• S Meier, G Zellweger, P Elsner, D Wüthrich, and M Krause.
• Departement für Innere Medizin, Universitätsspital Zürich.
• Swiss Med Wkly. 1994 May 28; 124 (21): 900-5.

AbstractWe describe the case of a 31-year-old man with a long history of juvenile rheumatoid arthritis who was admitted to the hospital because of painful purple skin lesions on hands and feet. On admission he presented the classical picture of "purpura fulminans" with extensive acrocyanosis and large blisters on the lower limbs which evolved into symmetrical peripheral gangrene. Laboratory findings revealed activated intravascular coagulation and bacteremia with coagulase-negative staphylococci and Xanthomonas maltophilia which was thought to be catheter-related. His condition improved markedly under therapy with antibiotics, intravenous heparin, iloprost and intensive local debridement including amputation of several toes. Coagulation studies two months after the acute phase of the disease revealed chronic activated coagulation with a significant protein S deficiency. Clinical findings, etiology, significance of impaired coagulation and therapeutic action in "purpura fulminans" are discussed.

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