Erythrokeratodermia variabilis (EKV) is an autosomal dominant disease characterized by erythematous lesions and hyperkeratosis caused by mutations in two epidermally expressed connexin genes, GJB3 (Cx31) and GJB4 (Cx30.3) [10],[11]. Here, GJB4 is linked to erythrokeratodermia variabilis.