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. The gene discussed is DSP; the disease is Arrhythmogenic right ventricular dysplasia.