Abstract
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.
Highlights
Erythrokeratoderma variabilis (EKV) is considered as the subtype of a group of skin diseases called Erythrokeratoderma [1]
The most important feature that differentiates PSEK from EKV is the lack of migration characteristics of the lesions
Palmoplantar keratoderma is more specific to PSEK [4]
Summary
Erythrokeratoderma variabilis (EKV) is considered as the subtype of a group of skin diseases called Erythrokeratoderma [1]. EKV is an autosomal dominant inherited genodermatosis and is characterized by fixed, brownish hyperkeratotic plaques and figurative erythematous patches [2]. Progressive symmetric erythrokeratoderma and ECV are accepted as two main subtypes of erythrokeratodermas. Since these ichthyosiform dermatoses have similar characteristics, it has been suggested to call progressive symmetric erythrokeratoderma as "EKV progressive" [3]. Two cases with rare ECV are presented here to emphasize that the disease may follow different clinical presentations
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