Degos disease DD is a rare obstructive vasculopathy characterized by distinctive skin lesions. Involvement of the soles, palms and genitalia is rare. In most cases, disease has an unfavorable course and involves gastrointestinal tract, central nervous system and occasionally other organs. Pleural and pericardial involvements are usually minor manifestations with prolonged course. Death occurs in approximately 50% of the patients usually due to intestinal perforation or central nervous system bleeding. We describe a 48-year-old man of lethal systemic DD. Widespread skin lesions with involvement of palmoplantar surfaces, genitalia and scalp were ignored for 3 years, whereas the disease revealed own malignant nature. The disorder progressed to nervous, gastrointestinal and cardiopulmonary system that led to death after 5 months from onset of systemic involvement as severe restrictive cardio-pulmonary insufficiency. Autopsy showed diffuse fibrotic changes in serosal membranes and internal organs.