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La púrpura fulminante adquirida postinfecciosa es una entidad aguda y grave, poco frecuente, caracterizada por necrosis cutánea asociada a coagulopatía intravascular diseminada (CID), en ausencia de infección activa o alteraciones previas de la coagulación. Afecta fundamentalmente a la población pediátrica y, en el 90 % de los casos, está precedida por un proceso infeccioso. El mecanismo fisiopatológico es un déficit transitorio de proteína S mediado por autoanticuerpos que favorece un estado de hipercoagulabilidad. Se presenta el caso de un varón de 8 años previamente sano, con lesiones cutáneas purpúricas características de púrpura fulminante asociada a CID en ausencia de sepsis. Se constató deficiencia plasmática transitoria de proteína S. Requirió tratamiento sustitutivo con plasma fresco congelado y anticoagulación; la evolución fue favorable. La actividad de la proteína S permaneció disminuida durante 2 meses.

Acquired postinfectious purpura fulminans is a rare, acute, and severe disease characterized by skin necrosis associated with disseminated intravascular coagulation (DIC) in the absence of active infection or previous coagulation disorders. It mainly affects the pediatric population and, in 90% of cases, it is preceded by an infectious process. The pathophysiological mechanism is a transient autoantibodymediated protein S deficiency that favors a hypercoagulable state. Here we describe the case of a previously healthy 8-year-old boy with purpuric skin lesions typical of purpura fulminans associated with DIC in the absence of sepsis. A transient plasma protein S deficiency was confirmed. He required replacement therapy with fresh frozen plasma and anticoagulation; he had a favorable course. Protein S activity remained decreased for 2 months.

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Acquired postinfectious purpura fulminans is a rare, acute, and severe disease characterized by skin necrosis associated with disseminated intravascular coagulation (DIC) in the absence of active infection or previous coagulation disorders. It mainly affects the pediatric population and, in 90% of cases, it is preceded by an infectious process. The pathophysiological mechanism is a transient autoantibody-mediated protein S deficiency that favors a hypercoagulable state. Here we describe the case of a previously healthy 8-year-old boy with purpuric skin lesions typical of purpura fulminans associated with DIC in the absence of sepsis. A transient plasma protein S deficiency was confirmed. He required replacement therapy with fresh frozen plasma and anticoagulation; he had a favorable course. Protein S activity remained decreased for 2 months.

La púrpura fulminante adquirida postinfecciosa es una entidad aguda y grave, poco frecuente, caracterizada por necrosis cutánea asociada a coagulopatía intravascular diseminada (CID), en ausencia de infección activa o alteraciones previas de la coagulación. Afecta fundamentalmente a la población pediátrica y, en el 90 % de los casos, está precedida por un proceso infeccioso. El mecanismo fisiopatológico es un déficit transitorio de proteína S mediado por autoanticuerpos que favorece un estado de hipercoagulabilidad. Se presenta el caso de un varón de 8 años previamente sano, con lesiones cutáneas purpúricas características de púrpura fulminante asociada a CID en ausencia de sepsis. Se constató deficiencia plasmática transitoria de proteína S. Requirió tratamiento sustitutivo con plasma fresco congelado y anticoagulación; la evolución fue favorable. La actividad de la proteína S permaneció disminuida durante 2 meses.

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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 rapidly evolving syndrome of cutaneous microvascular thrombosis and hemorrhagic necrosis. We present the case of a male patient, hospitalized for an infectious pathology and an acute cardiovascular event, who developed purpura fulminans due to protein C deficiency, related to a concomitant infectious condition.

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Abstract 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. A ten-year-old female patient developed a purpuric rash with fever. Upon arrival to the pediatric intensive care department, she was unconscious and in a poor general condition. We combined treatment with antibiotics, volume resuscitation, hydrocortisone, and CytoSorb® therapy resulted in a stabilization of hemodynamics, as well as control of hyperinflammation. We observed a significant decrease in vasopressor dosage in this patient.

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Purpura fulminans is a life-threatening disease, characterized by disseminated intravascular coagulation and endovascular thrombosis; can often occur secondary to heterogeneous etiologies, such as sepsis, and to a lesser extent, secondary to sepsis due to halophilic bacteria, such as V. vulnificus, found in marine environments. Patients with specific comorbidities are at the highest risk of worst scenarios, without prompt treatment, infection can rapidly evolve to fatal, with a mortality rate close to 100 %. We present a case of Purpura fulminans due to V. vulnificus septicemia.

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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. A ten-year-old female patient developed a purpuric rash with fever. Upon arrival to the pediatric intensive care department, she was unconscious and in a poor general condition. We combined treatment with antibiotics, volume resuscitation, hydrocortisone, and CytoSorb.½ therapy resulted in a stabilization of hemodynamics, as well as control of hyperinflammation. We observed a significant decrease in vasopressor dosage in this patient.

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Hump-nosed pit vipers of the genus Hypnale are highly venomous and reputed for the commonest venomous snakebites in Sri Lanka. They frequently cause local manifestations and less commonly cause systemic effects such as acute kidney injury and coagulopathy. There is no antivenom currently available in Sri Lanka for their envenoming. However, more and more complications of Hypnale bites are being recently described. Purpura fulminans, one of the rare complications of snakebites that we report following authentic Hypnale hypnale bite. A 58-year-old female was bitten by a hump-nosed viper and developed bilateral toe gangrenes, ultimately ended up with amputations. She got recovered with loss of toes in both feet for 46 days treatment at hospital.

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OBJECTIVE: To clarify the incidence and genetic risk of neonatal-thromboembolism, we conducted a nationwide study exploring the impact of thrombophilia on neonatal-thromboembolism in Japan. STUDY DESIGN: A questionnaire survey was conducted for perinatal centers in Japan, focusing on the clinical expression, genotype, treatment, and outcome of patients who developed thromboembolism within 28 days of birth from 2014 to 2018. RESULTS: The estimated incidence of neonatal-thromboembolism was 0.39 cases per 10 000 live births. Intracranial lesions and purpura fulminans occurred in 66 and 5 of 77 patients, respectively. Fifty-eight (75.3%) infants presented within 3 days after birth. Four (5.2%) died, and 14 (18.2%) survived with disability. At the diagnosis, <20% plasma activity of protein C was noted in 16 infants, protein S (in 2), and antithrombin (in 1). Thirteen genetic tests identified 4 biallelic and 5 monoallelic protein C-variants but no protein S- or antithrombin-variants. Protein C-variants had purpura fulminans (P < .01), ocular bleeding (P < .01), positive-family history (P = .01), and death or disability (P = .03) more frequently than others. Protein C-variants were independently associated with disability (OR 5.74, 95% CI 1.16-28.4, P = .03) but not death. Four biallelic variants had serious thrombotic complications of neurologic disability, blindness, and/or amputation. Three monoallelic variants survived without complications. The only protein C-variant death was an extremely preterm heterozygote infant. CONCLUSIONS: Monoallelic protein C-variants had a higher incidence of neonatal-thromboembolism than biallelic variants. Thrombophilia genetic testing should be performed in the setting of neonatal-thromboembolism and low protein C to identify the underlying genetic defect.

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ABSTRACT Introduction: Purpura fulminans (PF) is a rapid progressive thrombotic disease in which hemorrhagic infarction of the skin and disseminated intravascular coagulation (DIC) occurs. It can potentially cause acute kidney injury (AKI). However, there is no description in the medical literature of renal histological findings of PF. Case report: A 20-year-old female patient, previously healthy, was admitted to the emergency department (ED) with odynophagia, fever, generalized myalgia and anuria, which evolved with the appearance of purpuric plaques on the face and limbs. She required dialysis on admission. Laboratorial tests showed anemia, leukocytosis, thrombocytopenia, and elevation of lactic dehydrogenase (LDH). The purpuric lesions became bullous with ruptures and then necrotic and erosive, reaching the dermis, subcutaneous tissue and musculature, until bone exposure. There was no improvement with initial antibiotic therapy aimed at the treatment of meningococcemia. Thrombotic microangiopathy (TMA) and PF were then suspected. The patient remained in daily dialysis, requiring plasmapheresis. After sustained improvement of the thrombocytopenia, she underwent renal biopsy, which was not compatible with TMA, characterizing possible PF. A complete recovery of the renal function was achieved and cutaneous sequels were treated with grafts. Conclusion: When thrombotic and hemorrhagic phenomena overlap, obtaining a renal biopsy can be difficult. However, in the presented case, the biopsy allowed the exclusion of AKI caused by TMA, presenting for the first time, histological findings compatible with PF.

RESUMO Introdução: Purpura Fulminans (PF) é uma doença trombótica de rápida progressão, com infarto hemorrágico da pele e coagulação intravascular disseminada (CIVD). É potencialmente causadora de injúria renal aguda (IRA). Porém, não há descrição na literatura médica dos achados histológicos renais causados por PF. Relato de caso: Mulher, 20 anos, previamente hígida, hospitalizada por odinofagia, febre, mialgia generalizada e anúria, evoluiu com aparecimento de placas purpúricas em face e membros. Necessitou de hemodiálise (HD) já na admissão. Exames laboratoriais mostravam anemia, leucocitose, plaquetopenia e elevação de desidrogenase lática. As lesões purpúricas tornaram-se bolhosas com rompimento e progressão para necrose, se aprofundaram, atingindo derme, subcutâneo e musculatura, até a exposição óssea. Não houve melhora com antibioticoterapia inicial voltada para tratamento de meningococemia. Suspeitou-se, então, de microangiopatia trombótica (MAT) e PF. A paciente permaneceu em HD diária e necessitou também de plasmaférese, após melhora sustentada da plaquetopenia, foi submetida à biópsia renal, que não foi compatível com MAT, possivelmente caracterizando PF. Houve recuperação completa da função renal e as sequelas cutâneas foram tratadas com enxerto. Conclusão: Em casos nos quais os fenômenos trombóticos e hemorrágicos se sobrepõem, a obtenção da biópsia renal se torna difícil. Neste caso, a biópsia permitiu excluir IRA causada por MAT e mostrar, pela primeira vez, achados compatíveis com PF.

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La infección por Neisseria meningitidis es causa frecuente de meningitis y septicemia en personas con factores de riesgo; presenta graves complicaciones entre las que se encuentra la púrpura fulminans; patología poco frecuente pero devastadora que inicia con la aparición de lesiones purpúricas en la piel y que finalmente produce compromiso vascular importante, ocasionando necrosis de tejidos profundos, lo cual lleva a amputaciones. Se reporta el caso de un adulto joven, masculino, miembro de las fuerzas militares, en quien se confirma infección por meningococo; presenta meningococcemia sin meningitis, desarrolla púrpura fulminans y finalmente, después de estabilización hemodinámica, requiere amputaciones múltiples secundarias: transtibiales bilaterales y en falanges de mano izquierda. Posterior al egreso de la hospitalización inicia proceso de rehabilitación y 9 meses después logra deambulación con prótesis en miembros inferiores.

Neisseria meningitidis infection is a frequent cause of meningitis and septicemia in people with risk factors; it presents serious complications including purpura fulminans, a rare but devastating pathology that starts with the appearance of purpuric lesions on the skin and finally produces important vascular compromise, causing necrosis of deep tissues, which leads to amputations. We report the case of a young adult male, member of the military forces, in whom meningococcal infection is confirmed; he presents meningococcemia without meningitis, develops purpura fulminans and finally, after hemodynamic stabilization, requires multiple secondary amputations: bilateral transtibial and left hand phalanges. After discharge from the hospital, she started rehabilitation and 9 months later she was able to ambulate with prosthesis in the lower limbs.

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Abstract: Purpura is defined as a visible hemorrhage in the skin or mucosa, which is not evanescent upon pressure. Proper classification allows a better patient approach due to its multiple diagnoses. Purpuras can be categorized by size, morphology, and other characteristics. The course varies according to the etiology, as do the diagnostic approach and treatment. This review discusses pigmented purpuras and some cutaneous vascular occlusion syndromes.

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Neisseria meningitidis es la etiología de infecciones severas incluso letales, afectando principalmente a la población joven. No obstante, dado que el único reservorio es la nasofaringe humana, hay portadores asintomáticos. El meningococo es sensible a los antibióticos de uso habitual, como: cefalosporinas de tercera generación y quinolonas, pero el retraso en el diagnóstico por presentaciones clínicas infrecuentes y la administración tardía de la terapia, se asocian a elevada morbimortalidad. Presentamos una paciente de 18 años, con un cuadro de rápida evolución, con parámetros inflamatorios de laboratorio alterados, asociado a lesiones cutáneas purpúricas, que evolucionó con falla multiorgánica y desenlace fatal, lográndose la confirmación etiológica por el Instituto de Salud Pública. Reportamos el caso para tener en consideración el diagnóstico de meningococcemia, frente a pacientes con cuadro clínico y exámenes de laboratorio sugerentes de sepsis, que presentan lesiones cutáneas compatibles.

Neisseria meningitidis is the etiology of severe, even lethal infections, affecting mainly the young population. However, since the only reservoir is the human nasopharynx, there are asymptomatic carriers. Meningococcus is sensitive to commonly used antibiotics such as third generation cephalosporins and quinolones, but delayed diagnosis due to infrequent clinical presentation and delayed therapy are associated with high morbidity and mortality. We present an 18-year-old female patient with a rapid evolution, with altered inflammatory laboratory parameters, associated with purpuric skin lesions, which evolved with multiorgan failure and fatal outcome, and the etiological confirmation was obtained by the Public Health Institute. We report the case to take into account the diagnosis of meningococcemia in patients with clinical symptoms and laboratory tests suggestive of sepsis and compatible skin lesions.

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Resumen La púrpura fulminans (PF) neonatal es un estado de hipercoagulabilidad poco frecuente pero grave. Su presentación clínica es súbita con lesiones purpúricas-necróticas que pueden dejar secuelas permanentes o incluso tener una evolución fatal. Se caracteriza por trombosis en la microcirculación de la piel acompañada de hemorragia perivascular. Los sitios más afectados son las extremidades pélvicas, torácicas o las zonas de presión. La alteración funcional más común es el defecto de la proteína с que fisiológicamente regula la coagulación, el defecto puede ser de causa primaria o secundaria. Caso clínico: Recién nacido varón con 8 días de vida extrauterina que presenta súbitamente rechazo a la vía oral, irritabilidad y fiebre de 39 °С. Dos días después es hospitalizado por deshidratación y rechazo a la vía oral. Al ingreso no se documentó fiebre o foco infeccioso. A las 24 horas presentó lesiones purpúricas-necróticas en el pie derecho. Se realizó un ultrasonido Doppler que confirmó trombosis venosa y arterial. Los dímeros Deran positivos. Se dio tratamiento con plasma fresco congelado (PFC), anticoagulante y antiplaquetario con buena respuesta. Conclusión: La PF es un estado protrombótico grave que requiere un diagnóstico y tratamiento oportunos para mejorar el pronostico.

Abstract Neonatal Purpura Fulminans (PF) is an infrequent hypercoagulable state but very severe. Its clinical manifestation is sudden with purpuric-necrotic injuries. It can leave permanent sequels oreven have a fatal evolution. It is characterized by thrombosis in the skin's microcirculation, accompanied with perivascular hemorrhage. The most affected areas are the pelvic and thoracic limbs, and pressure zones. The most common molecular alteration is the protein с defect, which physiologically regulates coagulation; the defect can be of a primary or secondary cause. Case report: A male newborn with 8 days of extrauterine life suddenly presents oral rejection, irritability and a 39° с fever. Two days later, he was hospitalized for dehydration and oral rejection. He didn't show signs of fever or infection at the time of his admission. Twenty-four hours after his entry, he presented purpuric-necrotic injuries in the right foot, hence, he was diagnosed with purpura fulminans. D-dimer studies and doppler ultrasound were taken. They confirmed venous and arterial thrombosis. The treatment was initiated with fresh frozen plasma, an anticoagulant and an antiplatelet, with a good response. Conclusion: PF is a serious hypercoagulable state that requires an early diagnosis and therapy to improve the outcome.

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Uno de los trastornos hematológicos más graves del período neonatal es la deficiencia congénita de proteína C, de presentación muy rara, y causa de enfermedad tromboembólica severa y púrpura fulminante en recién nacidos. Se puede sintetizar como una entidad clínico-patológica, de aparición aguda, con trombosis de la vasculatura de la dermis, lo cual conduce a necrosis hemorrágica y progresiva de la piel, asociada a coagulación intravascular diseminada y hemorragia perivascular, que ocurre en el período neonatal. El paciente presentado exhibe los elementos clínico-patológicos que caracterizan la púrpura fulminante, cuyo origen se debe a una deficiencia hereditaria de proteína C, lo cual condujo a la aparición de complicaciones trombóticas severas(AU)

One of the most serious hematological disorders of the neonatal period is congenital C protein deficiency of very rare occurrence and the main cause of severe thromboembolic disease and purpura fulminans in newborns. It may be summarized as a clinical and pathological entity of acute occurrence, with dermis vasculature thrombosis that leads to progressive hemorrhagic necrosis of the skin, associated to disseminate intravascular coagulation and perivascular hemorrhage in the neonatal period. The patient of this report showed the clinical and pathological elements characterizing purpura fulminans the origin of which is due to hereditary C protein deficiency that led to onset of severe thrombotic complications in this patient(AU)

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Uno de los trastornos hematológicos más graves del período neonatal es la deficiencia congénita de proteína C, de presentación muy rara, y causa de enfermedad tromboembólica severa y púrpura fulminante en recién nacidos. Se puede sintetizar como una entidad clínico-patológica, de aparición aguda, con trombosis de la vasculatura de la dermis, lo cual conduce a necrosis hemorrágica y progresiva de la piel, asociada a coagulación intravascular diseminada y hemorragia perivascular, que ocurre en el período neonatal. El paciente presentado exhibe los elementos clínico-patológicos que caracterizan la púrpura fulminante, cuyo origen se debe a una deficiencia hereditaria de proteína C, lo cual condujo a la aparición de complicaciones trombóticas severas(AU)

One of the most serious hematological disorders of the neonatal period is congenital C protein deficiency of very rare occurrence and the main cause of severe thromboembolic disease and purpura fulminans in newborns. It may be summarized as a clinical and pathological entity of acute occurrence, with dermis vasculature thrombosis that leads to progressive hemorrhagic necrosis of the skin, associated to disseminate intravascular coagulation and perivascular hemorrhage in the neonatal period. The patient of this report showed the clinical and pathological elements characterizing purpura fulminans the origin of which is due to hereditary C protein deficiency that led to onset of severe thrombotic complications in this patient(AU)

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Background: Purpura fulminans is a condition characterized by rapidly evolving skin necrosis and disseminated intravascular coagulation. Early recognition and aggressive supportive management has led to a decrease in its mortality rate, but most of these patients must undergo extensive soft tissue debridement and partial or total limb amputation. There is controversial evidence about the timing of surgery, suggesting that some patients may benefit from delayed debridement with limb preservation. Methods: We present a case of an 86-year-old patient who developed skin necrosis of his four limbs after infectious purpura fulminans. He was treated in the ICU with supportive measures and antibiotic treatment. Surgical debridement was delayed for 4 weeks until necrosis delimitation. Results: Only upper extremity debridement was necessary. Four fingers, including one thumb, were salvaged and successfully treated with semi-occlusive dressing without complications. Conclusion: Early recognition of infectious PF and timely supportive management are important pillars of its treatment. Delayed surgical debridement allows for less aggressive resection and good functional outcome.

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Absent or defective splenic function is associated with a high risk of fulminant bacterial infections, especially due to encapsulated bacteria. Not knowing this condition may delay medical treatment. Streptococcus pneumoniae is the leading cause of sepsis in these patients. Asplenic patients are at high risk for septic shock and eventually purpura fulminans, a life-threatening condition. We report the case of a 3 years oíd girl, with mitral stenosis and recurrent pneumonía that was admitted due to fever but in the next few hours presented hypotension, purpura and livedo reticularis. Laboratory test showed leucopenia (3.400/mm³), bandemia (43 percent of immature forms), thrombocytopenia, hypoprothombinemia and severe lactic acidosis (ph: 7.0 and lactic acid 11 mmol/1). The patient developed septic shock and multiorganic failure. Mechanical ventilation, volume resuscitation, vasoactive drugs and antibiotic therapy was initiated. Ultrasound was performed on the second day, demostrating asplenia. Peripheral blood smear showed Howell-Jolly bodies. Patient had a positive blood culture for penicillin-resistant Streptococcus pneumoniae (serotype 19F). Patient died of intracerebral hemorrhage after 8 days of admission. Necropsy confirmed asplenia and bilateral suprarenal haemorrhage. Absence of spleen can lead to life threatening infections, it is important to recognize it because vaccination and antibiotic prophylaxis can provide life-saving protection. This case provides a reminder to pursue asplenia as a potential underlying mechanism for invasive bacterial infection in children.

La condición de asplenia predispone a infecciones invasoras por bacterias capsuladas. Desconocer previamente ese antecedente dificulta y retarda el tratamiento médico. Streptococcus pneumoniae es el agente habitualmente causal de sepsis en estos pacientes. Los individuos asplénicos son particularmente proclives a evolucionar con shock séptico y eventual-mente al desarrollo de purpura fulminans, entidad altamente letal. Comunicamos el caso de una paciente con 3 años de edad y antecedente de cardiopatía y neumonías a repetición. Ingresó con compromiso sensorial, febril, hipotensa, con púrpura y livedo reticularis. En los exámenes de laboratorio destacaba la presencia de leucopenia (3.400/ mm³) trombopenia e hipoprotrombinemia (39 por ciento). Se inició ventilación mecánica, reanimación con volumen, fármacos vasoactivos y antibioterapia con vancomicina, clindamicina y ceftriaxona. Evolucionó con shock séptico refractario y síndrome de disfunción orgánica múltiple. Al segundo día de evolución una ecograña de abdomen comprobó la ausencia de bazo. En el frotis sanguíneo se evidenciaron corpúsculos de Howell-Jolly. Hemocul-tivo (+) S. pneumoniae resistente a penicilina (serotipo 19F). Un infarto hemorrágico cerebral ocasionó su deceso al octavo día. El estudio necrópsico corroboró la asplenia y evidenció necrohemorragia suprarrenal bilateral. La sepsis en un paciente asplénico puede ser de alguna forma prevenible mediante profilaxis antimicrobiana y vacunación neumocóccica. Dado los antecedentes de la paciente la búsqueda de asplenia era fundamental.

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La púrpura fulminans (PF) es una enfermedad cutánea purpúrica aguda asociada a un síndrome de coagulación intravascular diseminada (CID). Sus causas conocidas incluyen infecciones virales y bacterianas así como trombofilias. Es sabido que durante el embarazo existen alteraciones en los mecanismos hemostáticos; sin embargo, no se ha demostrado que estos fenómenos por sí solos ayuden al desarrollo de la PF.Se describe el caso de una mujer de 22 años, quien tuvo PF en circunstancias inusuales tales como el desarrollo de su cuadro durante el embarazo y el origen probable en una infección por E. coli. Se presentan los hallazgos clínicos, las intervenciones médicas y quirúrgicas y el desenlace. La paciente sobrevivió pero hubo necesidad de amputarle las falanges distales de tres artejos del pie izquierdo.

PF is a serious cutaneous purpuric disease, associated with an intravascular disseminated coagulation syndrome. Among its known causes, besides those of thrombophilia, are viral and bacterial infections, mostly Neisseria meningitidis. During pregnancy there are alterations in the hemostatic mechanism. However, by themselves, they have not been shown to lead to PF. The case of a 22-year-old woman who developed PF during pregnancy, presumably due to her Escherichia coli urinary tract infection, is presented including clinical, paraclinical, therapeutic and surgical aspects. She recovered but suffered amputation of the distant phalanges of three toes in the left foot

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