Desmoplakin haploinsufficiency was suggested to be associated with keratosis palmoplantaris striata II (PPKS2; OMIM #612908), and recessive mutations in the DSP gene have been associated with skin fragility/woolly hair syndrome (OMIM #607655) and dilated cardiomyopathy (OMIM #605676) [2, 23]. This evidence concerns the gene DSP and isolated familial wooly hair disorder.