Mutations in connexin have been identified with various inherited diseases [4], including Cx32 mutation in X-linked Charcot Marie tooth disease, Cx26 and Cx30 mutations in deafness and skin diseases, Cx46 and Cx50 mutations in hereditary cataracts and Cx31 mutation in erythrokeratodermia variabilis (EKV) and hearing impairment with/without peripheral neuropathy. Here, GJB2 is linked to erythrokeratodermia variabilis.