Erythematokeratodermia variabilis et progressive (EKVP) is the first reported skin disease linked to mutations in connexins (Richard et al., 1998; Richard, 2005) and mutations in GJB3, GJB4, and GJA1, encoding Cx31, Cx30.3 and Cx43, respectively cause EKVP (Duchatelet and Hovnanian, 2015; Ishida-Yamamoto, 2016; Laird and Lampe, 2022). Here, GJB3 is linked to erythrokeratodermia variabilis.