Erythrokeratodermia variabilis with adult onset: report of a sporadic case unresponsive to systemic retinoids

Abstract

Erythrokeratodermia variabilis is a rare autosomal-dominant genodermatosis characterized by migratory and erythematous patches changing over the course of hours to days, and fixed keratotic plaques. The disease begins mostly at birth or within the first year of life; it very rarely starts after childhood. We present herein a sporadic case with adult onset that was unusually resistant to both isotretinoin and acitretin.