Case Presentation
A 47 years-old patient, presented to our department of dermatology with chronic leg ulcer complicated by dermohypodermitis (figure1). He was treated with Intravenous amoxicillin-clavulanic acid and prophylaxis doses of sub cutaneous enoxaparin 40 mg per Day. A venous Doppler ultrasound was performed to explore the persistence of his leg edema, which did not show any signs of thrombophlebitis, and ciprofloxacin 800 mg IV per day was added. Two days later, a cutaneous examination was remarkable for mild petechial lesions in his lower limbs. He complained of dyspnea, sweating and tachycardia. We realized a pulmonary scintigraphy which revealed a distal pulmonary embolism. His laboratory data showed a microcytic anemia (hemoglobin level of 8.6 g/dL), thrombocytopenia (platelet count of 12000/mm3), hepatic cytolysis and renal failure. A peripheral-blood smear showed schistocytes. Coomb’s test, and the rest of the immunological workup were negative. All of these symptoms led us to raise the diagnosis of TTP. The diagnosis was confirmed with ADAMTS13 activity under 5% and an elevated rate Anti-ADAMTS13 autoantibody. Three days later the patient presented with a generalized seizure with post-critical deficit state. The patient was transferred to the medical intensive care unit. Five sessions of plasmapheresis with steroids (80mg/day) were performed with a transient improvement. The patient received four doses of weekly rituximab at a dose of 375 mg/m2 per dose. The evolution was marked by a therapeutic escape and the appearance of thrombosis of multiple vessels. The Patient died of multiple organ failure, five days later.