Pemphigus and pemphigoid are potentially fatal autoimmune diseases with blisters that affect mucocutaneous surfaces and skin. The diagnosis is made on the basis of laboratory and histopathological tests, clinical examination and medical history. Histopathologically, pemphigus is characterized by acantholysis i.e. the breaking of intercellular connections (desmosomes) located in the spinous layer of the epithelium due to the formation of antibodies directed towards these connections. The consequence of these events is the formation of an intraepithelial blister. In pemphigoid the antibodies are directed against hemidesmosomes, multiprotein complexes that are located along the basement membrane and connect the epithelium with the connective tissue. There is a finding of a subepithelial blister. After the bursting of the blisters, erosions occur, lesions that can become infected if supportive antiseptic therapy with agents that stimulate epithelialization of the mucous membrane has not been conducted. The gold standard for the detection of the disease is direct immunofluorescence, which is performed by taking a sample from the unchanged perilesional tissue. In order to confirm the diagnosis of these autoimmune diseases, the tissue must not be necrotic and must contain deposits of immunoglobulin G and/or the C3 component of complement in the appropriate localization, depending on which antigen is affected. In addition to pathohistological analysis and direct immunofluorescence of the tissue, it is possible to use indirect immunofluorescence to monitor the course of the disease and ELISA, enzyme-linked immunosorbent assay, a highly sensitive and specific immunoenzymatic test. Timely diagnosis and appropriate therapy greatly influence the prognosis and the final outcome of the disease. The primary treatment of pemphigus are systemic corticosteroids in combination with adjuvant immunosuppressants or rituximab (anti-CD20 monoclonal antibody), while in severe cases intravenous immunoglobulins and plasmapheresis can also be used. Treatment of pemphigoid usually involves the topical application of a pain reliever with an anti-inflammatory effect, and topical corticosteroids are excellent for this purpose. Auxiliary immunosuppressive drugs may also be prescribed. Newer treatment methods and a clearer understanding of the pathogenesis of the disease have improved the prognosis of pemphigus/pemphigoid and reduced mortality.