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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 na...
Citations
... It was first described by Guelliot et al. in 1844 [1]. Neisseria meningitidis is the most common causative organism of AIPF, followed by Streptococcus pneumoniae and Haemophilus influenzae [3]. Bacteria of the genera Streptococcus [4,5], Staphylococcus aureus [6], and Capnocytophaga canimorsus have also been reported [7]. ...
The patient was a man in his 80s who had undergone laparoscopic anterior resection for rectal cancer. Bowel obstruction occurred on the third postoperative day but improved with a decompression tube by the fifth postoperative day. A high fever (in the 38 °C range) was also observed. Blood culture tests detected two sets of the gram-negative bacilli Klebsiella aerogenes within 24 h of collection. On the seventh postoperative day, the patient subsequently went into septic shock with disseminated intravascular coagulation (DIC). On the eighth postoperative day, the fingertips and toes became black, and the palms and dorsal surfaces of both feet were dark purple due to peripheral circulatory failure. This suggested acute infectious purpura associated with sepsis (acute infectious purpura fulminans (AIPF)). Intensive care was provided; however, the necrosis of both middle fingers worsened, both middle fingers were gangrenous, and the patient died on the thirtieth postoperative day. AIPF is rarely reported, especially in early-onset cases after elective surgery. We encountered a rare complication of bacterial translocation from postoperative bowel obstruction, leading to AIPF.
... To the best of our knowledge, this is the first study to report on C. violaceum-induced PF. Infection-induced PF is a non-specific response caused by various organisms and has a mortality rate of 41% according to a previous study (9). Shock liver-induced acquired protein C deficiency could result in DIC, causing microvascular thrombosis of the limbs and, eventually, PF (10)(11)(12). ...
... Apart from effective antiinfection treatment, other treatment options include heparin, antithrombin, zymogen protein C concentrates, or recombinant activated protein C (11). Regarding PF-associated SPG, approximately one-third of patients require amputation eventually (9). In our patient, PF-associated SPG was limited to the toes, and PF recovered as the patient was discharged. ...
Chromobacterium violaceum (C. violaceum) is a gram-negative bacillus that is widespread in tropical and subtropical areas. Although C. violaceum rarely infects humans, it can cause critical illness with a mortality rate above 50%. Here, we report the successful treatment of a 15-year-old male who presented with bloodstream infection of C. violaceum along with sepsis, specific skin lesions, and liver abscesses. Cardiogenic shock induced by sepsis was reversed by venoarterial extracorporeal membrane oxygenation (VA ECMO). Moreover, C. violaceum-related purpura fulminans, which is reported herein for the first time, was ameliorated after treatment. This case report demonstrates the virulence of C. violaceum with the aim of raising clinical awareness of this disease.
... Todos los casos se asocian a (3) una deficiencia hereditaria y/o transitoria de las proteínas C y/o S . La mortalidad y la morbilidad de (4) esta afección son altas, requiriendo amputación de los miembros afectos en la mayoría de los casos . ...
... En cuanto a los microorganismos implicados, el principal es el meningococo y en segundo lugar, el neumococo. En un estudio realizado en pacientes internados en una unidad de cuidados intensivos, se vio que la prevalencia de púrpura fulminante en el contexto de una infección por (4) Neisseria meningitis fue de 63,7% y para Streptococcus pneumoniae de 21,9% ...
... El tratamiento debe estar orientado hacia la infección subyacente y al soporte general del paciente. La administración temprana de antibióticos tan pronto como el diagnóstico de púrpura fulminans se ha evocado es una(4 ) importante ventaja terapéutica .Resulta interesante que, a pesar de los avances en el conocimiento de la fisiopatología de esta enfermedad, la mortalidad sigue siendo alta, asociada a una morbilidad no despreciable: amputación, insuficiencia renal, secuelas neurológicas, etc., por lo que el rápido actuar del médico internista y el apoyo multidisciplinario, en este caso del dermatólogo, es fundamental. ...
La púrpura fulminante o purpura fulminans es un síndrome de trombosis microvascular cutánea y necrosis hemorrágica de rápida evolución. Se presenta el caso de un paciente masculino, internado por patología infecciosa y evento cardiovascular agudo, que desarrolla púrpura fulminante por déficit de proteína C, relacionado a cuadro infeccioso concomitante.
... Purpura fulminans is a life-threatening, often fatal condition, if left untreated. 1 Rickettsial infections like scrub typus presenting as purpura fulminans are quite rare, more so when it is coinfected with acute hepatitis E infection. [2][3][4] Protein C and protein S deficiency, inherited or acquired, are related to this ...
... Purpura fulminans (PF) is a rare infectious disease carrying a high mortality and morbidity with 41% of the patients dying in the ICU and 28% of the survivors requiring limb amputations with a median number of 3 limbs amputated [1][2][3]. Neisseria meningitidis is the leading responsible bacterium accounting for two thirds of PF [1]. Obtaining a microbiological documentation of PF is crucial for confirming the diagnosis, as well as for adjusting the antibiotic therapy. ...
... Purpura fulminans (PF) is a rare infectious disease carrying a high mortality and morbidity with 41% of the patients dying in the ICU and 28% of the survivors requiring limb amputations with a median number of 3 limbs amputated [1][2][3]. Neisseria meningitidis is the leading responsible bacterium accounting for two thirds of PF [1]. Obtaining a microbiological documentation of PF is crucial for confirming the diagnosis, as well as for adjusting the antibiotic therapy. ...
... Given the high susceptibility of Neisseria meningitidis to β-lactam antibiotics, together with the high proportion of patients empirically treated before ICU admission [1], blood cultures may be sterile in half of the patients with meningococcal PF [4]. Moreover, lumbar puncture has been shown to be of limited diagnostic value in this context [5], if not contra-indicated because of severe thrombocytopenia and coagulation disorders, which are almost constant in patients with PF [1,4]. ...
Background
Neisseria meningitidis is the leading responsible bacterium of Purpura Fulminans (PF) accounting for two thirds of PF. Skin biopsy is a simple and minimally invasive exam allowing to perform skin culture and polymerase chain reaction (PCR) to detect Neisseria meningitidis . We aimed to assess the sensitivity of skin biopsy in adult patients with meningococcal PF.
Methods
A 17-year multicenter retrospective cohort study including adult patients admitted to the ICU for a meningococcal PF in whom a skin biopsy with conventional and/or meningococcal PCR was performed.
Results
Among 306 patients admitted for PF, 195 had a meningococcal PF (64%) with a skin biopsy being performed in 68 (35%) of them. Skin biopsy was performed in median 1 day after the initiation of antibiotic therapy. Standard culture of skin biopsy was performed in 61/68 (90%) patients and grew Neisseria meningitidis in 28 (46%) of them. Neisseria meningitidis PCR on skin biopsy was performed in 51/68 (75%) patients and was positive in 50 (98%) of them. Among these 50 positive meningococcal PCR, five were performed 3 days or more after initiation of antibiotic therapy. Finally, skin biopsy was considered as contributive in 60/68 (88%) patients. Identification of the meningococcal serogroup was obtained with skin biopsy in 48/68 (71%) patients.
Conclusions
Skin biopsy with conventional culture and meningococcal PCR has a global sensitivity of 88% and should be systematically considered in case of suspected meningococcal PF even after the initiation of antimicrobial treatment.
... It also depended on the clinical presentation and the clonal complex. Contou et al. reported the highest CFR (36.9%) in adults diagnosed with purpura fulminans caused by N. meningitidis [47], whereas Floret reported the highest CFR (79%) in the pediatric population [35]. Sg W:cc11 was also associated with higher CFR (4.0-27.8%) ...
... For instance, Weil-Olivier et al. reported 1.4-1.9% of adult cases (aged >19 years) with IMD having amputations [46]. Higher rates (11.6%) were reported in adult cases with N. meningitidis purpura fulminans [47]. In the same way, a prospective observational study found that 34.3% of adult cases had depressive symptoms one year after being discharged [40]. ...
Invasive meningococcal disease (IMD) remains a significant health concern due to its unpredictable nature and its rapid progression. Even if occurrence of IMD is strictly monitored by a national surveillance network, no information on long-term sequelae is reported, making it difficult to assess the entire clinical burden of IMD in France. The aim of this scoping review was to analyze the epidemiology and the clinical burden of IMD in France by reporting the main epidemiological parameters, and by describing the clinical consequences and the care pathway of patients. The process of the review followed the Preferred Reporting Items for Systematic Reviews and Meta-Analyses extension to the Scoping Reviews guidelines. In France, the incidence of IMD cases has been fluctuating over time, characterized by an overall downward trend linked to a decrease in Sg B cases and the introduction of mandatory vaccination against Sg C. Sg W cases increased in recent years (from 5% to 21% in 2019). The case fatality rate remained constant (6–12.9%). The most frequently reported sequelae were severe neurological disorder, epilepsy, and anxiety. However, data on sequelae and care pathways were scarce. Further research should concentrate on providing robust identification of sequelae and the subsequent impact on quality of life, as well as on the organization of optimal care and support for patients and their families.
... L e purpura fulminans (PF) est une maladie infectieuse rare caractérisée par l'association d'un état de choc septique et d'un purpura d'apparition et d'extension rapides. [1][2][3] Le PF représente moins de 0,5 % des causes de choc septique de l'adulte. 4 Les deux principales bactéries responsables sont le méningocoque et le pneumocoque. 1 Bien que ces bactéries soient extrêmement sensibles aux antibiotiques disponibles, le pronostic du PF reste sombre, avec une mortalité élevée en réanimation et un risque de séquelles sévères à distance chez les patients survivants. ...
... 4 Les deux principales bactéries responsables sont le méningocoque et le pneumocoque. 1 Bien que ces bactéries soient extrêmement sensibles aux antibiotiques disponibles, le pronostic du PF reste sombre, avec une mortalité élevée en réanimation et un risque de séquelles sévères à distance chez les patients survivants. 1,5 Quelles sont les bactéries responsables du purpura fulminans ? ...
... 4 Les deux principales bactéries responsables sont le méningocoque et le pneumocoque. 1 Bien que ces bactéries soient extrêmement sensibles aux antibiotiques disponibles, le pronostic du PF reste sombre, avec une mortalité élevée en réanimation et un risque de séquelles sévères à distance chez les patients survivants. 1,5 Quelles sont les bactéries responsables du purpura fulminans ? ...
PURPURA FULMINANS IN ADULT PATIENTS. Purpura fulminans is a rare life-threatening infectious disease characterized by the association of a sudden and extensive purpuric rash together with an acute circulatory failure. PF commonly affects young patients with no previous comorbidities. Neisseria meningitidis and Streptococcus pneumoniae are the leading causative bacteria. Diagnosing purpura fulminans before the apparition of the purpuric rash is challenging since prodromal symptoms are nonspecific and consistent with a "flu-like" syndrome. The clinical presentation of patients with purpura fulminans differs from that of patients with bacterial meningitis since most of the patients with purpura fulminans have no neurological impairment. Microbiological diagnosis relies on blood cultures and skin biopsy of purpuric lesions. The indication for lumbar puncture must be evaluated on a case-by-case basis because patients usually have no neurological signs but severe coagulation disorders. Treatment is no different from that of any other septic shock: antibiotic therapy with a third-generation cephalosporin as soon as the diagnosis is suspected and treatment of associated organ failures. Despite these pathogens being highly susceptible to broadly available antibiotics, the prognosis of PF is dismal with a mortality rate of 40% in the intensive care unit and a significant risk of distant sequelae in surviving patients.
... The clinical picture of PF is often dominated by shock with hypotension and hypovolemia. The cutaneous lesions are marked by vascular occlusion features, including retiform, branching purpuric patches that rapidly evolve into hemorrhagic necrosis, and are sometimes preceded by bulla formation [53,54]. In the acute phase, the laboratory findings of PF are those of the associated DIC, including thrombocytopenia, hypofibrinogenemia, increased fibrin degradation products (FDP), and prolonged prothrombin (PT) and activated partial thromboplastin (aPTT) times. ...
This section covers the strategies for managing the dermatologic infectious diseases and how they present in emergency and acute settings. In order to keep the staff and patients safe, emergency physicians should be able to limit the nosocomial infection spread.
... Several recent reports have been published based on a 17-year multicenter retrospective cohort study performed in France which included patients hospitalized with infectious PF in intensive care units [2][3][4][5][6]. Infectious PF was shown to be associated with 41 % mortality and high morbidity in those who survived: almost one-third required limb amputation [5]. ...
... Several recent reports have been published based on a 17-year multicenter retrospective cohort study performed in France which included patients hospitalized with infectious PF in intensive care units [2][3][4][5][6]. Infectious PF was shown to be associated with 41 % mortality and high morbidity in those who survived: almost one-third required limb amputation [5]. The most common infectious etiology of PF is N. meningitidis (64 %), followed by S. pneumoniae (22 %) [5]. ...
... Infectious PF was shown to be associated with 41 % mortality and high morbidity in those who survived: almost one-third required limb amputation [5]. The most common infectious etiology of PF is N. meningitidis (64 %), followed by S. pneumoniae (22 %) [5]. Numerous case reports describe PF secondary to S. pneumoniae infection in the setting of asplenia or hyposplenia [7][8][9][10][11]. ...
Purpura fulminans (PF) is a skin disorder with high morbidity and mortality which is characterized by microvascular thrombosis and development of hemorrhagic necrosis. PF can be caused by acute infection, most commonly due to Neisseria meningitidis, followed by Streptococcus pneumoniae. Prior reports describe cases of pneumococcal PF occurring in patients with asplenia or hyposplenia, though cases have also been reported in otherwise healthy adults without known splenic disease. Herein, we report a young adult patient with cirrhosis due to autoimmune hepatitis who had not received pneumococcal vaccination and developed rapidly progressive fatal S. pneumoniae sepsis with PF.
... We herein report a case of purpura fulminans due to Enterococcus cecorum in an asplenic patient resulting in an overwhelming sepsis with death occurring less than 12 h after ICU admission despite prompt and effective empirical antibiotic therapy. The leading causative bacteria for purpura fulminans are Neisseria meningitidis and Streptococcus pneumoniae [1], with half of pneumococcal purpura fulminans occurring in asplenic or hyposplenic patients [2] which are well-known to be exposed to fulminant sepsis due to encapsulated bacteria [3], the so-called "overwhelming post-splenectomy infection" (OPSI). The prognosis of patients with purpura fulminans and OPSI is dismal with high ICU mortality [1,3]. ...
... The leading causative bacteria for purpura fulminans are Neisseria meningitidis and Streptococcus pneumoniae [1], with half of pneumococcal purpura fulminans occurring in asplenic or hyposplenic patients [2] which are well-known to be exposed to fulminant sepsis due to encapsulated bacteria [3], the so-called "overwhelming post-splenectomy infection" (OPSI). The prognosis of patients with purpura fulminans and OPSI is dismal with high ICU mortality [1,3]. To the best of our knowledge, Enterococcus cecorum have never been reported as a causative bacterium for purpura fulminans or OPSI. ...
Enterococcus cecorum was initially isolated from the intestine of poultry and is an uncommon cause of human infection. We report here what we believe to be the first case of overwhelming post-splenectomy infection (OPSI) with purpura fulminans due to Enterococcus cecorum in a 51-year-old man. As opposed to other enterococci, Enterococcus cecorum remains susceptible to third-generation cephalosporin which is the first line empirical antibiotic therapy for both patients with purpura fulminans and asplenic patients with sepsis. Despite adequate antibiotic therapy, evolution in the intensive care unit (ICU) was overwhelming with death occurring 10 hours after ICU admission.
