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erythrokeratodermia variabilis


Definition: a dermatosis characterized by hyperkeratotic plaques of bizarre, geographic configuration, associated with erythrodermic areas that may vary remarkably in size, shape, and position from day to day; hair, nares, and teeth are not affected; onset is usually in the first year of life; autosomal dominant or recessive inheritance, caused by mutation in the connexin gene encoding gap junction protein beta-3 (GJB3) on 1p.

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© Copyright 2018 Wolters Kluwer. All Rights Reserved. Review Date: Sep 19, 2016.