Erythrokeratodermia figurata variabilis (EKV) is a rare disorder of cornification inherited as an autosomal dominant trait. Genetic linkage to the Rh locus on chromosome 1 has been recently documented. In 1957, Sommacal and Schnyder reported on a family with 14 affected members. We have reexamined this pedigree, which counts 77 members with 29 affected persons over five generations (45 females, 31 males). Twenty females and 9 males were affected. In all patients EKV presented in the first year of life, and several mothers noted the erythematous lesions at birth of their children. The hyperkeratotic lesions appeared later. The reddish macules changed within hours to days. The erythematous areas were sharply outlined and sometimes surrounded by an anemic border. Only few members stated that their erythema could persist for more than a week. Clear triggers were emotional stress and changes of temperature. In all but two of the patients erythema was prominent and in the others hyperkeratotic lesions were more severe. Most patients had a burning sensation in their red areas. There was a marked tendency for improvement of EKV after puberty. Five females reported regular superficial skin peeling on hands and feet. The features in these patients had some similarities with erythrokeratolysis hiemalis.