Previous studies have shown that inherited mutations in DSP are a characteristic feature of a number diseases of the skin and heart including, skin fragility or wooly hair syndrome [47], arrhythmogenic right ventricular dysplasia [17], [48], lethal acantholytic epidermolysis bullosa [49], and dilated cardiomyopathy with wooly hair and keratoderma [50]. Here, DSP is linked to Arrhythmogenic right ventricular dysplasia.