Blistering dermatosis are generally known to be constructed of two groups, autoimmune blistering dermatosis and genodermatosis. Autoantibodies are determined in autoimmune blistering dermatosis including pemhigus and bullous pemphigoid. We used to treat them with systemic corticosteroids and various immunosuppressants, with attention to side effects. Apheresis or intravenous immunoglobulin (IVIG) therapy is recommended as a combination therapy with systemic corticosteroids and various immunosuppressants. Some of responsible genes have been elucidated in blistering genodermatosis such as epidermolysis bullosa. Although no specific treatment is available, cell-based therapies using bone marrow cells and fibroblasts improve the quality of life in these patients. In this review, we present an overview of the treatment of blistering dermatosis.