Mutations in DSP have been linked to several Mendelian syndromes involving palmoplantar keratoderma [26], left ventricular cardiomyopathy [26], familial arrhythmogenic right ventricular dysplasia [27], and lethal ancantholytic epidermolysis bullosa [28]. This evidence concerns the gene DSP and epidermolytic palmoplantar keratoderma, 1.