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.

[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.

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.

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.

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.

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.

Rare presentation of rickettsial infection as purpura fulminans: a case report.

BACKGROUND: Purpura fulminans is an acute life-threatening disorder characterized by intravascular thrombosis and hemorrhagic infarction of the skin complicated with disseminated intravascular coagulation. It is commonly seen in acute infections following meningococcal and streptococcal infections. Few cases of purpura fulminans following rickettsial infections have been described in the literature. CASE PRESENTATION: We report a case of a 55-year-old Sri Lankan woman who presented to Teaching Hospital Peradeniya with a febrile illness, headache, and myalgia that progressed to an erythematous rash starting over the bilateral lover limbs and hands and that became black and necrotic with a few hemorrhagic blebs. She had normocytic anemia, platelet clumps, and monocytosis as well as a deranged clotting profile. The result of immunofluorescence antibody testing for rickettsial immunoglobulin G was strongly positive for Rickettsia conorii with a rise in titer convalescent sera, and a diagnosis of purpura fulminans following rickettsial infection was made. The patient made an excellent recovery with chloramphenicol treatment. CONCLUSIONS: The treating physician should consider the rare but very treatable condition of rickettsial infection as a differential diagnosis in the etiological diagnostic workup of patients presenting with severe purpuric and hemorrhagic rash with fever.

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.

Purpura Fulminans: A rare presentation of Streptococcus Pneumoniae infection.

A previously healthy man presented with fever for 2 days and rapidly progressive purpuric rash for 1 day. He progressed into hypotension, disseminated intravascular coagulation and refractory shock despite resuscitation and early antibiotic commencement. Blood culture grew Streptococcus pneumoniae This case report highlights the fact that purpura fulminans can be a rare presentation of S. pneumoniae infection as well.