Skin biopsy in adult patients with meningococcal purpura fulminans: a multicenter retrospective cohort study.

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.

Meningococcal purpura fulminans and severe myocarditis with clinical meningitis but no meningeal inflammation: a case report.

BACKGROUND: During fulminant meningococcal septicaemia, meningococci are often observed in the cerebrospinal fluid (CSF) although the patients have frequently no meningeal symptoms. Meningococcal meningitis, by contrast, usually features clinical meningeal signs and biochemical markers of inflammation with elevated white blood cell count (pleiocytosis) in the CSF. Cases of typical symptomatic meningitis without these biochemical features are uncommon in adults. CASE PRESENTATION: A 21-year-old male presented with meningococcal purpura fulminans and disseminated intravascular coagulation (DIC) associated with multiple organ dysfunction syndrome requiring hospitalization in the Intensive Care Unit. Despite typical meningeal clinical signs, lumbar puncture showed no pleiocytosis, normal glycorachia and normal proteinorachia, whereas the lactate concentration in the CSF was high (5.8 mmol/L). CSF culture showed a high inoculum of serogroup C meningococci. On day 2, after initial improvement, a recurrence of hypotension led to the diagnosis of acute meningococcal myocarditis, which evolved favourably within a week. During the hospitalization, distal ischemic and necrotic lesions were observed, predominantly on the fingertips, which were treated with local and systemic vasodilators. CONCLUSIONS: We report a rare case of adult meningococcal disease characterized by an intermediate form of meningitis between purulent meningitis and meningeal inoculation from fulminant meningococcal septicaemia, without classical signs of biological inflammation. It highlights the diagnostic value of CSF lactate, which may warrant administration of a meningeal dosing regimen of beta-lactam antibiotics. This case also demonstrates the potential severity of meningococcal myocarditis; we discuss its pathophysiology, which is distinct from other sepsis-related cardiomyopathies. Finally, the observed effects of vasodilators on the meningococcal skin ischemia in this case encourages future studies to assess their efficacy in DIC-associated necrosis.

Staphylococcal Purpura Fulminans: Report of a Case.

Purpura fulminans (PF) is associated with several infections and most commonly with meningococcemia. However, there are only a few reports of this entity in association with toxic shock syndrome toxin-1-producing Staphylococcus aureus. We report a 53-year-old man who presented with fever, progressive hemodynamic instability, multiorgan failure, and thrombocytopenia following lobectomy for a solitary lung metastasis from rectal adenocarcinoma. He developed progressive generalized eruption of nonblanching red, purple, and black macules, papules, and plaques on the trunk and extremities consistent with PF. He died on postadmission day 3. Autopsy examination revealed purulent pleural exudate, which grew toxic shock syndrome toxin-1-producing S. aureus. Premortem and autopsy skin biopsies demonstrated epidermal necrosis, subepidermal bullae, and fibrin thrombi within small cutaneous vessels with minimal perivascular lymphocytic inflammation and without accompanying vasculitis. With this case report, we would like to draw attention to the fact that staphylococcal toxic shock syndrome-associated PF may be highly underrecognized and much more common than reflected in the literature.

Group A Escherichia coli-related purpura fulminans: an unusual manifestation due to an unusual strain?

We describe an exceptional case of life-threatening group A Escherichia coli-induced purpura fulminans. Genotyping of common polymorphisms in genes involved in innate immunity or coagulation did not reveal known susceptibility to such a manifestation. Genetic analysis of the strain revealed an unusual conserved virulence plasmidic region, pointing out its potential virulence.

Two cases of meningococcal purpura fulminans: the 'less is more' approach.

This study presents a case series of patients with meningococcal purpura fulminans who were treated at a tertiary referral centre within a few days of each other. Presenting with signs and symptoms of florid meningococcal sepsis, they were managed initially by physicians and intensivists, whereas the development of large purpuric areas and tissue necrosis was managed expectantly by plastic surgeons. When the patients were deemed to have recovered clinically and the necrosis delineated, surgical management was implemented with subsequent involvement of various rehabilitation services. This article highlights the cases of two patients, and their clinical presentation, management and rehabilitation together with a current literature review on this area.

Diagnostic value of polymerase chain reaction analysis of skin biopsies in purpura fulminans.

Even though prompt diagnosis and treatment of purpura fulminans (PF) is essential to reduce mortality, early administration of antibiotics may preclude identification of the causative agent by standard bacterial cultures and thus render definitive diagnosis impossible. Here we present a case of an infant with PF and negative bacterial cultures for whom polymerase chain reaction (PCR) analysis of a cutaneous biopsy specimen obtained 4 days after initiation of antibiotics identified the genomic sequence of Neisseria meningitidis genogroup C. When bacterial cultures fail to provide useful information, PCR of skin biopsy specimens can be a valuable diagnostic tool in PF.

Neonatal purpura fulminans due to methicillin resistant Staphylococcus aureus.

Neonatal purpura fulminans is rare and may be inherited or acquired. It may ultimately lead to multiorgan failure and death. Purpura fulminans in a premature neonate resulting from Staphylococcus aureus septicemia is illustrated. Unfortunately, the baby succumbed to septicemia.

[Purpura fulminans in adult patients]. / Purpura fulminans de l'adulte.

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.

PURPURA FULMINANS DE L'ADULTE. Le purpura fulminans (PF) est une maladie infectieuse rare touchant préférentiellement l'adulte jeune sans comorbidités. Il se définit par l'association d'un état de choc septique et d'un purpura d'apparition et d'extension rapides. Les deux principales bactéries responsables sont le méningocoque et le pneumocoque. L'éruption purpurique est précédée par une phase prodromique faite de symptômes aspécifiques (syndrome pseudogrippal) rendant difficile un diagnostic précoce. La présentation clinique des patients ayant un purpura fulminans diffère de celle des patients ayant une méningite bactérienne. Le diagnostic microbiologique repose sur les hémocultures et sur la biopsie cutanée. L'indication de la ponction lombaire est à évaluer au cas par cas car les patients n'ont le plus souvent aucun signe neurologique mais des troubles sévères de l'hémostase contre-indiquant le geste. La prise en charge des patients ayant un PF n'a aucune spécificité comparativement à celle des patients ayant un choc septique lié à une autre porte d'entrée : antibiothérapie par une céphalosporine de troisième génération dès la suspicion diagnostique et traitement des défaillances d'organes associées. Bien que les bactéries responsables de purpura fulminans soient extrêmement sensibles aux antibiotiques, le pronostic du PF reste sombre, avec une mortalité en réanimation s'élevant à 40 % et un risque important de séquelles à distance chez les patients survivants.

Two cases of infectious purpura fulminans and septic shock caused by Capnocytophaga canimorsus transmitted from dogs.

We report 2 cases of Capnocytophaga canimorsus-induced septicaemia complicated by purpura fulminans in previously healthy individuals, both of whom had been exposed to dog saliva prior to disease. They both presented with purpuric skin lesions, as well as the tetrad of abdominal symptoms, haemolytic anaemia, metabolic acidosis, and renal failure, which may be common in C. canimorsus-associated purpura fulminans. The patients survived after treatment with broad-spectrum antibiotics and supportive intensive care. C. canimorsus should be considered as a possible cause of infectious purpura fulminans in the unresolved critically ill patient with a history of dog exposure.

Purpura fulminans caused by community-associated methicillin-resistant Staphylococcus aureus.

Sepsis-induced purpura fulminans is a rare but life-threatening condition characterized by rapidly progressive hemorrhagic infarction of the skin due to dermal vascular thrombosis resulting in tissue loss and severe scarring. Although most commonly related to meningococcal or invasive group A streptococcal disease, it may also be caused by several other bacterial or viral pathogens including Pneumococcus and Varicella. Purpura fulminans associated with Staphylococcus aureus sepsis is rare but has been reported in adults. However, the syndrome is very unusual in children, and to our knowledge, only 2 cases of staphylococcal purpura fulminans have been reported in children, both due to methicillin-susceptible S aureus in the United Kingdom. We report the first well-described case of purpura fulminans due to community-associated methicillin-resistant S aureus in a child.

Human skin allografts as a useful adjunct in the treatment of purpura fulminans.

OBJECTIVE: The aim of this report is to discuss the role of human skin allografts in surgical coverage procedures for patients with purpura fulminans. METHOD: We describe cases of purpura fulminans in three adults and one infant treated at our burns unit between October 2006 and January 2008. RESULTS: The application of cryopreserved human skin allografts allowed us to obtain immediate wound closure after necrosis excision and enabled our team to subsequently perform autografts on favourable graft recipient sites. CONCLUSION: Recourse to human skin allografts must be considered a pertinent therapeutic option in patients with purpura fulminans.

Non-typeable Haemophilus influenzae and purpura fulminans.

Haemophilus influenzae typically causes illness and infection in the paediatric population. We report a case of a 53-year-old man who developed invasive non-typeable H. influenzae infection associated with purpura fulminans and multiorgan failure. On review of the literature, this is the first reported case of non-typeable H. influenzae causing purpura fulminans. The patient was treated with intravenous ceftriaxone 2 g/day and was eventually discharged from the hospital almost 2 months after admission. We discuss the role that infection/sepsis plays in disturbances to the coagulation cascade leading to purpura fulminans and the virulence factors that make non-typeable H. influenzae unique. Finally, we review other cases of H. influenzae associated with purpura fulminans and discuss the similarities with our case.

Successful treatment of meningococcal bacteremia using oral doxycycline: A case report.

We report the case of an 18-year-old immunocompetent man who presented to the hospital with fever, headaches, and arthromyalgia, which progressed to include an erythematous rash. He had a history of a tick bite 72 h earlier. The diagnosis of rickettsial infection was suspected and a course of doxycycline was initiated for a total of 5 days. His evolution was rapidly favorable under treatment, with resolution of the symptoms within 24 h. Blood cultures came back positive for Neisseria meningitidis serotype B, indicating an authentic purpura fulminans. Purpura fulminans is a medical emergency, a syndrome of intravascular thrombosis characterized by a very rapid evolution that requires early recognition and specific treatment. It is commonly described in the young and healthy patient and has high mortality and morbidity. Common bacteria mainly associated with purpura fulminans are Meningococcus spp., Pneumococcus spp., and Staphylococcus spp.

Septic Shock and Purpura Fulminans Due to Streptococcus pneumoniae Bacteremia in an Unvaccinated Immunocompetent Adult: Case Report and Review.

BACKGROUND Despite proven efficacy of vaccinations against Streptococcus pneumoniae in preventing infection, only 70% of eligible individuals receive the vaccine in the United States. Pneumococcal bacteremia represents a form of invasive pneumococcal disease and is associated with high mortality, especially in immunocompromised patients and the elderly. Purpura fulminans is a rare complication and manifestation of disseminated intravascular coagulation and sepsis. It is exceedingly rare in the setting of pneumococcal bacteremia, particularly in immunocompetent individuals. CASE REPORT We report a generally healthy 67-year-old male with schizophrenia who refused pneumococcal vaccination. He had an intact and functional spleen with a functional immune system. The patient presented with fever and diarrhea. He subsequently progressed to develop purpura fulminans and septic shock due to S. pneumoniae bacteremia. Despite an extensive search for the primary source of infection, none could not be identified. Due to timely initiation of appropriate antibiotic therapy and aggressive supportive care in an intensive care unit, he recovered despite multi-organ failure that developed throughout his hospitalization. CONCLUSIONS We present a rare manifestation of a potentially preventable disease and emphasize the importance of pneumococcal vaccination in order to decrease the risk of developing invasive pneumococcal disease. Furthermore, we discuss etiology, diagnosis, differential diagnosis, and evidence-based management of purpura fulminans and invasive pneumococcal disease with a literature review. Purpura fulminans due to S. pneumoniae is exceedingly rare in immunocompetent patients and an unusual clinical manifestation of pneumococcal bacteremia.

Fulminant sepsis/meningitis due to Haemophilus influenzae in a protein C-deficient heterozygote treated with activated protein C therapy.

A 13-month-old Japanese female with Haemophilus influenzae type b meningitis presented with unusually severe septic shock and cerebral infarction in half a day of fever. The initial therapy of plasma-derived activated protein C (Anact C) led to an impressive effect on the aggressive condition. However, purpura fulminans and the consistent decline of plasma protein C activity (<20%) required prolonged activated protein C therapy and gene analysis. The patient carried a novel heterozygous mutation of PROC (exon 4; 335 GAC>TAC, Asp46Tyr). This is the first report of infectious purpura fulminans in a protein C-deficient heterozygote. The clinical onset and treatment course adequately corroborated the aggravated immune/hemostatic reactions and the cytoprotective effects of activated protein C replacement in human heterozygous protein C deficiency. The monitoring of plasma protein C activity and sufficient administration of activated protein C product could improve the outcome of severe sepsis in children.