Betrosian Alex P, Balla Margarita, Papanikolaou Metaxia, Christopoulou Maria, Kitsios Athanasios, Farmakis Epaminondas, Georgiadis George
Intensive Care Unit, Hippokration General Hospital, Athens, and the Intensive Care Unit, Corfu General Hospital, Corfu, Greece.
Am J Med Sci. 2004 Jun;327(6):373-5. doi: 10.1097/00000441-200406000-00016.
Purpura fulminans (PF) is a life-threatening disorder characterized by acute onset of progressive cutaneous hemorrhage, necrosis, and disseminated intravascular coagulation. Acute infectious PF occurs most commonly in the setting of meningococcal sepsis. When PF occurs in the setting of systemic lupus erythematosus (SLE), the catastrophic antiphospholipid antibody syndrome (CAPS) must be ruled out because urgent therapy is required. Plasmapheresis is effective in both cases, but immunosuppression (high-dose corticosteroids plus cyclophosphamide), although beneficial in patients with CAPS, could be harmful in patients with meningococcal PF. The authors report here a patient with SLE who presented to the intensive care unit with meningococcal PF, acute renal failure, and acute respiratory distress syndrome and discuss clinical similarities and laboratory differences from CAPS.
暴发性紫癜（PF）是一种危及生命的疾病，其特征为进行性皮肤出血、坏死和弥散性血管内凝血的急性发作。急性感染性PF最常见于脑膜炎球菌败血症的情况下。当PF发生在系统性红斑狼疮（SLE）背景下时，必须排除灾难性抗磷脂抗体综合征（CAPS），因为需要紧急治疗。血浆置换在这两种情况下均有效，但免疫抑制（大剂量皮质类固醇加环磷酰胺）虽然对CAPS患者有益，但对脑膜炎球菌性PF患者可能有害。作者在此报告了一名患有SLE的患者，该患者因脑膜炎球菌性PF、急性肾衰竭和急性呼吸窘迫综合征入住重症监护病房，并讨论了与CAPS的临床相似性和实验室差异。
