Erythrokeratoderma variabilis in two cases with localized and generalized lesions

Erythrokeratoderma variabilis is an autosomal dominant genodermatosis characterized by fixed, brownish hyperkeratotic plaques and migratuar erythematous patches. Here we report a 44-year-old female patient with a common itching and rash over a 4-month period and a 21-year-old male with stable, asymptomatic plaques in both antecubital regions for 3 years due to the rare diagnosis of EKV. The aim of this study is to draw attention to the fact that the disease may be encountered in different clinical manifestations and may be severe itchy or asymptomatic. The absence of extracutaneous involvement and recurrence after discontinuation of treatment indicate a benign and chronic course.