In dermatology, DSP mutations are mainly associated with two syndromes: Carvajal syndrome, which involves mutations in the C-terminal tail of DSP and affects both the skin and heart, and SFWHS, first described in 2011, caused by mutations in the N-terminal head of DSP. Here, DSP is linked to arrhythmogenic cardiomyopathy with wooly hair and keratoderma.