ABSTRACT
El pénfigo vulgar es el tipo más común de un grupo padecimientos crónicos autoinmunes identificados por la presencia de lesiones ampulosas situadas en las mucosas y piel. El pénfigo vulgar oral (PVO) se caracteriza por la presencia de ampollas localizadas en las encías, paladar blando, carrillos, pero cualquier sitio de la cavidad oral puede ser afectado. Estas lesiones se presentan primero en la cavidad oral y meses después en la piel, por lo que su diagnóstico temprano y oportuno es vital para el pronóstico. El propósito de este trabajo es presentar esta condición clínica en una persona adulta mayor.
Pemphigus vulgaris is the most common of a group of chronic autoim-mune conditions characterized by the presence of mucosal and dermal blisters. In the case of oral pemphigus vulgaris (OPV), these are typi-cally found on the gums, soft palate, and cheeks, though anywhere in the oral cavity can be affected. These lesions appear first in the oral cavity and then, months later, on the skin. Therefore, early diagnosis is crucial for prognosis. The aim of this paper is to present a case report of this condition in an older adul.
Subject(s)
Humans , Female , Aged , Mouth Diseases/classification , Pemphigus/diagnosis , Pemphigus/drug therapy , Prognosis , Prednisolone/therapeutic use , Treatment OutcomeABSTRACT
Pemphigus vulgaris is an autoimmune bullous disease whose therapy is based on systemic corticosteroids, with or without immunosuppressants. Rituximab is a chimeric monoclonal antibody of the IgG class, directed at a specific CD20 B cell surface antigen, used in pemphigus vulgaris empirically since 2002, with success in 90% of the cases and long periods of remission. Male patient, 33 years old, diagnosed with pemphigus vulgaris, confirmed by histopathology and direct immunofluorescence. He was treated for seven months with numerous treatments, including immunosuppressive drugs, with an unsatisfactory response, until he had complete remission with the use of rituximab. During a 34-month follow-up period, the patient presented a slight clinical relapse, which was successfully controlled with prednisone in a daily dose of 120mg, soon reduced to 20mg.
Pênfigo Vulgar é uma doença bolhosa auto-imune, cuja terapêutica é baseada em corticoesteróides sistêmicos, associados ou não a imunossupressores. Rituximabe é um anticorpo monoclonal quimérico da classe IgG direcionado a um antígeno CD20 de superfície celular específico da célula B, usado em pênfigo vulgar desde 2002, com sucesso em 90% e longos períodos de remissão. Paciente masculino, 33 anos, diagnóstico de pênfigo vulgar, confirmado por histopatologia e imunofluorescência direta. Durante 7 meses, recebeu inúmeros tratamentos com imunossupressores, apresentando resposta insatisfatória e progressão da doença, até que logo após a introdução de rituximabe teve completa remissão. Durante um acompanhamento de 34 meses, apresentou leve recidiva clínica, controlada com prednisona 120mg/dia, rapidamente reduzida e em uso atual de Prednisona 20mg/dia.
Subject(s)
Adult , Humans , Male , Antibodies, Monoclonal, Murine-Derived/administration & dosage , Drug Resistance, Multiple , Immunologic Factors/administration & dosage , Pemphigus/drug therapy , Glucocorticoids/administration & dosage , Prednisone/administration & dosage , Time Factors , Treatment OutcomeABSTRACT
La aproximación a las lesiones de tipo pénfigo que se presentan en cavidad oral parte de su apariencia clínica, manifestación sistémica, criterio diagnóstico y fisiopatología. Éste se describe, como una ampolla producida por la separación entre las uniones desmosomales debido a la presencia de autoanticuerpos en contra de las proteínas de adhesión, las desmogleinas. Su diagnóstico diferencial es el penfigoide y la etiología es de carácter hereditario o por causa externas como: radiación ultravioleta, dieta, pesticidas, medicamentos, entre otros. Su presentación característica es una ampolla con líquido seroso la cual se manifiesta en sitios como mucosas y piel. Se clasifica en 3 variedades principales, el vulgar, el foliáceo y el paraneoplásico. Su diagnóstico se da por apariencia clínica y con análisis de laboratorio (biopsia, entre otros). Para su tratamiento el uso de corticosteroides tópicos y sistémicos es el usual, iniciando con altas dosis y posteriormente con bajas en etapas de mantenimiento. Muchos de ellos ceden al tratamiento pero otros pueden generar complicaciones graves lo que depende de la respuesta inmune del paciente.
The approximation to Pemphigus-like lesions that occur in the oral cavity are based on clinical appearance, systemic manifestations, diagnostic criteria, and pathophysiology. This is described as a blister caused by the separation between the desmosomal junctions due to the presence of autoantibodies against adhesion proteins, the desmogleins. Differential diagnosis is pemphigoid and etiology of hereditary or external causes such as ultraviolet radiation, diet, pesticides, drugs, among others. The characteristic presentation feature is a blister with serous fluid which manifests as mucosal sites and skin. It is classified into three main varieties, the vulgar, the foliaceus and paraneoplastic. Its diagnosis is given by clinical appearance and laboratory tests (biopsy, etc.). Treatment includes the use of topical and systemic corticosteroids, starting with high doses and subsequently with low maintenance stages. Many improve with treatment, but some may lead to serious complications, which depending on the patient's immune response.
ABSTRACT
En la actualidad, para diagnosticar pénfigo, es necesaria una clínica con ampollas y erosiones, histopatología con acantólisis y detección de anticuerpos en la piel afectada (inmunofluorescencia directa) o en sangre circulante (inmunofluorescencia indirecta). Objetivos: Los objetivos del trabajo son comparar la sensibilidad y especificidad de estas dos últimas técnicas y demostrar si existe relación de los niveles de ELISA frente a desmogleínas con elgrado de afectación cutáneo-mucoso. Material y métodos: Se obtuvieron 117 determinaciones en 26 pacientes con pénfigo y 29 determinaciones en pacientes con otras enfermedades ampollosas como grupo control. Medimos anticuerpos antisustancia intercelular por inmunofluorescencia indirecta y anticuerpos antidesmogleína 1 y 3 por ELISA. También se midieron las cifras de anticuerpos antes y después de terapias como las inmunoglobulinas intravenosas y plasmaféresis. Resultados: La determinación de anticuerpos por ELISA frentea desmogleínas 1 y 3 es más sensible que la inmunofluorescencia indirecta. No encontramos diferencias en cuanto a especificidad. Los niveles de anticuerpos son paralelos a la actividad clínica. Estos niveles no descienden inmediatamente tras la terapia con inmunoglobulinas intravenosas.
Nowadays diagnostic criteria of pemphigus include:clinical presentation with blisters and erosions, acantholisison the conventional histopathological examinationand detection of antibodies on affected skin (directimmunofluorescence) or serum (indirect immunofluorescence). Objective: The aims of this study are to compare sensibility and specificity between the ELISA method and the indirect immunofluorescence test (IIF)and to investigate a possible correlation between desmoglein titers (detected by ELISA) and clinical severity. Materials and methods: 26 patients with pemphigus were included in the study. The control group included 29patients with other bullous diseases. In every patient, antiintercellular substance antibodies were detected by the indirect immunofluorescence test while anti-desmoglein1 and 3 antibodies were titered by ELISA. In addition, titers of antibodies were measured before and aftertherapy with intravenous immunoglobulins and plasmapheresis. 117 determinations were obtained frompatients with pemphigus and 29 from the control group.Results: ELISA detection of antibodies against desmoglein1 and desmoglein 3 is a more sensitive method than the indirect immunofluorescence test. No difference inspecificity has been found. There is a positive correlation between titers of antibodies and clinical activity. Intravenous immunoglobulin therapy does not induceimmediate tapering of antibody titers.
Subject(s)
Enzyme-Linked Immunosorbent Assay , Pemphigus , Fluorescent Antibody Technique, Indirect , PlasmapheresisABSTRACT
Os pênfigos são dermatoses bolhosas auto-imunes, em que há a produção de auto-anticorpos direcionados contra moléculas de adesão dos epitélios, levando à perda da coesão celular. A produção de auto-anticorpos ocorre quando os pacientes desenvolvem um desequilíbrio da resposta imune (quebra da tolerância imunológica), passando a reconhecer antígenos próprios. A resposta é geralmente direcionada contra um único epítopo alvo; entretanto, como conseqüência da resposta inflamatória do processo primário e do extenso dano tecidual ocasionado, pode haver exposição de componentes protéicos ocultos, levando à produção de diferentes auto-anticorpos. Assim, é possível que surja uma nova doença cutânea auto-imune, em decorrência do fenômeno intra ou intermolecular de epitope spreading. São revistos os principais conceitos desse fenômeno e sua ocorrência nas dermatoses bolhosas auto-imunes, com ênfase nos pênfigos, grupo de dermatoses bolhosas autoimunes mais prevalente no Brasil.
Pemphigus comprises autoimmune blistering skin diseases in which autoantibodies directed against antigens (epithelial adhesion molecules) are found, leading to loss of cell cohesion. The production of autoantibodies occurs due to an immune imbalance (break of immune tolerance) driving to recognition of self- antigens. The response is usually directed against an exclusive target epitope; however, due to the inflammatory response and to the extensive tissue damage, it is possible that the exposure of hidden protein components leads to distinct autoantibody production. Hence, a new autoimmune disease may occur in consequence of an intra- or intermolecular epitope spreading phenomenon. The authors review the main concepts of this phenomenon, and its occurrence in autoimmune blistering diseases, with emphasis on pemphigus, the most prevalent disease of this group in our country.