RESUMO
Abstract: Pemphigus vulgaris is a chronic autoimmune bullous dermatosis that results from the production of autoantibodies against desmogleins 1 and 3. It is the most frequent and most severe form of pemphigus, occurring universally, usually between 40 and 60 years of age. It usually begins with blisters and erosions on the oral mucosa, followed by lesions on other mucous membranes and flaccid blisters on the skin, which can be disseminated. There is a clinical variant, pemphigus vegetans, which is characterized by the presence of vegetating lesions in the large folds of the skin. Clinical suspicion can be confirmed by cytological examination, histopathological examination, and direct and indirect immunofluorescence tests. The treatment is performed with systemic corticosteroids, and immunosuppressive drugs may be associated, among them azathioprine and mycophenolate mofetil. More severe cases may benefit from corticosteroids in the form of intravenous pulse therapy, and recent studies have shown a beneficial effect of rituximab, an anti-CD20 immunobiological drug. It is a chronic disease with mortality around 10%, and septicemia is the main cause of death. Patients need long-term and multidisciplinary follow-up.
Assuntos
Humanos , Masculino , Feminino , Adulto , Pessoa de Meia-Idade , Pênfigo/diagnóstico , Pele/patologia , Autoanticorpos/imunologia , Inquéritos e Questionários , Pênfigo/classificação , Pênfigo/terapia , Pênfigo/epidemiologia , Imunoglobulinas Intravenosas/uso terapêutico , Desmossomos/imunologia , Diagnóstico Diferencial , Imunossupressores/classificação , Imunossupressores/uso terapêutico , Imunoterapia/métodosRESUMO
Pemphigus vulgaris [PV] is an autoimmune blistering disease of the stratified squamous epithelia. It is caused by an autoantibody against Desmoglein 3 [Dsg 3], a normal component of desmosomes which has a critical role in the attachment of keratinocytes. Several studies have demonstrated that healthy relatives of patients with autoimmune diseases including PV are more susceptible to produce autoantibodies or develop autoimmune diseases. This susceptibility to autoimmunity or autoimmune diathesis has been shown to be an autosomal dominant trait. In this study the anti-Dsg 3 autoantibody was found in the sera of 35% of 46 healthy relatives of Iranian patients with PV with immunoblot assay. This shows that the genetic susceptibility to autoimmunity is present in the family members of patients with PV and it is mostly transmitted as an autosomal dominant trait