European guidelines (S3) on diagnosis and management of mucous membrane pemphigoid, initiated by the European Academy of Dermatology and Venereology - Part I.

This guideline on mucous membrane pemphigoid (MMP) has been elaborated by the Task Force for Autoimmune Blistering Diseases of the European Academy of Dermatology and Venereology (EADV) with a contribution of physicians from all relevant disciplines and patient organizations. It is a S3 consensus-based guideline encompassing a systematic review of the literature until June 2019 in the MEDLINE and EMBASE databases. This first part covers methodology, the clinical definition of MMP, epidemiology, MMP subtypes, immunopathological characteristics, disease assessment and outcome scores. MMP describes a group of autoimmune skin and mucous membrane blistering diseases, characterized by a chronic course and by predominant involvement of the mucous membranes, such as the oral, ocular, nasal, nasopharyngeal, anogenital, laryngeal and oesophageal mucosa. MMP patients may present with mono- or multisite involvement. Patients' autoantibodies have been shown to be predominantly directed against BP180 (also called BPAG2, type XVII collagen), BP230, laminin 332 and type VII collagen, components of junctional adhesion complexes promoting epithelial stromal attachment in stratified epithelia. Various disease assessment scores are available, including the Mucous Membrane Pemphigoid Disease Area Index (MMPDAI), the Autoimmune Bullous Skin disorder Intensity Score (ABSIS), the 'Cicatrising Conjunctivitis Assessment Tool' and the Oral Disease Severity Score (ODSS). Patient-reported outcome measurements (PROMs), including DLQI, ABQOL and TABQOL, can be used for assessment of quality of life to evaluate the effectiveness of therapeutic interventions and monitor disease course.

European Guidelines (S3) on diagnosis and management of mucous membrane pemphigoid, initiated by the European Academy of Dermatology and Venereology - Part II.

This guideline has been initiated by the task force Autoimmune Blistering Diseases of the European Academy of Dermatology and Venereology, including physicians from all relevant disciplines and patient organizations. It is a S3 consensus-based guideline that systematically reviewed the literature on mucous membrane pemphigoid (MMP) in the MEDLINE and EMBASE databases until June 2019, with no limitations on language. While the first part of this guideline addressed methodology, as well as epidemiology, terminology, aetiology, clinical presentation and outcome measures in MMP, the second part presents the diagnostics and management of MMP. MMP should be suspected in cases with predominant mucosal lesions. Direct immunofluorescence microscopy to detect tissue-bound IgG, IgA and/or complement C3, combined with serological testing for circulating autoantibodies are recommended. In most patients, serum autoantibodies are present only in low levels and in variable proportions, depending on the clinical sites involved. Circulating autoantibodies are determined by indirect IF assays using tissue substrates, or ELISA using different recombinant forms of the target antigens or immunoblotting using different substrates. The major target antigen in MMP is type XVII collagen (BP180), although in 10-25% of patients laminin 332 is recognized. In 25-30% of MMP patients with anti-laminin 332 reactivity, malignancies have been associated. As first-line treatment of mild/moderate MMP, dapsone, methotrexate or tetracyclines and/or topical corticosteroids are recommended. For severe MMP, dapsone and oral or intravenous cyclophosphamide and/or oral corticosteroids are recommended as first-line regimens. Additional recommendations are given, tailored to treatment of single-site MMP such as oral, ocular, laryngeal, oesophageal and genital MMP, as well as the diagnosis of ocular MMP. Treatment recommendations are limited by the complete lack of high-quality randomized controlled trials.

Preval?ncia e severidade da dor orofacial em gestantes / Prevalence and severity of orofacial pain in pregnant women

Objective: To evaluate the prevalence and severity of oral pain in pregnant women. Methods: A cross-sectional study was conducted on a sample of pregnant women who were receiving dental care at a Dental School. A structured questionnaire about self-perception of oral conditions and the presence and severity of orofacial pain in the mouth or teeth in the last 6months was applied. Results: 80 pregnant women participated in the study and, of these, 58.8% reported having a problem with their teeth, 31.3% periodontal problems, 61.3% bad taste in mouth and 62.5% bad breath. Only 22.5% of pregnant women did not exhibit any form of oral pain. The most frequently reported pains were: pain with hot/cold liquids or sweets (56.2%) and spontaneous toothache (38.8%). With regard to severity, mild and moderate pain were the most frequently reported, but there was a group of 23.8% of pregnant women with severe or very severe pain caused by hot or cold liquids and 18.8% in the same condition in relation to spontaneous pain. Conclusion: The results of this study showed that, despite the high prevalence of pain detected in the pregnant women, severity was low and referred to specific situations.

Objetivo: Avaliar a preval?ncia e a severidade da dor orofacial em gestantes. M?todos: O estudo transversal foi realizado em uma amostra de gestantes em atendimento odontol?gico em uma faculdade de odontologia. Foi aplicado question?rio estruturado com perguntas sobre a auto percep??o das condi??es bucais e a presen?a e a severidade da dor orofacial sentida na boca ou dentes nos ?ltimos 6 meses. Resultados: Participaram do estudo 80 gestantes e, destas, 58,8% afirmaram ter algum problema nos dentes, 31,3% problema na gengiva, 61,3% gosto ruim na boca e 62,5% mau h?lito. Apenas 22,5% n?o apresentaram nenhum tipo de dor orofacial. As dores mais relatadas foram: dor com l?quidos quentes, frios ou doces (56,2%) e dor de dente espont?nea (38,8%). Quanto ? severidade, as dores de intensidade leve e moderada foram as mais frequentes, mas havia um grupo de 23,8% das gestantes com dores intensas ou muito intensas provocadas por l?quidos frios ou quentes e 18,8% na mesma condi??o em rela??o ? dor espont?nea. Conclus?o: Os resultados do estudo mostraram que, apesar da alta preval?ncia de dor orofacial encontrada, a severidade foi baixa e relacionada a algumassitua??es espec?ficas.