonest single type of clinical presentation, followed by ulcerative (n = 30), atrophic (n = 22), desquamative (n = 18) and plaque (n = 1). The median severity and activity scores were 13/6 (reticular), 39/20 (ulcerative), 20/9 (atrophic) and 23/11 (desquamative). Two or more clinical variants were seen in 37 cases. Statistical significance was observed for differences between clinical variants (P < 0.0001) and variation in scores (P < 0.01) when ulcerative LP was compared with all other types. Clinical sensitivity was statistically significant (P < 0.01), while reproducibility was high and allowed the response to therapy to be easily assessed. CONCLUSIONS: It is suggested that this scoring system is easy to use, reproducible and sensitive enough to detect clinical responses to therapy.

Impact of wearing an intra-oral lubricating device on oral health in dry mouth patients.

OBJECTIVE: To establish whether an intra-oral lubricating device for dry mouth alters the oral environment. DESIGN: A single-blind randomized cross-over study. METHOD: Twenty-nine dentate subjects from the Sjogren's syndrome clinic attended on five occasions at 4-week intervals. They were randomized, having the device fitted on either the second or the fourth visit for the experimental period, whilst using their preferred method of lubrication throughout the rest of the study. The preferred methods of lubrication were either water (group 1, n = 10) or saliva substitute (group 2, n = 9) or sugar-free chewing gum (group 3, n = 10). At each visit microbiological, unstimulated and stimulated saliva samples were collected. Dry mouth score, speech test and periodontal indices were recorded. RESULTS: The water lubrication group (1) had a resting salivary flow greater than lubrication groups (2 and 3) by post-ANOVA contrasts (P < 0.001). The postdevice data also demonstrated a salivary flow greater than lubrication group (3) by post-ANOVA contrasts (P < 0.05). The epithelial cell count using the Spearman correlation was high, possibly reflecting increased viscosity of the saliva (P = 0.044). The speech test indicated that the experimental subjects had difficulty in speaking (P = 0.001). This was slightly easier postdevice wear. Streptococcus mutans (P = 0.009) and Lactobacillus (P = 0.058) increased in the saliva after wearing the device. Salivary flow rate, Candida albicans, oral dryness, speaking and periodontal indices were unchanged. CONCLUSIONS: The oral environment was altered by wearing a lubricating device with an increase in the numbers of Strep. mutans and Lactobacillus. Clinical dryness and speech test correlated with the mean whole salivary flow suggesting a screening method for xerostomia.

Management of the oral mucosal lesions seen in association with HIV infection.

Oral lesions cause considerable morbidity in association with HIV infection. Their successful management depends upon accurate diagnosis and the use of appropriate therapy. Various treatment approaches are described for some of the common oral lesions including Kaposi's sarcoma, oral candidiasis, hairy leukoplakia and recurrent oral ulcers associated with HIV disease. This paper will discuss the therapies available in the USA and UK. In other countries some of the drugs discussed will be available in different doses and preparations. In addition other drugs may be available in other parts of the world that are not licensed for use in the USA or UK, and their availability may vary.