The Osseous Pathology of Purpura Fulminans in a TwoYear-Old Child: A Case Report

@#Purpura fulminans (PF) is a severe clinical manifestation of Neisseria meningitides infection that is associated with high mortality rates in children. Survivors are frequently left with debilitating musculoskeletal sequelae. There is a paucity of reports on the musculoskeletal pathology of purpura fulminans. We report on a 2-year-old boy with purpura fulminans due to meningococcemia. The child developed distal gangrene in both the upper and lower limbs. Amputations were done for both lower limbs. Histological examination of the amputated specimens showed an inflammatory process and features of osteonecrosis. The latest follow-up at the age of 6 years showed a right knee valgus due to asymmetrical growth arrest of the proximal tibia. PF and its complications are challenging to treat and may require a multidisciplinary approach to improve patient’s functional ability.

Immunoadsorption therapy for a meningococcemia patient with myocarditis, adrenal hemorrhage, and purpura fulminans: a case report

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.

A rare case of varicella gangrenosum in adult

Varicella gangrenosum is a gangrenous ulceration of varicella lesions involving the skin and soft tissues of the body. It most commonly occurs in children less than 5 years of age and life threatening. This is a very rare complication of chicken pox in adults which deserves early diagnosis and management. 21-year-old male presented with blackish discoloration in the lateral aspect of right thigh for 5 days. He has positive history of chicken pox for his brother and sister following which he acquired it 15 days back. During that episode he had fever, headache and blisters which ruptured to heal by scab. But scab in right thigh coalesced to form the gangrenous area with serous discharge. On presentation he had no fever with local lesion and surrounding erythema. Patient underwent radical surgical debridement and regular dressing. Pus culture was sent which showed no growth. He gradually improved and the ulcer granulated well and split skin graft is done. Varicella gangrenosum is a life-threatening condition which can be either wet, moist or purpura fulminans. Patients who develop disseminated intravascular coagulation and have a grave prognosis. Surgical debridement is the only proven treatment which has led to better outcome. Only about 10 cases reported in literature so far regarding this condition.

Renal histological findings in a patient with acute renal injury associated with purpura fulminans: a case report / Achados histológicos renais em paciente com injúria renal aguda associada à purpura fulminans: relato de caso

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.

Clinical characteristics of rare Streptococcal pneumonia disease in children / 中华实用儿科临床杂志

The clinical manifestations of Streptococcus pneumoniae disease(SPD) are various, and some patients with rare SPD types have a worse prognosis.Among them, the mortality is up to 70% after 48-72 hours of the onset of Streptococcus pneumoniae (SP)-related overwhelming post-splenectomy infection(OPSI). Purpura fulminans of SP(SP-PF) is often characterized by extensive purpura, fever, hypotension and disseminated intravascular coagulation, and prognosis of the patients without prodromal symptoms is poor.Infectious endocarditis has a mortality rate of 20.7% in SP-related cardiovascular diseases, and Austrian syndrome has a fatality rate of 43.5%.The mortality rate of bacterial pericarditis was 13.56%.At present, SP vaccine can not completely cover the 7 serotypes reported.Vasculitis mainly occurs in the thoracic and abdominal aorta and its branches, middle cerebral artery and so on.Of the 8 cases of hemophagocytic syndrome, 2 cases died.Early anti-infection, methylprednisolone shock and surgical treatment can improve the prognosis.Vaccination and drug prophylaxis can help those with under-lying diseases such as splenic dysfunction to reduce the incidence of SPD, such as SP-OPSI.

Clinical characteristics of rare Streptococcal pneumonia disease in children / 中华实用儿科临床杂志

The clinical manifestations of Streptococcus pneumoniae disease (SPD) are various,and some patients with rare SPD types have a worse prognosis.Among them,the mortality is up to 70％ after 48-72 hours of the onset of Streptococcus pneumoniae (SP)-related overwhelming post-splenectomy infection (OPSI).Purpura fulminans of SP (SP-PF) is often characterized by extensive purpura,fever,hypotension and disseminated intravascular coagulation,and prognosis of the patients without prodromal symptoms is poor.Infectious endocarditis has a mortality rate of 20.7 ％ in SP-related cardiovascular diseases,and Austrian syndrome has a fatality rate of 43.5 ％.The mortality rate of bacterial pericarditis was 13.56％.At present,SP vaccine can not completely cover the 7 serotypes reported.Vasculitis mainly occurs in the thoracic and abdominal aorta and its branches,middle cerebral artery and so on.Of the 8 cases of hemophagocytic syndrome,2 cases died.Early anti-infection,methylprednisolone shock and surgical treatment can improve the prognosis.Vaccination and drug prophylaxis can help those with under-lying diseases such as splenic dysfunction to reduce the incidence of SPD,such as SP-OPSI.

Amputaciones múltiples secundarias a púrpura fulminans en un adulto joven: una perspectiva en rehabilitación / Multiple amputations secondary to purpura fulminans in a young adult: a rehabilitation perspective

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.

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.

Pigmented purpura and cutaneous vascular occlusion syndromes

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.

Púrpura en los pies: Meningococcemia? Reporte de caso clínico y revisión de la literatura / Purpura in the feet: Meningococcemia? Clinical case report and literature review

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.

Purpura fulminans related to paroxysmal nocturnal haemoglobinuria: a case report and literatures review / 中华血液学杂志

Objective@#To improve the understanding of the rare clinical presentation and management of purpura fulminans (PF) in patients with paroxysmal nocturnal haemoglobinuria (PNH).@*Methods@#A case of PF occurring in PNH is reported, while the related literature review is conducted.@*Results@#A 49-year-old male patient suffered from one-week history of fever, greenish-brown colour urine, multiple well demarcated and painful purpura of the head and neck. He had been reported to have two thromboembolic events during the 22-year course of PNH. Skin biopsy displayed classic PF features. Laboratory testing showed a high PNH clone, intravascular hemolysis and coagulation system changes. After sufficient anticoagulation and short course of glucocorticoid therapy, the clinical conditions were improved correspondingly. During a follow-up period of 6 month, there was no recurrence of thrombosis.@*Conclusion@#PF should be considered in PNH patients with unexplained, quickly developed painful purpura. Extensive work-up should be performed to find out other potential thrombophilic risk factors after diagnosis of PF. Early diagnosis, adequate anticoagulation therapy and control hemolysis were essential to PF treatment occurring in PNH. The survival of patients and the qualities of life can be improved. The PNH clone detection is needed to evaluate the status of procoagulation and predict the risk of recurrent thrombosis.

Tratamiento de la púrpura fulminans neonatal. Reporte de caso y revisión de la literatura / Treatment for neonatal fulminans purpura. Case report and literature review

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.

Cerebral Palsy due to Intracranial Hemorrhage Caused by Consumptive Coagulopathy in Protein C Deficiency: A Case Report

Protein C (PROC) is a potent anticoagulant inactivating coagulation factors Va and VIIIa. PROC deficiency is very rare condition inherited as an autosomal dominant or recessive trait, and associated with various thromboembolic and ischemic conditions. Moreover, severe form of PROC deficiency can cause fatal hemorrhagic complications due to consumptive coagulopathy. We reported two children with hemorrhagic stroke who were diagnosed as severe PROC deficiency caused by two different types of compound heterozygous PROC gene mutations. We described results of laboratory tests, genetic analysis, brain magnetic resonance images, and functional outcomes. Both children received prophylactic anticoagulation therapy and presented with purple-colored skin lesions during rehabilitation. Purpura fulminans caused by insufficient anticoagulation should be differentiated from hematoma caused by excessive anticoagulation therapy in these children.

Cerebral Palsy due to Intracranial Hemorrhage Caused by Consumptive Coagulopathy in Protein C Deficiency: A Case Report

Protein C (PROC) is a potent anticoagulant inactivating coagulation factors Va and VIIIa. PROC deficiency is very rare condition inherited as an autosomal dominant or recessive trait, and associated with various thromboembolic and ischemic conditions. Moreover, severe form of PROC deficiency can cause fatal hemorrhagic complications due to consumptive coagulopathy. We reported two children with hemorrhagic stroke who were diagnosed as severe PROC deficiency caused by two different types of compound heterozygous PROC gene mutations. We described results of laboratory tests, genetic analysis, brain magnetic resonance images, and functional outcomes. Both children received prophylactic anticoagulation therapy and presented with purple-colored skin lesions during rehabilitation. Purpura fulminans caused by insufficient anticoagulation should be differentiated from hematoma caused by excessive anticoagulation therapy in these children.

Deficiencia congénita de proteína C en un recién nacido con trombosis y necrosis de tejidos extensa / Congenital C protein deficiency in a newborn with extensive thrombosis and necrosis of tissues

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)

Bilateral Torsion of Testes with Purpura Fulminans.

Background: Purpura fulminans and bilateral perinatal testicular torsion are rare and may co-exist. Case characteristics: A 3-day-old neonate with bilateral swelling of scrotum; torsion and gangrenous changes were observed on exploration. Interventions: Left orchidectomy with preservation of right testis was done. Outcome: At 2-month follow-up, right testis showed signs of atrophy. Child developed full thickness skin lesions and died of sepsis. Message: Perinatal testicular torsion can be bilateral, and requires urgent surgical exploration.

Overwhelming Pneumococcal Sepsis in a Patient with Splenic Hypoplasia / General Medicine

A 58-year-old female was admitted due to severe sepsis and multi-organ failure with a fulminant purpuric rash. Meropenem, vancomycin and levofloxacin were administered, although no focus of infection was detected. However, computed tomography revealed a profoundly hypoplastic spleen, and a blood smear detected Howell-Jolly bodies. Blood cultures grew <i>Streptococcus pneumoniae</i> (serotype 22F) three hours after admission. The patient was finally diagnosed as overwhelming pneumococcal sepsis with hyposplenism precipitated by splenic hypoplasia. Clinicians should pay attention to the splenic size and Howell-Jolly bodies in cases of sepsis of unknown origin.

Staphylococcal Bacteremia and Purpura Fulminans.

Purpura fulminans (PF) is a life threatening disorder of acute onset characterized by cutaneous hemorrhage and necrosis caused by DIC and dermal vascular thrombosis. It is commonly associated with meningococcemia or invasive streptococcal disease. In this article we report a case of purpura fulminans in the setting of staphylococcus aureus infection.

Spectrum of purpura fulminans: Report of three classical prototypes and review of management strategies.

Purpura fulminans is a rare syndrome of intravascular thrombosis and hemorrhagic infarction of the skin that is rapidly progressive and is accompanied by vascular collapse and disseminated intravascular coagulation. It usually occurs in children, but this syndrome has also been noted in adults. The three forms of this disease are classified by the triggering mechanisms. We describe three classical cases of purpura fulminans of the three classical prototypes treated at our center and their varied clinical outcomes. We also describe a case of acute infectious purpura fulminans secondary to systemic leptospirosis which to our best knowledge is the first reported case in world literature. The various treatment options for purpura fulminans have also been reviewed.