Erythrokeratodermia variabilis (EKV) is a rare heterogeneous skin disorder. The classical EKV first described by Mendes da Costa is characterized by two types of skin lesions hyperkeratotic plaques, and transient erythematous areas. Herein, we report two patients presenting with erythematous and hyperkeratotic lesions that were histopathologically diagnosed with EKV. There are two major subtypes of EKV:  EK variabilis (Mendes da Costa), and EK progressiva symmetrica (Gottron). The classical EKV initially described by Mendes da Costa is characterized by two types of skin lesions: (a) figurate hyperkeratotic plaques and (b) transient erythematous areas. These subtypes are independent, and their shapes and distribution can be changeable at any time.The lesions have propensity to locate on the distal extremities, buttocks, and trunk. Hyperkeratotic plaques are particularly distributed on the face, hip, and extensor aspect of the limb. In both cases, the hyperkeratotic lesions were located on the flexor areas. EKV usually presents at birth or during infancy. The case one had developed the lesions since age 5, and the case two had developed since age 2. Progressive symmetric erythrokeratoderma (PSEK) is less common among the erythrokeratoderma variants (that was initially described by Gottron in 1922). It presents as symmetrical plaques on the limbs, buttocks, and face during early childhood. The plaques progress in childhood and frequently stabilize in adolescent age. It is autosomal dominant disease, often with incomplete penetrance. There is considerable similarity between PSEK and EKV due to the presence of a symmetrically distributed, fixed or very slowly progressive erythematosus, scaly plaques. PSEK differs in the absence of migratory erythematosus lesions and in greater incidence of palmoplantar keratoderma. A distinctive feature of EKV from PSEK is the lack of facial involvement. Histopathological findings of PSEK are non-specific as well. The findings include loose hyperkeratotic stratum corneum, wide plugged hyperkeratotic follicular openings, acanthosis and papillamatosis, and perinuclear vacuolization and parakeratosis. The treatment of PSEK is similar to EKV. Patients with PSEK have been successfully treated with retinoids, however, the rate of recurrence is high.