Autosomal dominant and recessive variants in the desmoplakin gene (DSP) have been associated with arrhythmogenic right ventricular cardiomyopathy ARVC (OMIM: 607450) and biventricular DCM associated with keratoderma and woolly hair (OMIM: 605676). The gene discussed is DSP; the disease is Arrhythmogenic right ventricular dysplasia.