DSP and Palmoplantar keratoderma: A recessive mutation of desmoplakin has been proven to explain another cardiocutaneous syndrome, i.e. Carvajal disease [70], characterized by keratoderma, woolly hair and a biventricular form of ARVC/D [71], with distinct ultrastructural abnormalities of intercalated discs and decreased immunoreactive signals for desmoplakin, plakoglobin and Cx 43.