title: Meningococcal purpura fulminans in a patient with systemic lupus erythematosus: a mimic for catastrophic antiphospholipid antibody syndrome?
abstract: 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.
authors.affiliation: Intensive Care Unit, Hippokration General Hospital, Athens, and the Intensive Care Unit, Corfu General Hospital, Corfu, Greece. abetrosian@netscape.net
authors.forename: Alex P
authors.initials: AP
authors.lastname: Betrosian
authors.forename: Margarita
authors.initials: M
authors.lastname: Balla
authors.forename: Metaxia
authors.initials: M
authors.lastname: Papanikolaou
authors.forename: Maria
authors.initials: M
authors.lastname: Christopoulou
authors.forename: Athanasios
authors.initials: A
authors.lastname: Kitsios
authors.forename: Epaminondas
authors.initials: E
authors.lastname: Farmakis
authors.forename: George
authors.initials: G
authors.lastname: Georgiadis
mesh_terms: D000328:Adult; D016736:Antiphospholipid Syndrome / Q000175:diagnosis*; D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D011695:IgA Vasculitis / Q000175:diagnosis*; D008180:Lupus Erythematosus, Systemic / Q000175:diagnosis*; D008589:Meningococcal Infections / Q000175:diagnosis*