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The disease is characterized by necroptotic keratinocytes and a cytotoxic immune cell infiltrate at the dermo\u2010epidermal junction (DEJ), orchestrated by interferon (IFN)\u2010regulated proinflammatory cytokines. Molecular analyses revealed a strong upregulation of innate and adaptive immune pathways in lesional skin including DNA\u2010recognition pathways, chemokine signalling, antigen presentation and B\u2010 and T\u2010cell activation, which are believed to interact in a complex self\u2010amplifying network. Concerning adaptive immune signalling, particularly B cells are currently being studied as there is growing evidence for additional abilities besides autoantibody expression in skin autoimmunity. These detailed insights have paved the way for the development of drugs targeting crucial molecules of pathogenic immune cells and pathways. 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