Most authors currently recognize atrophic vascular poikiloderma as one of the variants of skin lymphoma - the poikiloderma form of mycosis fungoides. In some publications, this disease is classified as one of the variants of plaque parapsoriasis. The course of this disease is long, benign, with slow progression. The article presents a description of atrophic vascular poikiloderma in a young patient. At the time of treatment, the skin lesion had existed for about 4 years, the skin lesion was slowly progressing. Initially, the diagnoses were lichen sclerosus, lichen planus. A skin biopsy was performed with a histological examination, as a result of which a diagnosis of scleroatrophic lichen was established. In the future, the skin process progressed, rashes appeared that were not characteristic of scleroatrophic lichen. There was an assumption that the patient had vascular atrophic poikiloderma. The diagnosis was confirmed after performing histological and immunohistochemical studies of the skin biopsy. A feature of the case was the combination of two rare pathologies: rheumatic heart disease and vascular reticular poikiloderma. Establishing the diagnosis became possible only due to dynamic observation, repeated skin biopsy with histological and immunohistochemical studies