We report two patients, mother and daughter, with Erythrokeratoderma variabilis (EV). This rare genodermatosis is characterized by the presence of two components: migratory erythema and fixed hyperkeratosis. Our patients experienced symptomatic relief of pruritus associated with erythema with the use of an oral, low-sedating H1-antihistamine. Revision of the literature in order to allocate the frequency of pruritus in EV and discussion of this association will follow.