Erythrokeratoderma variabilis successfully treated with topical tazarotene.

Erythrokeratoderma variabilis, also known as Mendes da Costa syndrome, is a genodermatosis belonging to the group of diseases known as the erythrokeratodermias. Erythrokeratoderma variabilis is characterized by two distinctive manifestations: well-demarcated, variable, transient, figurate patches of erythema, and localized or generalized hyperkeratotic plaques. Treatments include topical retinoic acid, salicylic acid, and alpha-hydroxy acid in petrolatum, but all have been reported to have limited, variable success rates. We report a child with erythrokeratoderma variabilis with no family history of this entity, successfully treated with topical tazarotene.