Acute infectious purpura fulminans: a case series from India.

Acute infectious purpura fulminans is a serious, potentially fatal condition. We present a case series of 11 patients from March 2005 to March 2017, whose clinical symptoms were fever (100%), confusion (63.6%) and headache (55%), and whose common laboratory abnormalities were thrombocytopenia (100%), elevated alkaline phosphatase (70%) and anaemia (63.6%). Three patients (27%) developed gangrene and two presented in shock. Only one grew Neisseria meningitidis in cerebrospinal fluid (CSF) culture and another confirmed by latex agglutination and polymerase chain reaction in CSF. Five others had serology confirmed spotted fever rickettsioses (SFG). All received broad spectrum antibiotics; in 9/11 patients, this included doxycycline or azithromycin. The mean hospital stay was 10.2 days and overall mortality was 18.2%.

Clinical spectrum and short-term outcome of adult patients with purpura fulminans: a French multicenter retrospective cohort study.

PURPOSE: Data on purpura fulminans (PF) in adult patients are scarce and mainly limited to meningococcal infections. Our aim has been to report the clinical features and outcomes of adult patients admitted in the intensive care unit (ICU) for an infectious PF, as well as the predictive factors for limb amputation and mortality. METHODS: A 17-year national multicenter retrospective cohort study in 55 ICUs in France from 2000 to 2016, including adult patients admitted for an infectious PF defined by a sudden and extensive purpura, together with the need for vasopressor support. Primary outcome variables included hospital mortality and amputation during the follow-up period (time between ICU admission and amputation, death or end of follow-up). RESULTS: Among the 306 included patients, 126 (41.2%; 95% CI 35.6-46.9) died and 180 (58.8%; 95% CI 53.3-64.3) survived during the follow-up period [13 (3-24) days], including 51/180 patients (28.3%, 95% CI 21.9-35.5) who eventually required limb amputations, with a median number of 3 (1-4) limbs amputated. The two predominantly identified microorganisms were Neisseria meningitidis (63.7%) and Streptococcus pneumoniae (21.9%). By multivariable Cox model, SAPS II [hazard-ratio (HR) = 1.03 (1.02-1.04); p < 0.001], lower leucocytes [HR 0.83 (0.69-0.99); p = 0.034] and platelet counts [HR 0.77 (0.60-0.91); p = 0.007], and arterial blood lactate levels [HR 2.71 (1.68-4.38); p < 0.001] were independently associated with hospital death, while a neck stiffness [HR 0.51 (0.28-0.92); p = 0.026] was a protective factor. Infection with Streptococcus pneumoniae [sub-hazard ratio 1.89 (1.06-3.38); p = 0.032], together with arterial lactate levels and ICU admission temperature, was independently associated with amputation by a competing risks analysis. CONCLUSION: Purpura fulminans carries a high mortality and morbidity. Pneumococcal PF leads to a higher risk of amputation. TRIALS REGISTRATION: NCT03216577.

Fatal meningococcal septicemia without meningeal signs, contribution of the peripheral smear in diagnosis: Report of a case.

Acute meningococcemia is characterized by extensive purpurae consisting of both petechiae and ecchymoses. This condition can be rapidly fatal without treatment due to shock and severe consumptive coagulopathy. We report a case of fatal meningococcal septicemia in a military recruit who presented with fever and associated rapidly progressive purpuric rash (purpura fulminans) without any meningeal signs. Evaluation revealed evidence of disseminated intravascular coagulopathy and multiorgan failure. Diplococci were demonstrated in peripheral blood neutrophils and monocytes. On autopsy, extensive hemorrhages were found in both adrenals, lungs, liver, skin, and kidneys with secondary hemophagocytic lymphohistiocytosis in bone marrow. This report highlights useful information obtained from examination of peripheral blood smear in purpura fulminans.

A Fatal Case of Invasive Infection Caused By W135 Neisseria meningitidis in a Vaccinated French Soldier.

We report on the case of fatal "purpura fulminans" caused by Neisseria meningitidis W135 that occurred in a young French soldier vaccinated a few months earlier with the tetravalent conjugate vaccine ACYW135. Biological investigations revealed adequate titers of postvaccination antibodies against serogroups A, C, and W135 and led to the post-mortem diagnosis of a complete C7 complement deficiency. Late complement component deficiency is a well-known risk factor of meningococcal diseases, but usually exposes to recurrent mild infections, whereas severe invasive meningococcal diseases are more likely to occur among properdin-deficient patients. Awareness of the potentially life-threatening nature of late complement component deficiency should lead to improved diagnosis among young people, especially when past medical history reveals recurrent mild infections.

[Sepsis-associated Purpura Fulminans International Registry--Europe (SAPFIRE)]. / Ein europäisches Register für sepsisassoziierte Purpura fulminans (SAPFIRE).

BACKGROUND: Purpura fulminans is a rare life-threatening condition which is characterized by disseminated thrombosis in dermal and systemic microcirculation, cutaneous hemorrhages with progressing necrosis and multiple organ failure. The underlying pathogenesis is based on the disruption of the intrinsic anticoagulation cascade, with protein C deficiency being considered the leading factor in this process. In the majority of cases, the condition emerges as consumptive coagulopathy associated with severe sepsis. OBJECTIVES: Epidemiological data on sepsis-associated purpura fulminans (SAPF) are scarce and evidence-based treatment guidelines have not been established yet. While restoration of the balance in the coagulation cascade is a declared therapeutic goal, evaluations of the efficacy of different therapeutic approaches in randomized clinical trials are still lacking. The causal role of individual microbial pathogens also requires comprehensive evaluation. METHODS: A prospective multicenter Sepsis-Associated Purpura Fulminans International Registry-Europe (SAPFIRE) will be established in the first quarter of 2015. For the first time, participating centers will systematically collect information on etiology, clinical course, biomarkers, treatment, morbidity, and mortality of SAPF. RESULTS: The SAPFIRE data will be periodically evaluated and disseminated. Retrospective analysis of each center's data and regular access to aggregated information collected by other centers will enable the participants to monitor and update care quality standards.

[Impact of corticosteroids in the immediate management of invasive meningococcal disease associated with hyperinvasive strains of the ST-11 clonal complex in children]. / Impact des corticoïdes dans la prise en charge immédiate des infections invasives à méningocoque liées aux souches hyper-invasives du complexe clonal ST-11 chez l'enfant.

OBJECTIVES: We used data from the Groupe de pathologie infectieuse pédiatrique and Association clinique et thérapeutique infantile du Val-de-Marne (GPIP/ACTIV) National Survey of Bacterial Meningitis in children and the National Reference Center for Meningococci (CNRM) microbiological data to assess the potential impact of corticosteroids on the immediate management of invasive meningococcal disease (IMD) associated with different genotypes, including highly pro-inflammatory strains of the ST-11 clonal complex (genotype ST-11). METHODS: From 2001 to 2009, 259 pediatric wards and 168 microbiology laboratories distributed throughout France prospectively included all under-18-year-old patients with IMD (meningitis or purpura fulminans). The strains were sent to the CNRM for genotyping. We linked the ACTIV clinical data of IMD cases, where information on corticosteroid therapy was available, to strains isolated by the CRNM. RESULTS: A total of 1981 IMD cases were identified during the 8-year study, 805 cases (712 [88.5%] bacterial meningitis and 93 [11.5%] purpura fulminans) had steroid treatment data (33.8% received corticosteroids). The genotype of the strains was available for 410 patients (24.4% related to genotype ST-11; 100 patients). For all cases and regardless of the corticosteroids, mortality was significantly associated with the genotype ST-11 (OR=2.39, 95% CI [1.29; 4.42], P=0.004). For all cases and regardless of the genotypes of the isolates, mortality was also significantly higher for children with than without corticosteroid therapy (12.7% versus 4.5%, P<0.001). However, this treatment had been prescribed more frequently in severe cases, including shock, PF, coma and/or mechanical ventilation. For children who did not receive corticosteroids, the mortality rate was significantly higher with genotype ST-11 compared to other genotypes (OR=4.68 [1.91, 11.46], P=0.001). This difference disappeared in children who received corticosteroids. CONCLUSION: This study indicates that in the absence of corticosteroids, higher mortality in invasive meningococcal disease is associated with the ST-11 clonal complex strains. This suggests a possible positive effect of corticosteroid therapy depending on the genotype of the strain involved.

Severe meningococcal infection: a review of epidemiology, diagnosis, and management.

Neisseria meningitidis, also known as meningococcus, is a relatively uncommon cause of invasive infection, but when it occurs it is frequently severe and potentially life threatening. Meningococcus should be considered and investigated promptly as a potentially etiologic pathogen in any patient with meningitis, or sepsis accompanied by a petechial rash. Suspected patients should receive early appropriate antimicrobial therapy concomitantly with confirmatory invasive diagnostic tests. Vaccines have reduced the incidence of infection with certain non-B meningococcal serogroups, and new serotype B vaccines are on the horizon. This article reviews the epidemiology, diagnosis, and management of severe meningococcal infections.

Surgical management of acute infectious purpura fulminans.

Purpura fulminans is a syndrome characterized by hemorrhagic infarction of the skin and underlying soft tissue as a result of disseminated intravascular coagulation and intravascular thrombosis. In this study, the authors report their experience with surgical intervention for acute infectious purpura fulminans (AIPF). A retrospective chart review was performed including all patients diagnosed with AIPF from January 1, 2006, to December 31, 2008, and treated at an academic medical center. Primary endpoints of interest were overall survival rate and the need for and level of eventual amputation. Improvement in limb perfusion was included as a secondary endpoint. Nine patients were diagnosed with AIPF at the authors' institution during a 3-year period, and seven of these diagnoses were made within 12 months. Overall mortality was 44% (5/9). Amputation was required in 80% of survivors (4/5). All patients explored within 24 hours of diagnosis had evidence for compartment syndrome with visible bulging muscle on fascial release. AIPF is a devastating disease with significant mortality and morbidity primarily related to the loss of multiple limbs. This study suggests that early diagnosis and surgical intervention in the form of compartment release and sympathectomy should be performed concurrently with the initial treatment of sepsis to minimize amputations in surviving patients.