The first description of a myocardial disease linked to DSP mutations referred to patients with Carvajal syndrome, an autosomal recessive cardio-cutaneous syndrome, including dilated cardiomyopathy (DCM), palmoplantar keratoderma and wooly hair [3]. The gene discussed is DSP; the disease is arrhythmogenic cardiomyopathy with wooly hair and keratoderma.