Ascher's syndrome: a rare cause of lip swelling.

Ascher's syndrome is a rare, benign entity with just over 100 reported cases. The condition is characterised by a 'double' upper lip, blepharochalasis and non-toxic thyroid enlargement. It presents before the age of 20 years in the majority of cases and shows no racial or gender differences. While the exact cause is unknown, hormonal dysfunction and autosomal dominant inheritance have been suggested as possible aetiological factors. We present two cases of Ascher's syndrome referred for investigation of lip swelling.

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.

Oral Medicine for undergraduate dental students in the United Kingdom and Ireland-A curriculum.

INTRODUCTION: Oral Medicine focuses on care for patients with chronic, recurrent and medically related disorders of the orofacial region that are distinct from diseases of the periodontal and tooth tissues, with an emphasis on non-surgical management. At present, there are no shared outcomes for Oral Medicine to define the standards to be achieved before new graduates become registered dentists engaged with ongoing professional development. CURRICULUM: We present a consensus undergraduate curriculum in Oral Medicine agreed by representatives from 18 Dental Schools in the United Kingdom and Republic of Ireland. The scope of Oral Medicine practice includes conditions involving the oral mucosa, salivary glands, neurological system or musculoskeletal tissues that are not directly attributable to dental (tooth and periodontium) pathology. Account is taken of the priorities for practice and learning opportunities needed to support development of relevance to independent clinical practice. The outcomes triangulate with the requirements set out by the respective regulatory bodies in the UK and Republic of Ireland prior to first registration and are consistent with the framework for European undergraduate dental education and greater harmonisation of dental education. CONCLUSIONS: This curriculum will act as a foundation for an increasingly shared approach between centres with respect to the outcomes to be achieved in Oral Medicine. The curriculum may also be of interest to others, such as those responsible for the training of dental hygienists and dental therapists. It provides a platform for future collective developments with the overarching goal of raising the quality of patient care.

Assessment in a global context: An international perspective on dental education.

Assessments are widely used in dental education to record the academic progress of students and ultimately determine whether they are ready to begin independent dental practice. Whilst some would consider this a "rite-of-passage" of learning, the concept of assessments in education is being challenged to allow the evolution of "assessment for learning." This serves as an economical use of learning resources whilst allowing our learners to prove their knowledge and skills and demonstrating competence. The Association for Dental Education in Europe and the American Dental Education Association held a joint international meeting in London in May 2017 allowing experts in dental education to come together for the purposes of Shaping the Future of Dental Education. Assessment in a Global Context was one topic in which international leaders could discuss different methods of assessment, identifying the positives, the pitfalls and critiquing the method of implementation to determine the optimum assessment for a learner studying to be a healthcare professional. A post-workshop survey identified that educators were thinking differently about assessment, instead of working as individuals providing isolated assessments; the general consensus was that a longitudinally orientated systematic and programmatic approach to assessment provide greater reliability and improved the ability to demonstrate learning.

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.

Assessing the application of knowledge in clinical problem-solving: The structured professional reasoning exercise.

INTRODUCTION: Clinical reasoning is a fundamental and core clinical competence of healthcare professionals. The study aimed to investigate the utility of the Structured Professional Reasoning Exercise (SPRE), a new competence assessment method designed to measure dental students' clinical reasoning in simulated scenarios, covering the clinical areas of Oral Disease, Primary Dental Care and Restorative Dentistry, Child Dental Health and Dental Practice and Clinical Governance. MATERIALS AND METHODS: A total of 313 year-5 students sat for the assessment. Students spent 45 minutes assimilating the scenarios, before rotating through four pairs of 39 trained examiners who each independently assessed a single scenario over a ten-minute period, using a structured marking sheet. After the assessment, all students and examiners were invited to complete an anonymous perception questionnaire of the exercise. These questionnaires and the examination scores were statistically analysed. RESULTS AND DISCUSSION: Oral Disease showed the lowest scores; Dental Practice and Governance the highest. The overall Intraclass Correlation Coefficient (ICC) was 0.770, whilst examiner training helped to increase the ICC from 0.716 in 2013 to 0.835 in 2014. Exploratory factor analysis revealed one major factor with an eigenvalue of 2.75 (68.8% of total variance). The Generalizability coefficient was consistent at 0.806. A total of 295 students and 32 examiners completed the perception questionnaire. Students' lowest examination perceptions were an "Unpleasant" and "Unenjoyable" experience, whilst the highest were "Interesting", "Valuable" and "Important". The majority of students and examiners reported the assessment as acceptable, fair and valid. CONCLUSION: The SPRE offers a reliable, valid and acceptable assessment method, provided it comprises at least four scenarios with two independently marking and trained assessors. 3.

Analysis of dental students' written peer feedback from a prospective peer assessment protocol.

INTRODUCTION: Peer assessment and feedback is encouraged to enhance students' learning. The aim of this study was to quantitatively and qualitatively analyse pre-clinical and clinical dental students' written peer feedback provided as part of a continuous, formative and structured peer assessment protocol. MATERIALS AND METHODS: A total of 309 Year-2 and Year-5 dental students were invited to participate in a peer assessment and peer feedback protocol. Consenting volunteer students were trained to observe each other whilst working in the skills laboratory (Year-2) and in the dental clinic (Year-5). Subsequently, they followed a structured protocol of peer assessment and peer feedback using specially designed work-based forms during a complete academic year. The content of their written feedback was coded according to the UK General Dental Council domain, sign (positive or negative), specificity (task specific or general), and grouped into themes. RESULTS: A total of 108 participants (40 Year-2 and 68 Year-5) completed 1169 peer assessment work-based forms (516 pre-clinical and 653 clinical); 94% contained written feedback. The large majority (82%) of Year-2 feedback represented the clinical domain, 89% were positive, 77% were task specific, and they were grouped into 14 themes. Year-5 feedback was related mostly to Management and Leadership (37%) and Communication (32%), 64% were positive, 75% task specific, and they were clustered into 24 themes. DISCUSSION: The content of the feedback showed notable differences between Year-2 and Year-5 students. Senior students focused more on Communication and Management and Leadership skills, whilst juniors were more concerned with clinical skills. Year-5 students provided 13% negative feedback compared to only 2% from Year-2. Regulatory focus theory is discussed to explain these differences. Both groups provided peer feedback on a wide and different range of themes. However, four themes emerged in both groups: efficiency, infection control, time management and working speed. CONCLUSION: A structured peer assessment framework can be used to guide pre-clinical and clinical students to provide peer feedback focused on different domains, and on contrasting signs and specificities. It can also present an opportunity to complement tutors' feedback.

Azathioprine is effective for oral involvement in Crohn's disease but not for orofacial granulomatosis alone.

BACKGROUND: There have been no previous reports assessing the effectiveness of azathioprine (AZA) in the treatment of orofacial granulomatosis (OFG). This report is a review of patients receiving AZA for active OFG with or without concomitant gut Crohn's disease (CD) in a specialist tertiary referral centre. METHODS: Clinical response was defined by Global Physician Assessment at 4-, 12- and 24-month follow-up and a standardised oral disease activity score (ODAS). RESULTS: Sixty of 215 patients seen with OFG in our clinic over a 12-year period were treated with AZA. Of these, 22 had concomitant CD. The proportion of patients responding to AZA with a diagnosis of CD/OFG vs. OFG only at 4, 12 and 24 months were 54% vs. 21% (P = 0.03), 59% vs. 21% (P = 0.003) and 41% vs. 24% (P = 0.16), respectively. A statistically significant difference was seen between starting and follow-up ODAS scores at 4 months in the CD/OFG group which was not observed in the OFG only group. Factors predicting a need for AZA included a diagnosis of intestinal CD, sulcal swelling, sulcal ulcers and upper lip involvement. The factor predicting response to treatment was a diagnosis of CD at 12 months of follow-up. No difference in the number of adverse effects was observed between the two groups of patients. CONCLUSIONS: AZA is significantly more effective in the treatment of oral disease with a concurrent diagnosis of CD rather than in the treatment of OFG alone.

Dental students' reflective habits: is there a relation with their academic achievements?

INTRODUCTION: Reflection is regarded as an important and essential component of healthcare professionals' education and practice. This cross-sectional study aimed to explore the levels of reflection habits of dental students and clinical and PhD postgraduate trainees and to establish the relationship between students' reflection and their academic performance. MATERIALS AND METHODS: A total of 324 subjects (208 females, 56% ≥23 years of age) from King's College London Dental Institute, consisting of 281 undergraduate and 43 postgraduate trainees, responded to the invitation to participate and completed an online self-reported Reflection Questionnaire; this assesses two levels of non-reflective actions (Habitual Action and Understanding) and two levels of reflective actions (Reflection and Critical Reflection). Reflection results were compared amongst different cohorts and correlated with students' academic performance. RESULTS: Reflection Questionnaire mean scores were 10.7 for Habitual Action, 17.3 for Understanding, 17.0 for Reflection and 14.4 for Critical Reflection, with significant differences between cohorts of undergraduate students, clinical postgraduates and PhD trainees. Reflection and Critical Reflection mean scores were higher as students climbed courses. Further, those ≥24 years of age and those with previous university degrees demonstrated higher reflective habits, whilst there was no gender difference. Additionally, the assessment programme methods correlated differently to the Reflection Questionnaire scores. DISCUSSION: The Reflection Questionnaire's internal reliability was acceptable. The most common approaches used by both students and trainees were Understanding and Reflection, whilst those with high Understanding scores also tended to have good Reflective scores. Further, multiple choice questions encouraged students to 'understand', whilst ill-defined problems, such as those from essays to clinical reasoning cases, increased this to the reflection scale. CONCLUSION: To foster reflective thinking, tutoring should primarily be focused on younger students (≤23 years old) and those without a previous university degree. Further, a wide variety of assessment methods is suggested to stimulate different reflective constructs.

A practical guide to the use of thiopurines in oral medicine.

Thiopurines are widely used as first-line immunosuppressive therapies in the management of chronic inflammatory oral disease. However, despite over half a century of clinical experience, the evidence base for their use is limited. The aims of this paper were to review the evidence for the use of thiopurines in oral medicine and provide a contemporary model of thiopurine metabolism and mechanism of action and a rationale for clinical use and safe practice.

Dental students' peer assessment: a prospective pilot study.

INTRODUCTION: Peer assessment is increasingly used in health education. The aims of this study were to evaluate the reliability, accuracy, educational impact and student's perceptions of undergraduate pre-clinical and clinical dental students' structured and prospective Peer assessment and peer feedback protocol. MATERIALS AND METHODS: Two Direct Observation of Procedural Skills (DOPS) forms were modified for use in pre-clinical and clinical peer assessment. Ten year two dental students working in a phantom-heads skills laboratory and 16-year five dental students attending a comprehensive care clinic piloted both peer DOPS forms. After training, pairs of students observed, assessed and provided immediate feedback to each other using their respective peer DOPS forms as frameworks. At the end of the 3-month study period, students anonymously provided their perceptions of the protocol. RESULTS: Year 2 and year 5 students completed 57 and 104 peer DOPS forms, respectively. The generalizability coefficient was 0.62 for year 2 (six encounters) and 0.67 for year 5 (seven encounters). Both groups were able to differentiate amongst peer-assessed domains and so detect improvement in peers' performance over time. Peer DOPS scores of both groups showed a positive correlation with their mean end-of-year examination marks (r ≥ 0.505, P ≥ 0.051) although this was not statistically significant. There was no difference (P ≥ 0.094) between the end-of-year examination marks of the participating students and the rest of their respective classes. The vast majority of both groups expressed positive perceptions of the piloted protocol. DISCUSSION: There are no data in the literature on the prospective use of peer assessment in the dental undergraduate setting. In the current study, both pre-clinical and clinical students demonstrated the ability to identify those domains where peers performed better, as well as those which needed improvement. Despite no observable educational impact, most students reported positive perceptions of the peer DOPS protocol. CONCLUSIONS: The results of this pilot study support the need for and the potential benefit of a larger- and longer-term follow-up study utilising the protocol.

Development of a low phenolic acid diet for the management of orofacial granulomatosis.

BACKGROUND: Orofacial granulomatosis (OFG) is a rare disease of unknown cause. A cinnamon- and benzoate-free diet is successful in up to 72% of patients. Phenolic acids are among the chemical constituents restricted in this diet, which avoids some but not all of these structurally similar compounds. The present study aimed to: (i) develop a novel diet low in phenolic acids; (ii) implement this in a small clinical trial; and (iii) assess its nutritional adequacy. METHODS: A literature review identified 10 papers quantifying phenolic acids from which 91 10-mg phenolic acid exchanges were devised. A phenolic acid exclusion diet with precautionary micronutrient supplementation was designed and implemented in 10 patients. Phenolic acids were excluded for 6 weeks and were reintroduced at a rate of one exchange every second day for 6 weeks. Wilcoxon matched pairs tests analysed disease outcomes measured by an oral disease severity scoring tool at weeks 0, 6 and 12. Nutritional adequacy was assessed, excluding micronutrient supplementation, at weeks 0 and 6, and compared intakes with dietary reference values. RESULTS: The diet was nutritionally inadequate for a range of micronutrients. Seven of 10 patients responded. Mean [standard deviation (SD)] severity scores improved from week 0-6 [20.8 (9.39) and 10.1 (5.72); P = 0.009] and were maintained in five patients who completed the reintroduction [6.6 (3.13) and 7.2 (5.54); P = 0.713]. CONCLUSIONS: A low phenolic acid diet with micronutrient supplementation holds promise of a novel dietary treatment for OFG. Further work is required in larger studies to determine long-term outcomes.

Dietary intervention for oral allergy syndrome as a treatment in orofacial granulomatosis: a new approach?

BACKGROUND: Orofacial granulomatosis (OFG) is a chronic granulomatous condition of the mouth, face and lips. Recent work demonstrates a high rate of atopy and silver birch sensitisation from skin prick testing (SPT). Oral allergy syndrome (OAS) is an acute oro-pharyngeal IgE mediated reaction, triggered by foods that cross react with pollens, most commonly silver birch. The aim of this study was to determine if patients with OFG and positive SPT to common OAS associated pollens responded to avoidance of cross reactive foods. METHODS: Patients with OFG and positive SPT to silver birch, grass, mugwort, ragweed and latex were required to avoid cross reacting foods, for 6 weeks and, in those who responded, for a total of 12 weeks. All had standardized oral examinations and were given severity scores (SS) at each appointment. RESULTS: Twenty two of 47 (47%) patients had one or more positive SPT and 13/22 completed 6 weeks on the diet. No difference was seen in SS between weeks 0 (14.62 ± 11.16) and 6 (13.31 ± 10.33; P = 0.656). Six of 14 (43%) had significantly improved SS (week 0; 19.17 ± 12.95, week 6; 10.83 ± 4.99, P = 0.027). Five completed 12 weeks and no further improvement was seen (week 6; 11 ± 5.57, week 12; 10.4 ± 9.94; P = 0.068). Two patients required no further treatments. CONCLUSIONS: On an intention to treat basis, only 2/14 patients improved and required no further intervention. Whilst this diet cannot be recommended routinely, the improvement seen in some patients raises questions about the role of OAS in patients with OFG.

Review article: cinnamon- and benzoate-free diet as a primary treatment for orofacial granulomatosis.

BACKGROUND: Orofacial granulomatosis is a rare chronic granulomatous inflammatory disease of the lips, face and mouth. The aetiology remains unclear but may involve an allergic component. Improvements have been reported with cinnamon- and benzoate-free diets. AIMS: To explore the prevalence of compound and food sensitivity and examine the dietary treatments used in orofacial granulomatosis. METHODS: A comprehensive literature search was carried out and relevant studies from January 1933 to January 2010 were identified using the electronic database search engines; AGRIS 1991-2008, AMED 1985-2008, British Nursing and Index archive 1985-2008, EMBASE 1980-2008, evidence based medicine review databases (e.g. Cochrane DSR), International Pharmaceutical and Medline 1950-2008. RESULTS: Common sensitivities identified, predominantly through patch testing, were to benzoic acid (36%) food additives (33%), perfumes and flavourings (28%), cinnamaldehyde (27%), cinnamon (17%), benzoates (17%) and chocolate (11%). The cinnamon- and benzoate-free diet has been shown to provide benefit in 54-78% of patients with 23% requiring no adjunctive therapies. A negative or positive patch test result to cinnamaldehyde, and benzoates did not predict dietary outcome. The most concentrated source of benzoate exposure is from food preservatives. Use of liquid enteral formulas can offer a further dietary therapy, particularly in children with orofacial granulomatosis. CONCLUSION: Management of orofacial granulomatosis is challenging but cinnamon- and benzoate-free diets appear to have a definite role to play.

Orofacial presentations of sarcoidosis--a case series and review of the literature.

Sarcoidosis is a multi-system disease of unknown aetiology characterised by the presence of non-caseating granulomas, the lungs and lymph nodes being the most affected sites. Orofacial manifestations of the condition are increasingly recognised, with several recent case reports where the initial presentation of the disease is in the region. Here, we report six cases of orofacial sarcoidosis which help to illustrate the wide spectrum of the condition.

Meningioma as a cause of chronic orofacial pain: case reports.

We describe two middle-aged men whose chronic orofacial pain was caused by underlying meningiomas. In both cases treatment was delayed because evolving dentoalveolar and possible chronic idiopathic facial pain had been investigated before presentation. Subsequent disturbances of the ipsilateral VII (and later VIII) nerves prompted magnetic resonance imaging (MRI) of one patient, while the possibility of a central lesion was recognised at presentation in the second, whose atypical focus of trigeminal neuralgia was labile within the ipsilateral distribution of the trigeminal nerve. Both cases highlight the importance of considering proximal intracranial lesions as a possible cause of atypical or refractory chronic orofacial pain so unnecessary delay in the diagnosis of otherwise operable tumours can be avoided.

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.

Lip impressions: a new method for monitoring morphological changes in orofacial granulomatosis.

AIM: To develop and evaluate an objective method for assessing lip size and treatment-related morphological changes in orofacial granulomatosis (OFG) patients. MATERIALS AND METHODS: Patients with swollen lips because of OFG (n=21) were enrolled. A light-body polyvinylsiloxane material was used to take lip impressions before and after treatment (n=10), or during treatment (n=11). Plaster models were cast from the impressions and the lips were measured using callipers. The intra-examiner and inter-examiner reproducibility of the technique were assessed. RESULTS: OFG patients had significantly larger lips than controls (P<0.0001). The coefficient of variation on repeated measurements of the same impression was 1.6% and for duplicate impressions was 2.6%. Significant reduction in lip size was shown in all 10 patients after diet restriction (P<0.002). Seven of 11 patients whose impressions were taken at least 3 months after the initiation of cinnamon- and benzoate-free diet also showed reduction in lip size during follow up (P<0.002). CONCLUSIONS: Serial lip impressions appear to be reliable for routine quantification of morphological changes of the lips in OFG patients. We present a new reproducible and sensitive method for assessing changes in lip size in response to treatment in OFG.

Number VII Behçet's disease (Adamantiades syndrome).

Behçet's syndrome (BS; Adamantiades syndrome) is the association of the triple symptom complex of recurrent aphthous stomatitis (RAS) with genital ulceration, and eye disease (especially iridocyclitis) though a number of other systemic manifestations may also be seen. BS mainly affects young adult males, and there is an association with HLA-B5 and HLA-B51 (B5101). Features such as arthralgia and leucocytoclastic vasculitis suggest an immune-complex mediated basis, which is supported by finding circulating immune complexes and, although the antigen responsible is unidentified, heat shock proteins have been implicated. An inflammatory disorder, BS is now considered as a systemic vasculitis, characterised by a very wide spectrum of clinical features and by unpredictable exacerbations and remissions.

Epithelial biology.

The oral mucous membrane has features similar to skin but also differs in several ways. This paper reviews the aspects of epithelial biology necessary for an understanding of the vesiculoerosive disorders.