Clouston syndrome has a dominant autosomal pattern, therefore most patients havean affected family member, although new mutations have been reported aswell.6 Changes ingene GJB6, located in chromosome 13 (locus 13q11-q12) areresponsible for the syndrome, since the gene is involved in the differentiationand growth of keratinocytes.9This gene codifies the cell-junction protein, connexin 30, a transmembraneprotein which facilitates intercellular communication and is present in thestratum corneum, sweat glands and hair follicles. Here, GJB6 is linked to Clouston syndrome.