[Malignant atrophic papulosis (Degos' syndrome)]

In a 49-year-old female patient with an eruption of lentil-shaped papules on the trunk and extremities, persisting for six years, originally in another department scleroderma guttata was diagnosed. For one year the patient suffered also from dyspeptic complaints, loss of weight and relapsing polyserositis. The complaints receded temporarily after immunosuppressive therapy. On surgical revision of the abdominal cavity on account of serous peritonitis during the last three months eruption of whitish foci on the intestinal serosa and omentum was found. Bioptic excision of the skin revealed an old bland infarct. From the clinical course and dermatological picture papulosis maligna atrophicans (Degos' syndrome) was diagnosed. Autoptic examination confirmed the clinical diagnosis of the syndrome by the finding of thromangiitis of Bürger's type with bland infarcts of the small intestine and perforation of the jejunum.