ARVC clusters in families and its pattern of inheritance is generally autosomal dominant with age-related, reduced penetrance.2 The discovery that Naxos disease, a rare cardiocutaneous autosomal recessive form of ARVC, was caused by pathogenic variants in JUP-encoded plakoglobin3 prompted rapid identification of pathogenic variants in other desmosomal genes (PKP2, DSP, DSG2, and DSC2) in ARVC populations. This evidence concerns the gene DSG2 and Arrhythmogenic right ventricular dysplasia.