Purpura fulminans in a hyposplenic patient arising from pneumococcal sinusitis.

We report a previously healthy woman in her 50s who presented with sepsis, rapidly progressive purpuric rash and disseminated intravascular coagulation. She was diagnosed with acute infective purpura fulminans due to invasive pneumococcal infection likely secondary to sinusitis. Our case report discusses our initial diagnostic uncertainty and approach in investigating and treating such a critically unwell patient.

[Pediatric infectious emergencies-from febrile seizure to purpura fulminans]. / Infektiologische Kindernotfälle ­ vom Fieberkrampf bis zur Purpura fulminans.

Febrile seizures, which are relatively common in young children, are often triggered by an infection and resolve quickly. Prompt presentation to a pediatric department is mandatory after any first seizure and every time for children ≤ 12 months. Central nervous system (CNS) diseases in childhood are able to cause seizures or other neurological disorders. Even the slightest suspicion of a seizure with CNS involvement must be promptly treated. In case of doubt, both an antiviral and an antibacterial treatment are started in parallel, which can be stopped after detecting the pathogen. Lumbar puncture is strictly indicated unless there are contraindications. Meningococcal sepsis is a severe clinical feature comprising high fever, chills and disorders of consciousness. The first skin symptoms are petechiae as a red flag sign. With progression, potentially lethal purpura fulminans may develop. Waterhouse-Friderichsen syndrome is a severe complication of acute bacterial meningitis. Lethality rate is 35%. The pediatric assessment triangle and the ABCDE algorithm help to identify critically ill children in a standardized, structured, and rapid manner.

Isolated Protein C Deficiency in a Newborn.

BACKGROUND: Congenital protein C deficiency is a rare hereditary thrombophilia, neonatal purpura fulminans is the most serious form of this deficit. The purpose of this observation is two-fold. The first is the need to make an early diagnosis in order to improve the prognosis. The second, is to discuss the need. In case of extensive purpura fulminans in the neonatal period, the search for a deficiency in anticoagulant factor, in particular the dosage of protein C, in the newborn and in both parents. METHODS: The diagnosis is biological and is based on the quantitative determination of functionally active protein C. We use the Berichrom® Protein C assay on an automated coagulation analyzer from Siemens Healthcare Diagnostics, which allows the chromogenic determination of Protein C activity. RESULTS: We report an observation of cutaneous necrosis in a newborn having developed a purpura fulminans extensive secondary to a total congenital protein C deficiency. In front of this clinical picture, thrombophilia assessment is requested, revealing an isolated deficit in protein C < 1%. CONCLUSIONS: In the case of extensive purpura fulminans in the neonatal period, the search for a deficiency in anticoagulant factor, in particular the dosage of protein C, is essential in the newborn and in both parents.

Use of hyperbaric oxygen therapy of purpura fulminans in an extremely low birth weight preterm: A case report.

Purpura fulminans (PF) is a rare and fatal complication of septic shock or diffuse intravascular coagulation (DIC) resulting in skin and soft tissue necrosis. PF can be caused by congenital or acquired protein C (PC) or protein S (PS) deficiency. The most common cause of PF in a neonate is sepsis. In our extremely low birth weight preterm case, due to PF that started in the right-hand fingers, examination was made and protein S deficiency was detected as well as MTHFR (A1298C) and Factor V Leiden (R506Q) homozygous mutations. While being unresponsive to fresh frozen plasma (FFP) and unfractionated heparin (UFH) therapy, we want to highlight the curative treatment with hyperbaric oxygen (HBOT), which has not previously been used in extremely low birth weight preterm infants for this purpose.

Lower Limb Necrosis Secondary to Purpura Fulminans: A Case Report.

Purpura fulminans is a rare and rapidly progressive septic process characterized by the development of hemorrhagic and ecchymotic lesions and skin necrosis. In this work, we report a case of a 52-year-old woman admitted to the Department of Emergency due to progressive purpura. The physical examination demonstrated a decreased skin temperature, unpalpable dorsalis pedis arteries, and ecchymoses covering both lower extremities. Laboratory tests indicated disseminated intravascular coagulation with prolonged activated partial thromboplastin time (APTT), low prothrombin time (PT), elevated d-dimer levels, and a low platelet count. A diagnosis of purpura fulminans was made, and steroids, therapeutic plasma exchange and empiric therapy, including antibiotic and anticoagulation therapy, were initiated immediately. Our treatment resulted in a good and sustained clinical response, as evidenced by the receding of blood blisters and the normalization of the patient's coagulation factors, but bilateral below-knee amputation was inevitable. Finally, the patient recovered well and was discharged home without any complications other than amputation.

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.

Meningococcaemia In Children-An Under Recognized Public Health Problem In Pakistan.

Background: Meningococcaemia is a serious bacterial disease caused by Neisseria meningitidis resulting in septicaemia and meningitis in previously well individuals and is associated with serious consequences including mortality. As the data from our region is scarce, the study was conducted with an aim to highlight the disease manifestations and outcomes so that appropriate interventions are devised. Method: A descriptive study was conducted in Paediatrics A Unit from 1st March 2020 to 30th September 2021 after approval of institutional review board. Children admitted with features of meningococcaemia in the form of fever and typical petechial purpuric rash were included in the study. Results: A total of 46 patients with meningococcaemia were included in the study. Of these, 24 (52.2%) were male. Majority of the patients 22 (47.8%) were in the age group of 1 year to five years, Major complications were meningitis in 39 (84.8%), septic shock in 26 (56.5%) and purpura fulminans in 12 (26.1%) patients. Mortality was documented in 6 (13%) patients. Outcome was associated with age. (p= 0.039). There was a significant difference in outcome in patients with prolonged PT/APTT (p=0.031), purpura fulminans (p=0.000) and septic shock (p=0.021). Conclusion: Meningococcaemia is prevalent in our region in paediatric population. The disease has a fulminant course with a myriad of complications and potentially fatal outcomes especially in children under one year of age.

An Atypical Case of Idiopathic Purpura Fulminans.

Idiopathic purpura fulminans (PF) is rare but has been reported in pediatric patients, commonly following infections. We present a case of a 5-year-old boy, heterozygous for factor V Leiden, with no history of recent infections, who presented with PF secondary to acquired protein S deficiency. Despite initial supportive treatment, the patient required surgical fasciotomy and extensive skin grafts. The protein S level normalized 4 months following the presentation. In this context, an autoimmune component with transient anti-protein S antibodies was believed to be involved. This case report highlights the course of idiopathic PF due to noninfectious acquired protein S deficiency.

Purpura fulminans due to Streptococcus pneumoniae bacteraemia in an unsplectomised immunocompetent adult without primary hypocomplementaemia.

Invasive pneumococcal disease occurs in high-risk patient population which includes patients with asplenia and primary hypocomplementaemia. Pneumococcal sepsis can rarely cause disseminated intravascular coagulation (DIC) and intravascular thrombosis of small and medium sized vessels called purpura fulminans which is associated with a high mortality rate. We present the case of an immunocompetent woman in her 50s with an intact spleen who presented with septic shock from Streptococcus pneumoniae bacteraemia. Her hospital course rapidly progressed to multiorgan dysfunction, DIC and purpura fulminans. She was treated aggressively with broad spectrum antibiotics, coagulation factor replacement, multiple vasopressor support, renal replacement therapy and mechanical ventilator support. Despite aggressive measures, she succumbed to the multiorgan failure.

Routine use of 16S rRNA PCR and subsequent sequencing from blood samples in septic shock: about two case reports of Capnocytophaga canimorsus infection in immunocompetent patients.

BACKGROUND: Capnocytophaga canimorsus infection happens frequently in immunosuppressed patients with reported domestic animal bites. Clinical presentation ranges from simple cellulitis to fulminant septic shock with disseminated intravascular coagulopathy, with an overall mortality of 30%. Conventional blood culture is often negative as this is a slow-growing pathogen. Nevertheless, the increasing use of 16S rRNA gene amplification and Sanger sequencing allows a much more rapid diagnostic confirmation. We present two case reports where 16S rRNA gene sequencing helped to diagnose Capnocytophaga canimorsus infection. CASE PRESENTATION: Case 1: A 53-year-old man with a history of non-cirrhotic chronic alcohol consumption was admitted to the intensive care unit (ICU) for septic shock and disseminated intravascular coagulopathy (DIC) of unknown origin. Blood cultures remained negative and a 16S rRNA PCR was performed leading to the identification of Capnocytophaga Canimorsus on day 4. Targeted antibiotic therapy with ceftriaxone for 14 days lead to overall recovery. Afterwards, the patient recalled a dog bite 2 days before hospitalization with a punctiform necrotic wound localized on a finger, which was not obvious at admission. Case 2: A 38-year-old man arrived to the emergency department for acute alcohol intoxication and history of a dog bite 2 days before. At admission, septic shock with purpura fulminans was diagnosed and required ICU hospitalization, invasive mechanical ventilation, vasopressor support and renal replacement therapy due to the rapid clinical deterioration. In the context of septic shock with purpura fulminans, DIC and recent dog bite, the diagnosis of Capnocytophaga canimorsus septic shock was suspected, and early confirmed by 16S rRNA PCR coupled to Sanger sequencing on day 2. Blood cultures became only positive for Capnocytophaga canimorsus 5 days after admission. Ceftriaxone alone was infused for 10 days in total, and the patient was discharged from the ICU on day 25. CONCLUSIONS: 16S rRNA gene PCR proves an important diagnostic tool when facing a sepsis of unknown origin. In these two cases of septic shock related to Capnocytophaga canimorsus, initial blood cultures remained negative at 24 h, whereas the diagnosis was achieved by 16S rRNA PCR sequencing performed from blood samples obtained at admission.

Prevalence and clinical features of secondary skin lesions in septic patients with bloodstream infections.

Cutaneous manifestations developed in the course of sepsis are poorly documented in the medical literature beyond those related to specific pathogens or classical clinical pictures such as purpura fulminans or ecthyma gangrenosum. The objective of this study was to determine the overall prevalence of sepsis-related skin findings and evaluate their possible impact on the prognosis of septic patients. Single-centre, retrospective study of septic patients with documented bloodstream infections admitted in a tertiary hospital during 2019. Primary skin and soft tissue infections, and non-sepsis-related skin conditions diagnosed during hospital admission were excluded. Unselected sample of 320 episodes of sepsis in 265 patients. Secondary skin lesions were documented in 57 sepsis episodes (17.8%) in 47 patients. Purpura (petechiae/ecchymosis) was the most frequent cutaneous finding in septic patients (35.5%), with non-acral involvement in more than one-third of the episodes (38.5%), followed by skin and soft tissue erythema/oedema (25.8%) and maculopapular rashes (11.3%). Secondary skin lesions occurred more frequently in sepsis of respiratory (p = 0.027) and skin and soft tissue (p = 0.018) origin, as well as in sepsis caused by Pseudomonas aeruginosa and Stenotrophomonas maltophilia (p = 0.001). Mean hospital stay was 38.58 days and sepsis-related mortality 21.1%. Our results suggest that cutaneous involvement in the course of sepsis is frequent, with purpura being the main clinical sign. The semiology described in this study, easily identifiable by non-dermatologists, should alert clinicians to the potential unfavourable course of these patients.

New-onset diabetic ketoacidosis with purpura fulminans in a child with COVID-19-related multisystem inflammatory syndrome.

BACKGROUND: Coronavirus disease 2019 (COVID 19) usually causes a mild illness among children. However, in a minority of children, it may be associated with the life-threatening multisystem inflammatory syndrome (MIS-C), or thrombotic microangiopathy, or sequelae like type-1 diabetes mellitus (T1DM). We describe a previously healthy, 12-year-old boy with new-onset T1DM with diabetic ketoacidosis (DKA) in the setting of MIS-C, with a course complicated by thrombotic microangiopathy. CASE PRESENTATION: The patient presented with four days history of fever, non-bilious vomiting, polyuria and polydipsia. On evaluation, he was noted to have diabetic ketoacidosis. Although Diabetic ketoacidosis with insulin and intravenous fluids, his hospital course was notable for shock requiring vasopressor, purpura fulminans with eschar formation, neurological manifestations (left hemiparesis due to right middle cerebral artery territory infarct, mononeuritis multiplex) and thrombotic microangiopathy. MIS-C-like illness secondary to COVID-19 was suspected due to diabetic ketoacidosis, thrombotic microangiopathy, elevated inflammatory markers, history of contact with COVID-19-infected individual and detectable COVID-19 IgG antibodies. He improved following management with methylprednisolone, intravenous immunoglobulin, low-molecular-weight heparin and aspirin, and was discharged on hospital day 48. CONCLUSION: MIS-C-like illness should be considered in children and adolescents presenting with complex multisystem involvement in this era of COVID 19. Management with immunomodulatory agents can be lifesaving.

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

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.

A rare association between group A Streptococcus purpura fulminans and diffuse alveolar hemorrhage in a young girl: A case report.

We report the case of a 7-year-old girl with septic shock and coagulopathy associated with purpura fulminans (PF) and diffuse alveolar hemorrhage (DAH) due to group A Streptococcus (GAS) infection identified with 16S ribosomal RNA analysis performed on the skin biopsy. GAS infection with PF associated with DAH is rare in healthy young children but pediatricians should be aware of this condition because of the poor prognosis. The initial treatment for circulatory failure and severe disseminated intravascular coagulation as well as the prompt initiation of antibiotic treatment may be crucial for the outcomes of S. pyogenes PF.

Reconstructing Severe Lower Extremity Skin Necrosis in a Pediatric Patient.

ABSTRACT: Purpura fulminans can result in significant full-thickness wounds, posing a challenge in the pediatric population, given the paucity of donor sites for reconstruction. The authors present the case of an 11-month-old patient for whom a split-thickness skin allograft (TheraSkin) was successfully implemented as a temporizing measure for a large leg wound.

Diffuse Intracerebral Hemorrhage in an Infant With a Novel Homozygous Variant Leading to Severe Protein C Deficiency.

Protein C is a circulating anticoagulant that inhibits factor Va and VIIIa and promotes fibrinolysis. Compound heterozygous or homozygous variants in the Protein C gene (PROC) lead to severe deficiency of protein C and affected neonates typically present shortly after birth with purpura fulminans. We describe an infant who suffered a diffuse intracranial hemorrhage as a neonate and presented with purpura fulminans as an older infant which led to investigations that were consistent with severe protein C deficiency. We demonstrate subacute findings on neuroimaging and suggest this condition should be considered with neonatal presentations of bilateral intraparenchymal hemorrhage.

Streptococcus pyogenes purpura fulminans and septic shock: A case highlighting the ethical considerations of high-risk extracorporeal membrane oxygenation.

Separately, refractory septic shock and purpura fulminans have very poor outcomes. The ethics involved in offering extracorporeal membrane oxygenation to very high-risk patients is complex. We report a novel case of refractory shock requiring veno-arterial extracorporeal membrane oxygenation and continuous renal replacement therapy due to Streptococcus pyogenes bacteremia with purpura fulminans to highlight the ethical challenges in offering extracorporeal membrane oxygenation to a patient with such a poor likelihood of survival.

[Inflammatory myopathy following acute meningoccemia in a properdin-deficient patient: A case report]. / Myopathie inflammatoire post-méningococcémie aiguë chez un patient présentant un déficit en properdine : à propos d'un cas.

INTRODUCTION: Myalgia is a classical sign in invasive meningococcal diseases (IMD), but severe and persistent myalgia following an IMD have never been reported to date. CASE REPORT: A 20-year-old man presented with purpura fulminans and meningitis caused by Neisseria meningitidis serogroup Y, revealing properdin deficiency. Although meningitis symptoms improved after antibiotherapy, initial myalgia of the lower limbs increased, associated with mild rhabdomyolysis. Magnetic resonance imaging (MRI) revealed an increased STIR (Short TI inversion recovery) signal of both quadriceps muscles, without abscess. After exclusion of other causes of myopathy, a post-infectious myositis was diagnosed. A four-week course of corticosteroids led to dramatic improvement. CONCLUSION: Post-infectious inflammatory myopathy should be suspected in case of severe and persistent myalgia associated with rhabdomyolysis following an IMD, after exclusion of pyomyositis especially. A short course of corticosteroids seems to be effective.

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.