Mutations in DSP (MIM#125647) may manifest as Carvajal syndrome, lethal acantholytic epidermolysis bullosa, skin fragility–wooly hair syndrome, striate palmoplantar keratoderma, and other phenotypes involving hair, nails, and skin, while early cutaneous findings may herald future cardiac involvement [11]. This evidence concerns the gene DSP and arrhythmogenic cardiomyopathy with wooly hair and keratoderma.