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Erythrokeratodermia (EK) is a rare skin disorder, likely genetic and usually present from infancy. 1 There is patchy symmetrical involvement over the body surface, manifested in progressive figurate plaques of hyperkeratosis and more transient areas of erythema. There is significant overlap in the clinical and genetic features of the "variabilis" and "progressiva" forms of EK. Restricted cutaneous syndromes of EK have been described associated with mutations in the connexin (GJB3, GJB4, anddoi:10.1212/nxg.0000000000000263 pmid:30065956 pmcid:PMC6066365 fatcat:56svydx7jvfuldab75wj7d6sau