The erythematous patches of EKV can be treated with topical corticosteroids. Hyperkeratotic lesions usually show a good response to retinoids, however, the erythematous lesions do not respond to retinoids.However, patient's response to retinoid therapy is only limited during the therapy course. The lesions reappear during the untreated period. Medication choice should be made based on its side-effect because of its prolonged use.The postulated pathogenesis is systemic ectodermal vascular dysplasia and abnormal vascular dilatation that may lead to the disturbance of keratinization.Electron microscopy studies show grain-like cells at the junction of stratum granulosum and corneum, containing large amounts of clumped perinuclear tonofilaments. Unmyelinated nerve axons and Schwann cells can be seen in the superficial dermis of erythematous patches and uninvolved skin. The presence of these nerves may link to the variability of lesions that can change with temperature changes, wind and emotional stress.