Ikuto Takeuchi¹, Ken-ichi Muramatsu¹, Soichiro Ota¹, Youichi Yanagawa^1,

Purpura fulminans (PF) is a rare, life-threatening syndrome characterized by disseminated intravascular coagulation (DIC) and endovascular thrombosis resulting in a characteristic pattern of cutaneous purpura. There have been no reports of the combination of PF and anti-centromere antibody (ACA). A 70-year-old woman with a 2-day history of lumbago was transported from a local medical facility to our hospital due to right hydronephrosis due to ureterolithiasis and urosepsis. She had systemic lupus erythematosus (SLE) and schizophrenia. On arrival, she had consciousness disturbance and was in a shock state with cyanotic extremities. She was additionally diagnosed with renal failure, DIC and ACA positivity after examinations. She was treated with vasopressor, antibiotics and ureter stent placement. However, she developed unstable circulation, which necessitated tracheal intubation and continuous hemodiafiltration. Urine and blood cultures showed Escherichia coli. However, the cyanosis of all of her extremities changed to necrosis and she died on day 8 due to multiple organ failure. This is the first case of acute infectious PF with ACA positivity. As ACA is known to be a risk factor for digital necrosis, the present case suggests that in addition to a decrease of protein C or S, ACA may contribute to the development of PF.

purpura fulminans, anticentromere antibody, pyelonephritis