Patterns of oral mucosa lesions in patients with epidermolysis bullosa: comparison and agreement between oral medicine and dermatology.

BACKGROUND: The oral mucosa in patients with epidermolysis bullosa (EB) can be affected with different lesions and degrees of severity. However, patterns of oral lesions in distinct types of EB are still unclear. OBJECTIVES: The purpose of this study was to determine the frequency and distribution of four types of lesions (erythema, erosion, atrophy, and blister) for each oral site and to calculate the interobserver reliability for each type of lesion in each site. METHODS: Ninety-two patients with different EB types were assessed independently by an oral medicine specialist and a dermatologist. The degree of agreement was calculated by the intraclass correlation coefficient (ICC). RESULTS: The most affected oral site was the tongue, with the most frequent lesion being erythema and atrophy [54(58.7%) patients] for the oral medicine specialist and erosion [54(58.7%) patients] for the dermatologist. Patients with recessive dystrophic EB-severe generalized (RDEB-sev gen) showed the highest mean of sites involved by each lesion for both oral medicine and dermatology. The interobserver reliability on the total of lesions was excellent on only 3 sites: lower lip (ICC: 0.89; 95%CI:0.83-0.92), hard palate (ICC:0.85; 95%CI:0.72-0.91), and tongue (ICC:0.89; 95%CI:0.84-0.92), whereas the interobserver reliability calculated for each single oral lesion showed a lower agreement. CONCLUSION: Total distribution of sites involved by four types of lesions was higher in RDEB-sev gen than in the rest of EB types, with a predominance of erythema followed by erosion. The agreement on the type of lesion was found to be poor-moderate for many oral sites.

Epidermolysis Bullosa Oropharyngeal Severity (EBOS) score: a multicenter development and reliability assessment.

BACKGROUND: Epidermolysis bullosa (EB) is a genetic mucocutaneous disorder characterized by blister formation upon mild trauma. All 4 EB types may show oropharyngeal lesions involving either hard or soft tissues. Currently, there are very few data on EB scoring that include the oropharyngeal cavity. OBJECTIVES: We sought to develop an oropharyngeal severity score that was objective, valid, reliable, reproducible, easy to perform, and appropriate for all EB types. METHODS: In this study, oral medicine specialists developed a new score, the EB Oropharyngeal Severity (EBOS) score. This measured oropharyngeal disease activity (erythema, atrophy, blisters, erosion/ulceration) and structural damage (microstomia, ankyloglossia, scarring phenotype beyond microstomia and ankyloglossia, enamel hypoplasia). It was tested on 92 patients with different types/subtypes of EB, and interobserver and intraobserver reliability were assessed. RESULTS: The EBOS mean total score was 12.9 ± 10.9 (range: 0-34). Both interobserver and intraobserver reliability for total score on all patients with EB were considered excellent (intraclass correlation coefficient 0.94; 95% confidence interval 0.90-0.96 and intraclass correlation coefficient 0.90; 95% confidence interval 0.84-0.94, respectively). Even analyzing each single parameter of the disease activity and structural damage, a substantial to excellent correlation was found in the interobserver (except for 4 sites) and intraobserver reliability. A significant correlation was found between EB types/subtypes and the EBOS median score (P < .001), but not between age and the EBOS mean total score in each group. LIMITATIONS: The sample size was small and the number of EB subtypes was limited. CONCLUSIONS: The EBOS score seems to represent an instrument capable of truly quantifying the oropharyngeal severity in different types/subtypes of EB, demonstrating excellent interobserver and intraobserver reliability.

High-dose curcuminoids are efficacious in the reduction in symptoms and signs of oral lichen planus.

BACKGROUND: Curcuminoids are components of turmeric (Curcuma longa) that possess anti-inflammatory properties. OBJECTIVE: We sought to study the efficacy of curcuminoids in controlling the signs and symptoms of oral lichen planus, at doses of 6000 mg/d (3 divided doses), and their safety at this dose. METHODS: Twenty consecutive, eligible patients who consented were enrolled into this randomized, double-blind, placebo-controlled clinical trial in 2007 through 2008. Measurement of symptoms and signs of oral lichen planus using the Numerical Rating Scale (NRS) and the Modified Oral Mucositis Index (MOMI), respectively; complete blood counts; liver enzymes; C-reactive protein; and interleukin-6 levels was done at baseline and day 14. Two-sided P values are reported. RESULTS: In the placebo group, the percentage changes from baseline in NRS (median [interquartile range] = 0.00 [-29 to 16.7], P > .99), erythema (0.00 [-10 to 16.7], P = .98), ulceration (0.00 [0.00 to 26.7], P = .63), and total MOMI scores (-3.2 [-13 to 9.09], P = .95) were not statistically significant, whereas they were statistically significant in the curcuminoids group: NRS (-22 [-33 to -14], P = .0078); erythema (-17 [-29 to -8.3], P = .0078), ulceration (-14 [-60 to 0.00], P = .063), MOMI (-24 [-38 to -11], P = .0039). The curcuminoids group showed a greater reduction in clinical signs and symptoms as compared with the placebo group, measured by percentage change in erythema (P = .05) and total MOMI score (P = .03), and proportion showing improvement in NRS (0.8 vs 0.3, P = .02) and total MOMI score (0.9 vs 0.5, P = .05). Adverse effects were uncommon in both groups. LIMITATIONS: The small sample size resulted in limited power, particularly for multivariate analyses. CONCLUSIONS: Curcuminoids at doses of 6000 mg/d in 3 divided doses are well tolerated and may prove efficacious in controlling signs and symptoms of oral lichen planus.

A treatment for oral precancerous lesions: Why do we not yet have a treatment?

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Validation of instruments to measure the symptoms and signs of oral lichen planus.

OBJECTIVE: The aim of this study was to validate the visual analog scale (VAS), numeric rating scale (NRS), and change in symptoms scale (CSS) in measuring symptoms of oral lichen planus, and the modified oral mucositis index (MOMI) in measuring the signs of oral lichen planus. STUDY DESIGN: Criterion validity, construct validity, and internal consistency reliability were evaluated using data from a randomized, double blind, placebo-controlled clinical trial of curcuminoids in oral lichen planus. RESULTS: Moderate to high correlations were found between VAS, NRS, and CSS. Correlations of symptom scores with clinical signs ranged from minimal to high. Correlation of NRS with clinical signs was stronger than that of VAS with clinical signs. Significant changes from baseline at each follow-up in NRS, VAS, and MOMI scores were seen. The Cronbach alpha for erythema and ulceration scores from the MOMI were 0.66. CONCLUSIONS: This study gives some evidence of the validity of NRS, VAS, CSS, and MOMI for use in oral lichen planus. The NRS has better construct validity than VAS, based on higher correlations with clinical signs. Erythema plus ulceration is a better measure than ulceration alone.

Oral lichen planus and oral lichenoid lesions: diagnostic and therapeutic considerations.

Several therapeutic agents have been investigated for the treatment of oral lichen planus (OLP). Among these are corticosteroids, retinoids, cyclosporine, and phototherapy, in addition to other treatment modalities. A systematic review of clinical trials showed that particularly topical corticosteroids are often effective in the management of symptomatic OLP lichen planus. Systemic corticosteroids should be only considered for severe widespread OLP and for lichen planus involving other mucocutaneous sites. Because of the ongoing controversy in the literature about the possible premalignant character of OLP, periodic follow-up is recommended. There is a spectrum of oral lichen planus-like ("lichenoid") lesions that may confuse the differential diagnosis. These include lichenoid contact lesions, lichenoid drug reactions and lichenoid lesions of graft-versus-host disease. In regard to the approach to oral lichenoid contact lesions the value of patch testing remains controversial. Confirmation of the diagnosis of an oral lichenoid drug reaction may be difficult, since empiric withdrawal of the suspected drug and/or its substitution by an alternative agent may be complicated. Oral lichenoid lesions of graft-versus-host disease (OLL-GVHD) are recognized to have an association with malignancy. Local therapy for these lesions rests in topical agents, predominantly corticosteroids.

Tacrolimus powder in Orabase 0.1% for the treatment of oral lichen planus and oral lichenoid lesions: an open clinical trial.

OBJECTIVES: The aim of our study was to evaluate the clinical efficacy and safety profile of a novel compound, Tacrolimus powder in Orabase 0.1% in patients with LP and LL. STUDY DESIGN: Seven patients with LP and 3 with LL were asked to participate. All patients received a 1 week treatment of Fluconazole, prior to entering the study, and on follow up visit were provided with a 15 g container of the study medication. Patients were asked to treat the most symptomatic site, three times a day for two weeks. RESULTS: Disease control (signs) was achieved in most patients by the end of two weeks (from 1.58 to 0.55); all patients experienced a high degree of discomfort (pain) at baseline, which dropped quickly by the end of the second week of treatment (from 1.95 to 0.45); none developed yeast during the course of treatment. Recurrent headaches were reported by one patient with erosive LP, and transient burning by a patient with reticular LP. CONCLUSIONS: Tacrolimus powder in Orabase 0.1% appears to have a relatively safe profile, and represents a likely alternative to topical steroids in the treatment of LP and LL, especially in those at risk for oral candidiasis.

Common benign oral soft tissue masses.

This article reviews some of the more common benign oral soft tissue masses with emphasis on their etiology, epidemiology, clinical presentation, histopathology, and treatment. These lesions include traumatic fibroma, mucocele, warts/papilloma, pyogenic granuloma, peripheral giant cell granuloma, generalized gingival hyperplasia, gingival fibromatosis, lateral periodontal cyst, lipoma, and denture-induced hyperplasia.

Hepatitis C virus and lichen planus: a review.

INTRODUCTION: The relationship between hepatitis C virus (HCV) infection and oral lichen planus (OLP) remains a matter of controversy. It is important to determine whether there is an association between OLP and HCV infection so that guidelines regarding the routine HCV testing of patients with OLP can be developed for clinicians. OBJECTIVES: The objective of this article was to review and summarize the published literature on the association between OLP and HCV and to describe future directions. METHODS: A search of the computerized database MEDLINE (1966-June 2003) was conducted. The bibliographies of articles identified by means of MEDLINE were also searched. Any studies reporting the prevalence of HCV in a group of patients with LP either with or without a control group were included in this review. Also included were studies comparing the clinical and histologic features of LP among patients with and without HCV infection, studies on the presence of HCV within LP lesions, and studies of HCV genotypes among patients with LP. RESULTS: Thirty-two studies conducted in various parts of the world were identified. Study types included prevalence studies on HCV exposure among patients with OLP (0%-62%), prevalence of OLP among patients with HCV infection (1.6%-20%), and case-control studies of this association. In addition, the results from 3 studies on the replication of HCV in the oral mucosa, 3 studies on the genotype of HCV in OLP patients, and 4 studies comparing the clinical and histologic features of OLP in HCV-infected and uninfected individuals have been summarized. CONCLUSION: At present, studies on the association of OLP and HCV provide enough information to raise a number of interesting questions about this association. Important biases-including selection bias; investigator bias due to lack of blinding and the possible resultant nondifferential misclassification of disease; and possible confounding by age in the studies published-make it difficult to draw firm conclusions. However, the need for future studies that take into consideration all these factors in the study methodology is highlighted by this review.

The diagnosis and management of recurrent aphthous stomatitis: a consensus approach.

BACKGROUND: Recurrent aphthous stomatitis, or RAS, is a common oral disorder of uncertain etiopathogenesis for which symptomatic therapy only is available. This article reviews the current data on the etiopathogenesis, diagnosis and management of RAS in a primary care setting. METHODS: The authors reviewed publications on Medline from 1995 through 2000, the period since the last major reviews were published. RESULTS: RAS may have an immunogenetic background owing to cross-reactivity with Streptococcus sanguis or heat shock protein. Predisposing factors seen in a minority include haematinic (iron, folate or vitamin B12) deficiency, stress, food allergies and HIV infection. While topical corticosteroids remain the mainstay for therapy, a number of other immunomodulatory modalities now are available. CONCLUSIONS: There is still no conclusive evidence relevant to the etiopathogenesis of RAS, and therefore therapy can attempt only to suppress symptoms rather than to address the basic issues of susceptibility and prevention. CLINICAL IMPLICATIONS: In the majority of patients, symptomatic relief of RAS can be achieved with topical corticosteroids alone, with other immunomodulatory topical agents or by combination therapy.

The first international consensus on mucous membrane pemphigoid: definition, diagnostic criteria, pathogenic factors, medical treatment, and prognostic indicators.

OBJECTIVE: We aimed to develop consensus-based recommendations for streamlining medical communication among various health care professionals, to improve accuracy of diagnosis and treatment, and to facilitate future investigations for mucous membrane pemphigoid. PARTICIPANTS: Because of the highly specific nature of this group of diseases, the 26 invited participants included either international scholars in the field of mucous membrane pemphigoid or experts in cutaneous pharmacology representing the 3 medical disciplines ophthalmology, oral medicine, and dermatology. EVIDENCE: The first author (L.S.C.) conducted a literature search. Based on the information obtained, international experts who had contributed to the literature in the clinical care, diagnosis, and laboratory investigation for mucous membrane pemphigoid were invited to participate in a consensus meeting aimed at developing a consensus statement. CONSENSUS PROCESS: A consensus meeting was convened and conducted on May 10, 1999, in Chicago, Ill, to discuss the relevant issues. The first author drafted the statement based on the consensus developed at the meeting and the participants' written comments. The draft was submitted to all participants for 3 separate rounds of review, and disagreements were reconciled based on literature evidence. The third and final statement incorporated all relevant evidence obtained in the literature search and the consensus developed by the participants. The final statement was approved and endorsed by all 26 participants. CONCLUSIONS: Specific consensus-based recommendations were made regarding the definition, diagnostic criteria, pathogenic factors, medical treatment, and prognostic indicators for mucous membrane pemphigoid. A system of standard reporting for these patients was proposed to facilitate a uniform data collection.