Ocular cicatricial pemphigoid. From a rheumatology and ophthalmology point of view / Penfigoide ocular cicatrizal, enfoque diagnóstico y terapéutico integral entre el oftalmólogo y el reumatólogo

ABSTRACT Ocular cicatricial pemphigoid (OCP) is a chronic, immune-mediated, bullous, cicatricial disease within the spectrum of mucocutaneous membranous pemphigoids (MMP). Although the diagnosis is often ophthalmological, due to the autoimmune nature of the pathology, it requires a joint approach with rheumatologists and immunologists. The objective of this narrative review was to explore the evidence available in the literature from 2000 to 2020 with respect to clinical manifestations, diagnosis, and treatment. The clinical presentation varies widely, from mild cases with slow progression of years of progression, to severe cases with a torpid and rapidly progressive evolution to fibrosis, refractory to multiple treatments. A com plete evaluation of the patient will help guide the diagnosis. The gold standard for diagnosis is conjunctival biopsy with direct immunofluorescence, although on occasions it can be reached if the symptoms are characteristic. Treatment is local and systemic according to its severity and evolution. The evidence on topical and systemic therapeutics is obtained mainly from uncontrolled observational and experimental studies. Immunomodulatory therapy has made it possible to preserve vision and, in many cases, prevent sequelae. The evolu tion is linked to the early diagnosis and immunosuppressive treatment, so it is essential to be aware of this disease, the diagnostic methods, as well as the immunomodulating and immunosuppressive therapies available.

RESUMEN El penfigoide ocular cicatrizal (POC) es una enfermedad crónica, inmunomediada ampollar, mucosinequiante, comprendida dentro del espectro de penfigoides membranosos mucocutáneos (PMM). El diagnóstico es, con frecuencia, oftalmológico, pero debido al carácter autoinmune de la patología, requiere el abordaje en conjunto con reumatólogos e inmunólogos. El objetivo de esta revisión narrativa fue explorar la evidencia disponible en la literatura, desde el año 2000 hasta el 2020, en lo que respecta a sus manifestaciones clínicas, diagnóstico y tratamiento. La presentación clínica varía ampliamente, desde casos leves con progresión lenta de años de evolución hasta casos severos con evolución tórpida y rápidamente progresiva a la fibrosis, refractarios a múltiples tratamientos. Una evaluación completa del paciente ayudará a guiar el diagnóstico. El estándar de oro diagnóstico es la biopsia conjuntival con inmunofluorescencia directa, si bien en ocasiones puede diagnosticarse por la clínica característica. El tratamiento es local y sistêmico de acuerdo con su severidad y evolución. En los últimos 20 anos, la evidencia sobre los tratamientos tópicos y sistêmicos corresponde en su mayoría a estudios observacionales y experimentales no controlados. Los métodos de tratamiento inmunomoduladores han permitido preservar la visión y, en muchos casos, prevenir secuelas. La evolución está ligada al diagnóstico temprano y a los tratamientos disponibles, por lo que es fundamental el conocimiento de esta patología, los métodos diagnósticos y los tratamientos inmunomoduladores e inmunosupresores.

Penfigoide de las mucosas a lo largo de los años en nuestro Servicio / Mucous membrane pemphigoid through the years in our Department

Introducción: el penfigoide de las mucosas (PM), antes llamado mucoso, cicatrizal o mucosinequiante, representa un grupo heterogéneo de enfermedades ampollares autoinmunes inflamatorias crónicas que comprometen las mucosas o la piel, con tendencia a dejar secuelas cicatrizales. Existen autoanticuerpos contra distintos componentes de la zona de la membrana basal (BPAG1, BPAG2, integrina α6ß4, laminina 332, colágeno VII, entre otros), por lo que la inmunofluorescencia directa (IFD) es de suma importancia, así como la clínica, para su diagnóstico.Objetivo: realizar una revisión de los casos de PM diagnosticados durante un período de 24 años (enero de 1997- marzo de 2021) en el Sector de Enfermedades Ampollares del Hospital Ramos Mejía para determinar la epidemiología, la clínica y la terapéutica de esta enfermedad.Diseño: estudio retrospectivo descriptivo y observacional, en el que se analizaron las características clínicas e inmunopatológicas de 34 pacientes con diagnóstico de PM atendidos en el Servicio de Dermatología del Hospital Ramos Mejía desde enero de 1997 hasta marzo de 2021. Materiales y métodos: mediante las historias clínicas y los regis-tros iconográficos, se evaluaron las siguientes variables: prevalencia del diagnóstico de PM en los pacientes atendidos en el Sector, sexo, edad, antecedentes personales, mucosas afectadas, tiempo de evolución hasta el diagnóstico, hallazgos en la IFD, seguimiento clínico y tratamientos instaurados. Resultados: se estudió la evolución clínica de 34 pacientes diagnosticados con PM (5,3% del total de pacientes evaluados en el Sector de Patologías Ampollares). El sexo más afectado fue el femenino y la edad promedio en el momento del diagnóstico fue de 64 años. El 70,6% de los pacientes presentaron comorbilidades asociadas como hipertensión e hipotiroidismo. La mayoría refirió algún evento emocional como factor desencadenante. El sitio más comprometido fue la mucosa ocular y la cavidad oral fue la segunda en frecuencia. El tiempo de evolución promedio hasta el momento del diagnóstico fue de 4 años y 11 meses. El hallazgo más frecuente en la IFD fue la IgG lineal. El 17,6% de los pacientes interrumpieron el seguimiento clínico. El tratamiento más utilizado fue el mofetil micofenolato, con el que se obtuvo buena respuesta terapéutica. Conclusiones: el PM es una enfermedad autoinmune infrecuente que compromete las mucosas y, ocasionalmente, la piel. En este estudio, se observó que la principal mucosa afectada fue la conjuntival, a diferencia de lo referido en la bibliografía internacional dermatológica. El diagnóstico interdisciplinario temprano es fundamental para evitar las secuelas irreversibles.

Introduction: mucous membrane pemphigoid (MMP), also known as benign mucous membrane pemphigoid, cicatricial or mucosynechial pemphigoid, belongs to an heterogeneous group of chronic inflammatory autoimmune blistering diseases, which involves the mucous membranes (oral, ocular, pharyngeal, nasal, esophageal, laryngeal and anogenital) and/ or skin with tendency to scar formation. There are autoantibodies against different components of the basement membrane zone (BPAG 1- BPAG2, Integrin α6ß4, Laminin 332, Col VII, among others). The direct immunofluorescence (DIF) will be of paramount importance, as well as the clinical diagnosis.Objective: review the cases diagnosed with mucous membrane pemphigoid for 24 years (January 1997- March 2021) in the Blistering Disease Clinic at the Dermatology Department at the Ramos Mejia Hospital to establish the epidemiologic, clinic presentation and available treatments in this pathology.Design: retrospective descriptive and observational study of the clinical and immunopathological characteristics of 34 patients with MMP that were treated at the Blistering Disease Clinic at the Dermatology Department at the Ramos Mejia Hospital between January 1997 and March 2021.Materials and methods: though the medical histories and the photographic registries, we evaluated the following variables: prevalence of MMP within the patients that came to consult at the Blistering Disease Clinic at the Dermatology Department, sex, age, personal history, the affected mucous, evolution time until the diagnosis, direct immunofluorescence findings, clinical follow-ups and treatments.Results: we studied the clinical evolution of 34 patients diagnosed with MMP at our institution (5.3% from the total of patients at the Blistering Disease Clinic).The most affected gender was female and the average age at diagnosis was 64 years. 70.6% presented comorbidities such as hypertension and hypothyroidism. Most of our patients referred an emotional triggering event. The most affected membrane mucous was the ocular one and the oral was the second one. The delay in diagnosis was 4 years and 11 months. Linear deposits of IgG was the most frequent result in the direct immunofluorescence. 17.6% did not continue clinical follow-up. Mycophenolate mofetil was the most used drug with a good therapeutic response. Conclusions: MMP is a rare autoimmune disease that affects mucous membrane and occasionally the skin. In this study, the ocular involvement was the most frequent one, differing with the international reports. The early interdisciplinary diagnosis is essential to avoid irreversible sequelae.

Afecciones perianales no infecciosas, no neoplásicas, del adulto / Noninfectious, non-neoplasic adult perianal affections

Las enfermedades perianales del adulto, de carácter no infeccioso y no neoplásico, son un motivo de consulta poco frecuente. Se caracterizan por la variedad de su etiología y de su sintomatología clínica, y plantean dificultad en el diagnóstico y en la terapéutica. El objetivo del presente trabajo es abordar una patología que plantea la necesidad de una intervención interdisciplinaria. Se incluyen consideraciones anatomopatológicas, clínicas y terapéuticas. (AU)

Noninfectious, non- neoplasic perianal affections are uncommon diseases. They are characterized by the variety of the etiology and clinical symptomatology, posing difficulty in diagnosis and therapeutics. The objective of this paper is to address a pathology that raises the need for interdisciplinary intervention anatomopathological, clinical and therapeutic considerations are included. (AU)

Síndrome Sjögren y penfigoide de las membranas mucosas en paciente con antecedente de timoma: Reporte de caso y revisión de literatura / Sjögren Syndrome and Mucous Membrane Pemphigoid in a Patient with a History of Thymoma: Case Report and Literature Review

RESUMEN Los timomas son las neoplasias mediastínicas más comunes en adultos. Los pacientes con timoma son frecuentemente diagnosticados con síndromes paraneoplásicos y generalmente están asociados con varias condiciones autoinmunes. El síndrome de Sjögren (SS) es un trastorno inflamatorio autoinmune, que se caracteriza por la destrucción mediada por linfocitos de las glándulas exocrinas, que conduce a la ausencia de secreción glandular, en particular las salivales y las lagrimales; la hiposalivación puede variar desde una ligera reducción en el flujo de saliva con molestias transitorias, a un grave deterioro de la salud oral y malestar psicológico. El penfigoide de membranas mucosas (PMM) es una enfermedad autoinmune crónica, muestra predominante afectación de la mucosa, afectando con mayor frecuencia la cavidad oral, seguida de la conjuntiva, la cavidad nasal y el esófago; intraoralmente incluye gingivitis descamativa, vesículas, erosiones cubiertas por pseudomembranas y úlceras. El objetivo de este trabajo es presentar el primer caso de una paciente con antecedente de timoma, la cual, conjuntamente presentó SS y PMM; además evidenciar la importancia del manejo multidiciplinario para un diagnóstico preciso y temprano, ya que así, aumenta la eficiencia y la eficacia de la estrategia de tratamiento.

ABSTRACT Thymomas are the most common mediastinal neoplasms in adults. Patients with thymoma are frequently diagnosed with paraneoplastic syndromes and are generally associated with several autoimmune conditions. Sjögren's syndrome (SS) is an autoimmune inflammatory disorder, characterized by the lymphocyte-mediated destruction of the exocrine glands, which leads to the absence of glandular secretion, particularly salivary and lacrimal; hyposalivation can vary from a slight reduction in the flow of saliva with transient discomfort, to a serious deterioration of oral health and psychological distress. Mucous membrane pemphigoid (MMP) is a chronic, autoimmune disease that shows predominant mucosal involvement, most commonly affecting the oral cavity, followed by the conjunctiva, the nasal cavity and the esophagus; intraorally includes scaly gingivitis, vesicles, erosions covered by pseudomembranes and ulcers. The objective of this work is to present the first case of a patient with a history of thymoma, which, jointly presented SS and MMP; also highlight the importance of multidisciplinary management for an accurate and early diagnosis, as this increases the efficiency and efficacy of the treatment strategy.

Conjuntivitis cicatrizal recalcitrante: Tratamiento de nueve pacientes con rituximab / Recalcitrant cicatrizing conjunctivitis. Treatment of nine patients with rituximab

La conjuntivitis cicatrizal es la consecuencia de distintas enfermedades oculares. Entre ellas, las más graves son el penfigoide cicatrizal y el síndrome de Stevens-Johnson crónico. El tratamiento de estas enfermedades con corticoides e inmunosupresores es habitualmente exitoso, pero unos pocos pacientes siguen un curso recalcitrante. En los últimos años se introdujo el uso de rituximab, asociado o no a gammaglobulina endovenosa, en forma abierta, para el control de la inflamación conjuntival. Describimos aquí el tratamiento de siete pacientes con penfigoide y dos con Stevens-Johnson recalcitrante, con rituximab. Ocho recibieron también gammaglobulina y todos alcanzaron la remisión de la actividad. Tres recayeron y recibieron dos o tres nuevos cursos de la medicación con mejoría sintomática. El rituximab probó ser una droga efectiva para el tratamiento de la conjuntivitis cicatrizal crónica recalcitrante.

Cicatrizing conjunctivitis is the final consequence of several diseases. The most severe among them are cicatricial pemphigoid and chronic Stevens-Johnson syndrome. Systemic immunosuppressive drugs and steroids are usually an effective approach to these diseases. However, a few patients follow a recalcitrant course unremitting to usual therapy. We describe the treatment with rituximab of seven patients with cicatricial pemphigoid and two with chronic Stevens-Johnson syndrome. Eight of them also received gammaglobulin and all achieved clinical remission. Three relapsed and required two or three new courses of rituximab with good control of disease activity. Rituximab proved to be an efficacious drug for chronic recalcitrant cicatrizing conjunctivitis.

Consensus on the treatment of autoimmune bullous dermatoses: bullous pemphigoid, mucous membrane pemphigoid and epidermolysis bullosa acquisita - Brazilian Society of Dermatology

Abstract: Bullous pemphigoid, mucous membrane pemphigoid and epidermolysis bullosa acquisita are subepidermal autoimmune blistering diseases whose antigenic target is located at the basement membrane zone. Mucous membrane pemphigoid and epidermolysis bullosa acquisita can evolve with cicatricial mucosal involvement, leading to respiratory, ocular and/or digestive sequelae with important morbidity. For each of these dermatoses, a literature review covering all therapeutic options was performed. A flowchart, based on the experience and joint discussion among the authors of this consensus, was constructed to provide treatment orientation for these diseases in Brazil. In summary, in the localized, low-risk or non-severe forms, drugs that have immunomodulatory action such as dapsone, doxycycline among others may be a therapeutic option. Topical treatment with corticosteroids or immunomodulators may also be used. Systemic corticosteroid therapy continues to be the treatment of choice for severe forms, especially those involving ocular, laryngeal-pharyngeal and/or esophageal mucosal involvement, as may occur in mucous membrane pemphigoid and epidermolysis bullosa acquisita. Several immunosuppressants are used as adjuvant alternatives. In severe and recalcitrant cases, intravenous immunoglobulin is an alternative that, while expensive, may be used. Immunobiological drugs such as rituximab are promising drugs in this area. Omalizumab has been used in bullous pemphigoid.

An unusual presentation of ocular rosacea / Uma apresentação incomum da rosácea ocular

ABSTRACT Rosacea is a chronic, progressive disease of unknown cause affecting the eye and the facial skin. Ocular rosacea is often underdiagnosed if the ophthalmologist does not inspect the patient's face adequately during the ocular examination. Severe ocular complications and blindness can occur if the treatment is delayed because of non-diagnosis of the rosacea. Here, we present a case of ocular rosacea in a 78-year-old Caucasian woman. Based on the ocular lesions, which preceded cutaneous involvement, she was misdiagnosed as having ocular cicatricial pemphigoid initially. This case emphasizes the difficulty in diagnosis when ocular findings precede those of skin manifestations, and rosacea should be kept in mind in the differential diagnosis of chronic cicatrizing conjunctivitis.

RESUMO A rosácea é uma doença crônica e progressiva de causa desconhecida que afeta o olho e a pele do rosto. A rosácea ocular é muitas vezes pouco diagnosticada se o oftalmologista não inspecionar adequadamente o rosto do paciente durante o exame ocular. Podem ocorrer complicações oculares graves e cegueira se o tratamento for adiado devido ao não diagnóstico da rosácea. Um caso de rosácea ocular em uma mulher caucasiana de 78 anos de idade é apresentado. Com base nas lesões oculares, que precederam o envolvimento cutâneo, ela foi mal diagnosticada como penfigóide cicatricial ocular inicialmente. Este caso enfatiza a dificuldade de diagnóstico quando os achados oculares precedem aqueles das manifestações da pele e um diagnóstico de rosácea deve ser mantido em mente no diagnóstico diferencial da conjuntivite cicatrizante crônica.

Microscopia confocal a laser na avaliação in vivo da gengivite descamativa: padrões no penfigóide das membranas mucosas, pênfigo vulgar e líquen plano oral / Confocal microscopy in the in vivo evaluation of desquamative gingivitis: patterns in mucous membrane pemphigoid, pemphigus vulgaris and oral lichen planus

Introdução: Gengivite descamativa (GD) se refere a uma manifestação clínica associada com diversas doenças mucocutâneas. Suas causas mais comuns são penfigóide das membranas mucosas (PMM), pênfigo vulgar (PV) e líquen plano oral (LP). A diagnose específica é melhor estabelecida através de avaliação histopatológica e de imunofluorescência. Objetivos: Examinar casos de gengivite descamativa utilizando microscopia confocal a laser e comparar os achados com aqueles encontrados na gengiva normal. Além disso, comparar os achados de microscopia confocal da gengivite descamativa com os da histopatologia convencional das lesões biopsiadas a fim de estabelecer critérios para este método diagnóstico não invasivo. Método: Doentes com manifestações clínicas de gengivite descamativa foram incluídos, totalizando quarenta e três casos. A microscopia confocal foi realizada na gengiva de um indivíduo saudável e nas lesões gengivais. Todas as lesões sem exame histopatológico prévio foram biopsiadas a fim de permitir uma correlação entre a microscopia confocal e a histopatologia. Resultados: O exame de microscopia confocal das lesões suspeitas de penfigóide das membranas mucosas revelou uma separação ao nível da junção dermo-epidérmica, preenchida por pequenas estruturas brilhantes, interpretadas como hemáceas. Os aspectos histopatológicos e de imunofluorescência confirmaram o diagnose. Para os casos de pênfigo vulgar, os achados da microscopia confocal foram de fenda intraepitelial com células arredondadas interpretadas como queratinócitos acantolíticos. Hiperqueratose e espongiose, associadas com infiltrado inflamatório, caracterizado por células pequenas e brilhantes permeando a estrutura intraepitelial de queratinócitos conhecida como favo de mel foram vistos no líquen plano. Estruturas arredondadas pouco brilhantes, interpretadas como queratinócitos necróticos, e estruturas estelares também pouco brilhantes, interpretadas como melanófagos, foram encontrados...

Background: Desquamative gingivitis refers to a clinical manifestation associated with several mucocutaneous disorders. The most common are mucous membrane pemphigoid, pemphigus vulgaris and lichen planus. Their specific diagnosis is better established by histopathological and immunofluorescence evaluation. Objective: To examine cases of desquamative gingivitis using reflectance confocal microscopy and compare the findings with those of normal gingiva. Moreover, confocal microscopy findings in desquamative gingivitis were compared to conventional histopathology of the biopsied lesions, in order to establish criteria for this non-invasive diagnostic technique. Methods: Patients with clinical manifestations of desquamative gingivitis were included, totalizing forty-three cases. Reflectance confocal microscopy was performed the gingival of a healthy person and on gingival lesions. All lesions were biopsied in order to perform a reflectance confocal microscopy- histopathologic correlation. Results: Reflectance confocal microscopy exam of the gingival lesions suspected of mucous membrane pemphigoid revealed a separation at the level of dermal-epidermal junction, filled with small bright structures interpreted as blood cells. Histopathological and immunofluorescence aspects confirmed the diagnosis. For pemphigus vulgaris, reflectance confocal microscopy aspects were of intraepithelial cleft with round detached cells interpreted as acantholytic keratinocytes, similar to the histopathological features. Hyperkeratosis and spongiosis associated with infiltration of inflammatory cells, recognized as small bright cells intermingling the honeycomb keratinocyte epithelial structure, were seen in lichen planus. Mild bright round structures interpreted as necrotic keratinocytes and mild bright stellate structures, interpreted as melanophages in the dermis were also seen. These features were present in histopathology, confirming the diagnosis of lichen...

Penfigoide benigno de las mucosas: presentación de un caso / Bening pemphigoid of the mucous membranes: report of a clinical case

Fundamento: el penfigoide benigno de las mucosas es una enfermedad que afecta principalmente a la mucosa oral y ocular. El daño a la mucosa bucal es observado en la mayoría de los pacientesy cuando se presenta en la gingiva, produce un cuadro parecido al de una gingivitis descamativa. Objetivo: relatar un caso de penfigoide benigno de las mucosas donde el paciente presenta las lesiones solo en boca. Caso clínico: varón de 30 años, fumador, se presentó con diversas lesiones bucales de naturaleza vesiculoampollar, sangramiento espontáneo y provocado e intenso enrojecimiento de la encía. Al examen físico se observó un estado bucal desfavorable, caries, placa dentobacteriana, sarro, obturaciones caídas, recesiones gingivales generalizadas. No existió toma del estado general y los estudios analíticos dentro de límites normales. Se descartó lesiones oftalmológicas. Se discutió el diagnóstico y manejo de esta enfermedad poco habitual en hombres jóvenes. Se utilizó la prednisonapor vía oral, se dio el alta de los servicios dentales y se mantuvo una óptima higiene bucal por parte del paciente y vigilancia estricta del periodoncista. El penfigoide es una enfermedad grave y de larga evolución. Conclusiones: El paciente está en tratamiento, con una evolución satisfactoria.

Tratamiento sistémico del penfigoide cicatrizal ocular / Systemic treatment of ocular cicatricial pemphigoid

El penfigoide cicatrizal ocular (PCO) es una enfermedad ampollar autoinmune que produce daño conjuntival grave. Se conoce poco acerca de la respuesta del PCO al tratamiento inmunosupresor. Describimos un grupo de 76 pacientes con PCO, 62 mujeres y 14 hombres. La edad media al diagnóstico fue de 67 ± 14 años, con un retraso de 7.5 ± 10 años. Sesenta se siguieron en nuestro servicio por 19 ± 21 meses. De 51 en quienes se describe la gravedad de la enfermedad al inicio del tratamiento, fue leve en 19 pacientes, moderada en 19, grave en cinco y muy grave en ocho. Las drogas mayormente prescriptas fueron dapsona en 35 pacientes, de los que 23 la discontinuaron por efectos adversos, y metotrexate en 42, de los que nueve lo suspendieron. Otros recibieron azatioprina, ciclofosfamida y ciclosporina. A 17 se les indicaron corticoides orales, además del inmunosupresor. Cuatro combinaron dos drogas para controlar la enfermedad. Tres pacientes refractarios recibieron gammaglobulina EV con buena respuesta. De 48 evaluados, 39 mostraron mejoría, ocho no tuvieron cambios y uno progresó. En nuestra experiencia, metotrexate y azatioprina son efectivos, con baja toxicidad. Dapsona es útil en casos leves, con efectos adversos frecuentes. La gammaglobulina EV fue efectiva en casos refractarios.

Ocular cicatricial pemphigoid (OCP) is a blistering autoimmune disease that can produce severe conjunctival damage. Its response to immunosuppressive treatment is poorly known. We describe a group of 76 patients, 62 women and 14 men. Mean age at diagnosis was 67±14 years old, with a delay to diagnosis of 7.5±10 years. Sixty patients continued their follow up in our services for 19±21 months. Nineteen out of 51 had mild disease, 19 moderate, 5 severe and 8 very severe at onset of treatment. The more frequently prescribed drugs were dapsone, in 35 (23 discontinued it because of adverse effects), and methotrexate in 42 patients, nine of them stopped it. Other patients received azathioprine, cyclophosphamide and ciclosporine. Seventeen received oral steroids in addition to immunosuppresive drugs. Four patients combined two immunosupressive drugs to control their disease. In three refractory cases IV immunoglobulin (Ig) was administered with good response. From 48 evaluated patients, 39 improved with treatment, eight remained stable and one progressed. In our experience, methotrexate and azathioprine were effective drugs, with low toxicity. Dapsone was useful in mild cases, with frequent adverse effects. IVIg was effective for refractory cases.

Estudo epidemiológico das doenças dermatológicas imunologicamente mediadas na cavidade oral / An epidemiological study of immune-mediated skin diseases affecting the oral cavity

FUNDAMENTO: As doenças dermatológicas imunologicamente mediadas compõem diversas patologias que apresentam formas variadas de manifestação no organismo. OBJETIVO: Foi proposição desta pesquisa, estabelecer a prevalência das principais doenças dermatológicas imunologicamente mediadas que apresentam manifestação oral. MÉTODOS: Foram avaliados laudos histopatológicos de 10.292 casos arquivados no Serviço de Anatomia Patológica da Disciplina de Patologia Oral da Universidade Federal do Rio Grande do Norte, no período de 1988 a 2009. Dos casos diagnosticados como algum tipo de doença em estudo, coletaram-se dados clínicos como sexo, idade, raça, sítio anatômico e sintomatologia das doenças. RESULTADOS: Do total de casos registrados, no serviço supracitado, 82 (0,8 por cento) corresponderam a doenças dermato lógicas imunologicamente mediadas com manifestação na cavidade oral. As doenças encontradas neste estudo foram: líquen plano oral, pênfigo vulgar e penfigoide benigno das membranas mucosas, sendo o líquen plano oral a lesão mais prevalente, representando 68,05 por cento dos casos analisados, dos quais 64,3 por cento apresentavam-se em mu lheres, sendo a mucosa jugal o sítio anatômico mais acometido (46,8 por cento). CONCLUSÃO: A ocorrência de doenças dermatológicas imunologicamente mediadas que apresentam manifestação oral ainda é um fato incomum, semelhante ao observado na maioria das regiões mundiais. No entanto, a busca pelo diagnóstico precoce é um requisito essencial para a condução do tratamento dessas doenças, tendo em vista o possível comprometimento sistêmico do organismo nos pacientes.

BACKGROUND: Immune-mediated skin diseases encompass a variety of pathologies that present in different forms in the body. OBJECTIVE: The objective of this study was to establish the prevalence of the principal immune-mediated skin diseases affecting the oral cavity. METHODS: A total of 10,292 histopathology reports stored in the archives of the Anatomical Pathology Laboratory, Department of Oral Pathology, Federal University of Rio Grande do Norte, covering the period from 1988 to 2009, were evaluated. For the cases diagnosed with some type of disease relevant to the study, clinical data such as the gender, age and ethnicity of the patient, the anatomical site of the disease and its symptomatology were collected. RESULTS: Of all the cases registered at the above-mentioned service, 82 (0.8 percent) corresponded to immune-media ted skin diseases with symptoms affecting the oral cavity. The diseases found in this study were: oral lichen planus, pemphigus vulgaris and benign mucous membrane pemphigoid. Oral lichen planus was the most common lesion, comprising 68.05 percent of the cases analyzed. Of these cases, 64.3 percent were women and the cheek mucosa was the anatomical site most commonly affected (46.8 percent). CONCLUSION: Immune-mediated skin diseases affecting the oral cavity continue to be rare, the prevalence found in this study being similar to that reported for the majority of regions worldwide. Nevertheless, early diagnosis is indispensable in the treatment of these diseases, bearing in mind that systemic involvement is possible in these patients.

Penfigoide de membranas mucosas com estenose esofágica grave / Mucous membrane pemphigoid with severe esophageal stricture

O penfigoide de membranas mucosas é entidade nosológica encarada como um fenótipo, que engloba várias dermatoses autoimunes com lesões bolhosas subepidérmicas, ocorrendo predominantemente nas membranas mucosas, com êxito cicatricial. O acometimento esofágico no penfigoide de membranas mucosas é raro e observado em pacientes com lesão disseminada. As alterações mais comuns são múltiplas membranas ou constrições esofagianas. No presente relato, os autores apresentam paciente com PMM sem lesões cutâneas e estenose esofágica grave, que entrou em remissão após uso de imunoglobulina venosa.

Mucous membrane pemphigoid (MMP) is a rare nosological entity. MMP consists of a clinical phenotype in which several autoimmune subepidermal bullous diseases are classified. It occurs predominantly in the mucous membranes and usually results in scarring. Esophageal involvement in MMP is rare and is generally seen in patients in whom lesions are widespread. The most common alterations are multiple esophageal membranes or strictures. In the present case, the authors report on a patient with MMP without any skin lesions and with severe esophageal strictures who went into remission following use of intravenous immunoglobulin.

Seguimiento multidisciplinario en el control del penfigoide de membranas mucosas / Multidisciplinary follow-up in mucous membranes pemphigoid control

El término penfigoide de membranas mucosas designa un grupo de enfermedades vesículo-bulosas autoinmunes crónicas, que afectan predominantemente mucosas. El presente artículo describe dos casos clínicos de penfigoide de membranas mucosas con compromiso gingival y tuvo como objetivo resaltar la importancia de un correcto diagnóstico de la lesión y discutir la conducta terapéutica. Fueron realizados diversos exámenes complementares como, examen de Nikolsky, biopsia incisional, investigación de células LE y de anticuerpos antinucleares, además de la evaluación de médicos especializados en dermatología y oftalmología. Fue fundamental la substitución de dentífricos, la orientación nutricional y psicológica, el cuidado con la higiene, además del uso de corticoides tópicos. Hubo periodos de remisión y exacerbación del cuadro clínico durante el seguimiento, fue necesario el ajuste en la terapéutica y refuerzo en los cuidados con la higiene bucal. Es muy importante la interacción multidisciplinaria en la atención de estos casos para el control del tratamiento y seguimiento, además de reforzar todas las orientaciones y cuidados en lo que se refiere a la salud bucal y cautela en el uso de corticoides(AU)

The term Mucous Membrane Pemphigoid includes a group of chronic and autoimmune vesiculobullous diseases involving mainly the mucosa. The aim of present paper is to describe two clinical cases of Mucous Membrane Pemphigoid with gingival involvement to arrive to a proper diagnosis of the lesion and also to discuss the therapeutic behavior. Different complementary examinations were conducted including Nikolsky test, incisional biopsy, the LE cells study and of antinuclear antibodies, as well as the evaluation of dermatologists and ophthalmologists. It was necessary the replacement of toothpastes, the nutritional and psychological guiding, the hygiene care and the use of topic corticoids. There were periods of remission and exacerbation of clinical picture during follow-up being necessary the therapeutics fitting and the oral hygiene care reinforcement. It is important the multidisciplinary interaction in the care of these cases for treatment and follow-up control, as well as to reinforce all orientations and cares related to oral hygiene and the caution in the use of corticoids(AU)

Prevalência e aspectos clínicos de doenças gengivais de origem imune e influência da placa bacteriana na melhora dos sinais e sintomas de lesões gengivais de líquen plano bucal / Prevalence and clinical aspects of gingival diseases of immune origin and influence of plaque in the improvement of signs and symptoms of gingival lesions of lichen planus

O presente projeto de pesquisa tem como objetivo avaliar a prevalência, os aspectos clínicos, a severidade e os fatores de risco das manifestações gengivais de doenças de origem imunológica, além de avaliar a efetividade de medidas de controle da placa bacteriana na melhora dos sinais e sintomas de pacientes que apresentam líquen plano bucal com envolvimento gengival. Para isso, foram elaborados três estudos. No primeiro deles, com o objetivo de avaliar a epidemiologia da gengivite descamativa em um Serviço de Medicina Bucal, foram selecionados casos de líquen plano, penfigóide das membranas mucosas, pênfigo vulgar, lupus eritematoso e eritema multiforme com manifestações gengivais, atendidos no Serviço de Medicina Bucal da Faculdade de Odontologia de Araraquara - Unesp, no período de janeiro de 1995 a dezembro de 2004. Dos 4.776 prontuários avaliados, 48 apresentaram lesões de manifestações gengivais, sendo 68.8% mulheres, com idade média de 43.3 anos. As doenças mais comuns encontradas na amostra foram o líquen plano, o penfigóide das membranas mucosas e o pênfigo vulgar. A maioria dos pacientes avaliados apresentava sintomatologia dolorosa (83.3%) e apenas 35.4% dos casos apresentaram lesões restritas à gengiva. Nossos resultados contribuíram para mostrar a importância do diagnóstico precoce e consequentemente do adequado tratamento das manifestações gengivais associadas a doenças de origem imunológica. O Segundo estudo, também retrospectivo, objetivou avaliar a prevalência de lesões gengivais em portadores de líquen plano bucal, bem como os aspectos e fatores de risco associados, a partir de dados de prontuários de pacientes atendidos no mesmo Serviço, no período compreendido entre janeiro de 1995 e dezembro de 2009. Dos 9.475 prontuários analisados, 84 apresentaram...

This research project aims to assess the prevalence, clinical aspects, severity and risk factors of gingival disease of immune origin, and to evaluate the effectiveness of control plaque in the improvement of signs and symptoms of patients lichen planus presenting with gingival involvement. For this, we prepared three studies. In the first, in order to assess the epidemiology of desquamative gingivitis in a Department of Oral Medicine, were selected cases of lichen planus, oral pemphigoid, pemphigus vulgaris, lupus erythematosus and erythema multiforme in the gingiva, the Service's Oral Medicine Faculty of Dentistry of Araraquara - UNESP, from January 1995 to December 2004. Of 4,776 records evaluated, 48 had lesions associated with desquamative gingivitis, and 68.8% women, mean age of 43.3 years. The most common disease found in the sample were lichen planus, oral pemphigoid and pemphigus vulgaris. Much of the patients had painful symptoms (83.3%) and only 35.4% of cases had lesions limited to the gingiva. Despite the small sample size, the results helped show the importance of early diagnosis and consequently the appropriate treatment of diseases associated with desquamative gingivitis. The second study, also retrospective study aimed to assess the prevalence of gingival lesions in patients with lichen planus, as well as the aspects and associated risk factors in medical records of patients attending the same service in the period between January 1995 and December 2009. Of 9,475 charts analyzed, 84 had lichen...

Penfigoide de membranas mucosas: a propósito de dos casos clínicos / Mucous membrane pemphigoid: report of two cases

El penfigoide de membranas mucosas representa un heterogéneo grupo de enfermedades ampollares, autoinmunes y crónicas, que afecta las mucosas y/o la piel. Es una patología que origina como secuelas, cicatrices que motivan importantes incapacidades funcionales en las mucosas involucradas, provocando ceguera, dificultades en la masticación, la deglución o en la respiración. Su diagnóstico, tratamiento y control deben ser realizados por un equipo multidisciplinario. Se presentan dos casos clínicos de penfigoide de membranas mucosas de localización oral por considerarlos de interés, dadas sus manifestaciones clínicas y la diferente evolución en cada uno de ellos. Se describen los métodos de diagnóstico y tratamientos durante 4 años de seguimiento y control, realizados en la Cátedra de Semiología y Patología BMF, Clínica de Estomatología de la Facultad de Odontología. Universidad de la República.

Mucous membrane pemphigoid describes an heterogeneous group of chronic, autoimmune, blistering diseases that affects the mucous membranes with or without skin involvement. Scarring is the hallmark, leading to blindness, poor nutrition and difficulties to eat, to swallow and to breathe. The diagnosis, treatment and control must be made by a multidisciplinary team. We present two cases of mucous membrane pemphigoid of oral location, considering the interest because of the clinical symptoms and the different clinical course in each of them. We refer the methods for diagnosis, treatments performed in the 4 years of follow up andcontrol, assisted by the Chair of Semiology and Pathology BMF, Clinic of Stomatology, School of Dentistry, Universidad de la República.

Redução das lesões orais no penfigoide benigno das mucosas / Reduction of oral lesions on mucous membrane phemphigoid

O penfigoide benigno de mucosa (PBM) é uma doença sistêmica, mucocutânea, vesículobolhosa que pode acometer a mucosa de revestimento da cavidade bucal, assim como a pele, conjuntiva ocular, laringe, nasofaringe, esôfago, trato geniturinário e ânus. Não é uma condição comum e é capaz de provocar sinais e sintomas que variam de leve a moderado, podendo muitas vezes, exigir controles terapêuticos prolongados por toda vida do paciente. Sua etiologia permanece obscura, mas sabe-se que possui etiopatogenias de caráter autoimune. Este trabalho é um relato de caso clínico de PBM com manifestação bucal, em que a aplicação de manobras de cuidados bucais resultou em melhora clínica da paciente.

Management of mucous membrane pemphigoid with dapsone plus weekly steroid injections in a young patient: a case report / Tratamento de penfigóide membranoso mucoso com dapsona e injeções semanais de esteróide em um paciente jovem: relato de caso

Purpose: Mucous Membrane Pemphigoid (MMP) is an autoimmune subepithelial blistering disorder usually affecting older individuals. This paper describes the pharmacological management of MMP in a young patient. Case description: A 23 year-old woman showed erythematous ulcerative gingiva in the maxillary palatal region and around the retromolar region. A perilesional excisional biopsy was done, and the histologic and immunohistochemical analysis confirmed the clinical diagnosis of MMP as the cause of desquamative gingivitis in this patient. The patient was treated with topical steroids plus daily Dapsone 100 mg and weekly steroid injections (40 mg). The lesions began to resolve after 12 weeks of therapy. Conclusion: This case of MMP in a young female patient was effectively managed with a combination therapy of topical steroids, Dapsone and steroid injections.

Objetivo: O penfigóide membranoso mucoso (PMM) é uma desordem autoimume subeptelial bolhosa que geralmente afeta indivíduos mais velhos. Este relato de caso descreve a abordagem farmacológica do PMM em uma paciente jovem. Descrição do caso: Uma paciente do sexo feminino, com 23 anos de idade, apresentava a gengiva eritematosa e ulcerada na região palatina e na região retromolar. A biópsia excisional total foi realizada e as análises histológica e de imuno-histoquímica confirmaram o diagnóstico clínico de PMM como causa da gengivite descamativa nesta paciente. A paciente foi tratada com esteróides tópicos, Dapsona 100 mg diariamente e injeções de esteróides mensais (40 mg). A resolução das lesões se iniciou após 12 semanas de terapia medicamentosa. Conclusão: Este caso de PMM em uma paciente jovem foi efetivamente tratado com uma terapia combinada de esteróides tópicos, Dapsona e injeções de esteróides.

A importância do diagnóstico precoce do penfigoide cicatricial: relato de caso / The importance of early diagnosis in cicatricial pemphigoid: case report

O penfigóide cicatricial é uma doença bolhosa inflamatória de caráter crônico e autoimune que afeta primariamente as superfícies mucosas. Auto anticorpos são produzidos contra o complexo de adesões dos hemidesmossomas da membrana basal da pele e mucosas. Afeta a cavidade bucal, laringe esôfago, membrana ocular e raramente a pele. A formação de cicatrizes é característica e pode resultar em cegueira, quando envolve a conjuntiva ocular. O envolvimento da mucosa bucal é observado na maioria dos pacientes e, quando acomete a gengiva, produz um quadro de gengivite descamativa. A etiologia é desconhecida, no entanto tem sido relatada a indução por drogas como possível desencadeador da doença. O diagnóstico é obtido com base na história clínica, biópsia e imunofluorescência direta. O tratamento é feito através de corticosteroide tópico ou sistêmico, na dependência da gravidade da doença. O presente trabalho tem por objetivo relatar um caso de penfigoide cicatricial onde a paciente apresentava apenas lesões bucais, tendo sido estabelecido o diagnóstico por meio da histopatologia através da microscopia óptica a qual apresentou aspecto bastante característico. O Tratamento intituído foi o uso de corticosteroide sistêmico. A paciente está em tratamento, com evolução satisfatória e compatível com o diagnóstico.

The cicatricial pemphigoid is a bullous disease of character chronic inflammatory and autoimmune diseases primarily affecting the mucosal surfaces. Auto-antibodies are produced against the hemidesmosomal complex of members of the basal membrane of the skin and mucous membranes. Affects the oral cavity, larynx, esophagus, eye membrane and rarely the skin. The formation of scar tissue is characteristic and can result in blindness if it involves the ocular conjunctiva. The involvement of the oral mucosa is observed in most patients and when affects the gingiva produces a table of gingivitis peel. The etiology is unknown, however, has been reported to be induced by drugs as a possible trigger of the disease. The diagnosis is made based on clinical history, biopsy and direct immunofluorescence. Treatment is realized by topical or systemic corticosteroids, depending on the severity of the disease, This study aims to report a case of cicatricial pemphigoid in which the patient had oral lesions only was the diagnosis established by histopathology by light microscopy which showed very characteristic appearance. The treatment was the use of systemic corticosteroids. The patient is in treatment, with satisfactory evolution and is compatible with the disgnosis.

Pengifóide benigno de mucosa / Benign pengifóide of mucosa

De etiologia auto-imune, o penfigóide benigno das mucosas é uma doença vesículo-bolhosa rara, que acomete a mucosa de revestimento da cavidade bucal. Este trabalho objetivou relatar um caso clínico onde foram observadas importantes manifestações clínicas, discutindo-se os fatores prognósticos de acordo com a terapêutica disponível.

Mucous membrane pemphigoid is a rare auto immune bullous disease, characterized by involvement of oral mucous membrane. This paper aimed to report a case with important clinical manifestations.

Penfigoide cicatricial, causa poco común de estenosis supraglótica / Cicatricial pemphigoid: uncommon cause of supraglottic stenosis

El penfigoide cicatricial o de mucosas es una patología que afecta principalmente a la mucosa oral y ocular. Sólo en 25% de los pacientes afecta también la piel y en alrededor de 10% de los pacientes compromete a la mucosa faringo-laríngea, produciendo en los casos severos disnea que incluso puede requerir traqueostomía. En el tratamiento del penfigoide cicatricial habitualmente se utilizan corticoides e inmunosupresores. En este artículo presentamos un caso de una mujer de 69 años con diagnóstico de penfigoide cicatricial que se manifiesta con disfagia y se comprueba con nasofibroscopía una estenosis supraglótica. Se discute el diagnóstico y manejo de esta patología poco habitual.

Cicatricial or mucous membranes pemphigoid is a rare pathology that affects mainly the ocular and oral mucosae. In only 25% of patients the skin is affected, and in 10%, the pharyngolaryngeal mucosa, resulting in serious cases in dyspnea that could even require a tracheostomy. Treatment is usually based on corticoids and immunosupressors. In this article we present the case of a 69 year old woman diagnosed with cicatricial pemphigoid, that presented dysphagia. A nasoendoscopy revealed supraglottic stenosis. Diagnosis and treatment of this uncommon disease are discussed.