[Erythrokeratoderma periorificialis with involvement of the extremities]

A case of acquired atypical erythrokeratoderma in a 16-year-old boy is reported. Most of the lesions were localized at the periorificial areas of the body, mainly at the angles of the mouth, the lower lip, as well as in perinasal, genital, and perianal regions. They were associated with acral involvement and pachyonychia. There were no similar skin lesions in the patient's family. The author regards the present case as an erythrokeratoderma periorificialis on the basis of an own observation and the data from the literature.