Erythrokeratodermia Variabilis

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Abstract
• Erythrokeratodermia variabilis is a rare genodermatosis; American authors have reported only four previous cases. It had been a problem to obtain a large pedigree for clinical investigation. We studied a family with 12 involved members in five generations. Symmetrically distributed migratory patches and scaling plaques are characteristic and were found to involute with a combination of keratolytic agents and topical steroids. Exacerbations of these patches and plaques were noted in our female patients during such high estrogen states as pregnancy or oral contraceptive usage. (Arch Dermatol 114:259-261, 1978)