The article deals with ethiopathogenetic factors of origin of progredient symmetric erythrokeratodermia of Gottron which is mainly characterized by autosomal dominant mode of inheritance and is classified as a disorder of cornification with extra epidermal cellular proliferation.Clinicopathologic criteria on the basis of which this pathology can be verified are described here, such as: onset of a disease at an early age, strictly divided polycyclic, fixed, hyperkeratotic plaques with a narrow erythematic crown and a clean-cut, sometimes hyper pigmented edge, which are symmetrically located on skin on head, buttocks, limbs and are not prone to quick peripheral growth; large frequency of palmoplantar keratoderma and also histological changes typical of erythrokeratodermia.The article describes a rare clinical case of autosomal-recessive progredient symmetric erythrokeratodermia of Gottron which is distinguished by seasonal changes of skin pathological process — emergence of rash in winter and complete remissions in summer.