Pathogenic variants in its major constituent desmoplakin (DP; gene DSP) may give isolated cardiomyopathy, skin fragility, palmoplantar keratoderma (PPK) or woolly hair (WH), but also a combination of these, like in Carvajal syndrome.2, 3. This evidence concerns the gene DSP and arrhythmogenic cardiomyopathy with wooly hair and keratoderma.