PURPURA FULMINANS IN STILLS DISEASE AND B ACTEREMIA WITH XANTHOMONAS-MALTOPHILIA AND COAGULASE-NEGATIVE STAPHYLOCOCCI

We describe the case of a 31-year-old man with a long history of juven ile rheumatoid arthritis who was admitted to the hospital because of p ainful purple skin lesions on hands and feet. On admission he presente d the classical picture of ''purpura fulminans'' with extensive acrocy anosis and large blisters on the lower limbs which evolved into symmet rical peripheral gangrene. Laboratory findings revealed activated intr avascular coagulation and bacteremia with coagulase-negative staphyloc occi and Xanthomonas maltophilia which was thought to be catheter-rela ted. His condition improved markedly under therapy with antibiotics, i ntravenous 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, sign ificance of impaired coagulation and therapeutic action in ''purpura f ulminans'' are discussed.