Malignant atrophic papulosis (Degos’ disease) is a rare disease of unknown etiology involving small and middle-sized arteries of skin and other organs. The first symptoms are skin lesions within months or years followed by signs of organ damage, especially those of digestive tract and nervous system. The benign form involves only the skin, in malignant form also other organs are compromised. The case of 52-year-old woman with three years lasting skin symptoms without systemic complications is presented. Thorough examination revealed neither other organ involvement nor any other associated disease. Because for four years, since the time of diagnosis, and seven years, since the development of skin lesions, the patient presents no other organ involvement, the presented case might be considered as a benign form of the disease, further follow-up is required, however.

        Key words: malignant atrophic papulosis – benign form – skin signs – histopathology – prognosis