DSP and epidermolytic palmoplantar keratoderma, 1: Skin disease is particularly remarkable for its intragenic heterogeneity, for example distinct dominant and recessive mutations in the desmosomal Desmoplakin gene DSP can result in a spectrum of disease phenotypes ranging from arrhythmogenic right ventricular cardiomyopathy (ARVC) and striate palmoplantar keratoderma to palmoplantar keratoderma with woolly hair and ARVC (reviewed in [134]).