Erythrokeratodermia Variabilis

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Abstract
Three patients with erythrokeratodermia variabilis (EKV), representing three consecutive generations of the same family, are presented and the literature is reviewed. The major features of this disorder are: (1) onset during early infancy; (2) autosomal dominant mode of inheritance; and (3) distinctive cutaneous lesions which consist of sharply demarcated, hyperkeratotic plaques with a geographic outline and which frequently are associated with sharply outlined and independent areas of erythrodermia. The comparative clinical and histopathologic features of EKV and congenital ichthyosiform erythroderma are briefly reviewed. Most authors suggest that EKV is a localized variant of congenital ichthyosiform erythroderma.

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