Autoimmune skin diseases are a kind of diseases caused by the immune response to self-antigens, resulting in damages to skin with or without to other tissues and organs. The traditional immunosuppressants exert their curative effect, but accompanied by a wide range of adverse reactions. In-depth understanding of pathogenesis facilitated the development of various cytokine-based therapies or drugs targeting the signaling pathways, which has significantly improved the patient's condition. However, these therapies often require long-term maintenance treatment, and the targeting pathways of many autoimmune skin diseases are still not quite clear. Therefore, emerging therapeutic strategies and innovative drugs are still in need to balance the homeostasis of the immune system and induce the long-term remission of the disease, providing new hope for the treatment of autoimmune skin diseases. This review discusses the development of drugs treating autoimmune skin diseases according to different mechanisms of action and targets.