Activation of TRPV3 in keratinocytes has been shown to lead to release of proinflammatory messengers, and several “gain-of-function” mutations in trpv3 have been found to cause the human congenital skin disorder Olmsted syndrome, characterized by bilateral mutilating palmoplantar keratoderma, periorificial keratotic plaques, and hair loss with follicular papules15–22. The gene discussed is TRPV3; the disease is Olmsted syndrome 1.