Maligne atrophische Papulose

: A 53-year-old woman had noticed numerous papules on her trunk and proximal parts of the limbs. They had healed with a central scar surrounded by a reddish wall. Four months later she had to undergo laparotomy for ileus, resulting from a perforation in the region of the middle jejunum: it was excised and sutured over. At laparotomy white plaque-like lesions were noted on the serosa of both small and large intestine. Histological examination of the jejunal exudate and of a skin biopsy both demonstrated malignant atrophic papulosis (Kohlmeier-Degos' disease). Treatment with ticlopidine, a platelet-aggregation inhibitor (250 mg twice daily), was initiated and she has now been free of extracutaneous symptoms for 6 years.