These include Naxos syndrome, which is caused by heterozygous mutation in desmoplakin gene; Carvajal-Huerta syndrome, which is due to homozygous mutation in desmoplakin protein; the woolly hair/hypotrichosis; ectodermal dysplasia–skin fragility, which is caused by mutation in PKP1; and tricho-hepato-enteric syndrome, which is caused by a mutation in SKIC2. The gene discussed is PKP1; the disease is ectodermal dysplasia syndrome.