Like BPAG1, desmoplakin has also been shown and described to be involved in several pemphigoid autoimmune skin disorders (paraneoplastic pemphigus, pemphigus foliaceus, erythema multiforme, and mucosal-dominant pemphigus vulgaris) including bullous pemphigoid, whose pathology is associated with antibodies binding to desmoplakin, and which presents with blistering [37,38,39]. Here, DSP is linked to paraneoplastic pemphigus.