Autoimmune bullous dermatoses (AIBD) are a heterogeneous group of chronic mucocutaneous diseases with a blister as the primary lesions. Blisters are a consequence of pathological autoimmune reaction caused by auto-antibodies which react with structural glycoprotein components of the epidermis and the epidermal-dermal barrier. AIBD are severe diseases which have been, prior to the discovery of corticosteroid therapy, considered as lethal diseases. Nowadays, the mortality rate of these diseases is significantly reduced; however, the letal outcome may occur due to the side effects of immunosuppressive therapy or resistance to conventional therapeutic approach. Two most common pathological entities in this group are pemphigus vulgaris and bullous pemphigoid, most commonly affecting the middle-aged and older population. Therapeutic approach depends not only on the type of AIBD, but also on the severity of the disease, comorbidities and tolerability of the therapy or its adverse effects. Corticosteroids are most widely used treatment, which have been recognized as a gold standard since 1950s. However, progress in understanding complexity of immunopathogenesis has enabled the use of new therapeutic options, such as monoclonal antibodies, in the treatment of patients with the severe disease types or of those who have not responded satisfactorily to conventional therapy.