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  • Pemphigus and the oral cavity : a narrative review
    Publication . Curado, Paulo Ricardo Nunes da Risca; Silva, Francisco Salvado e; Almeida, Luís Miguel Soares de
    Pemphigus is a group of autoimmune bullous diseases in which autoantibodies of mainly IgG isoform target structural proteins of desmosomes, desmoglein 1 and desmoglein 3. These desmosomes are adhesion structures that, alongside desmogleins, are essential for cell-cell adhesion between keratinocytes and have an important role in structural intercellular integrity in stratified squamous epithelia, such as skin and oral mucosa. Pemphigus comprises three main clinical presentations, pemphigus vulgaris, which often presents with painful oral erosions/blisters, pemphigus foliaceus, that presents exclusively cutaneous lesions, and paraneoplastic pemphigus, that presents with both humoral and cellular autoimmune responses and is frequently associated with hematologic malignancies. Rare forms of pemphigus include pemphigus herpetiformis, IgA pemphigus and drug-induced pemphigus. Differential diagnosis of pemphigus diseases is based on a combination of interpretation of clinical findings, such as Nikolsky´s sign, direct immunofluorescence microscopy (DIF), and histopathological presentation, and serological tests. Treatment modalities include mainly corticosteroids, alone or associated with immunosuppressive drugs, azathioprine, and mycophenolate mofetil, that act as steroid-sparing agents, and B-cell depleting therapies including anti-CD20 antibodies such as Rituximab. Novel therapies for pemphigus treatment include neonatal Fc receptor antagonists and bruton tyrosine kinase inhibitors. Since pemphigus presents frequently with oral lesions, and in many cases is the first manifestation of the disease, this review pretends to serve as a practical guide for dentists and oral medicine specialist to understand pemphigus diseases, and to properly diagnose and treat such diseases, correlating them with the oral cavity.
    2022-11-26Dissertação de mestrado Acesso aberto Ver mais