Almost at the same time, Carvajal-Huerta and colleagues reported a homozygous truncating mutation in the DSP gene (encoding for desmoplakin) as causative of Carvajal Syndrome; a similar disease, characterized by biventricular cardiomyopathy, wooly hair, and PPK (24). Here, DSP is linked to arrhythmogenic cardiomyopathy with wooly hair and keratoderma.