Desmosomal ARVC predominantly follows autosomal dominant inheritance with incomplete penetrance; exceptions include Naxos disease (triad of autosomal recessive ARVC, palmoplantar keratoderma, and woolly hair) [28,54], and Carvajal syndrome (a variant of Naxos disease with ALVC, associated with early morbidity), caused by recessive mutations in JUP and DSP, respectively. Here, DSP is linked to arrhythmogenic right ventricular cardiomyopathy.