The autoimmune vesiculobullous diseases of the skin and mucous membranes are a fascinating group of diseases characterized by blisters of the skin and mucous membranes. These diseases are among the most intriguing, well-characterized, and potentially serious skin diseases known. In recent years, there has been major progress made in the understanding of their pathophysiology, in the development of new diagnostic techniques and of new therapeutic approaches. These advances have placed the autoimmune blistering diseases of the skin and mucous membranes at the forefront of dermatologic advances in the late twentieth century. This article discusses several of the most important autoimmune blistering disease, including bullous pemphigoid, mucous membrane pemphigoid (formerly known as cicatricial pemphigoid), epidermolysis bullosa acquisita, linear IgA bullous dermatosis, pemphigus and paraneoplastic pemphigus, with particular emphasis on the use of new and emerging therapeutic approaches.