[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.

Management of purpura fulminans skin loss at a regional burns centre: Sixteen-year experience.

INTRODUCTION: Purpura fulminans (PF) is a rare syndrome of cutaneous purpura which is the consequence of severe circulatory shock causing intravascular thrombosis, haemorrhagic necrosis, and consequent tissue loss. The aim of this study was to present our 16-year experience of managing PF in a regional burns centre. METHODS: We performed a single-centre retrospective case series of all patients admitted to the St Andrews Burns Centre at Broomfield Hospital, Chelmsford, Essex, UK, between June 2006 and July 2022 with a diagnosis of PF. Data were extracted by retrospectively searching hospital case notes. RESULTS: Thirteen individuals were identified [five children (mean age 5, range 1-14) and eight adults (mean age 39, range 24-54)]. The total body surface area of cutaneous necrosis ranged from 5% to 80%, with a mean of 27.2%. Patients were treated with an established surgical sequence of total wound debridement and immediate coverage with a cadaveric allograft, followed by staged wound autografting. The mean time from disease onset to wound autografting was 37.3 days (range 20-64 days). Eight individuals (61.6%) required major amputation of at least one limb (proximal to the ankle or wrist joint). Only one mortality (of 80% total body surface area skin loss) was observed in the identified cohort. CONCLUSIONS: The large body surface areas often involved in PF cases make management of these wounds well suited for burns centres, wherein established facilities and multidisciplinary teams exist that are familiar with managing large cutaneous burns. We provide a suggested algorithm to aid the management of PF.

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.

Two Cases of COVID-19 Patients With Associated Purpura Fulminans.

Purpura fulminans (PF) is a life-threatening emergency involving coagulopathy and widespread skin necrosis. Early treatment, especially surgical management, is imperative as prognosis can be very poor. PF is most commonly associated with severe bacterial illness; however, viral causes are also possible. Currently in the literature, there have only been a handful of PF cases associated with COVID-19. We present two cases of PF in the setting of COVID-19 infection. Both patients had a history of underlying coagulopathies. PF can be a sign of underlying coagulopathy in a COVID-19 patient, who is already at increased risk for thromboembolic events due to the inflammatory nature of COVID itself. Due to how quickly PF can develop into life-threatening necrosis and multiorgan failure, it is imperative that these patients are referred early to a burn center for more advanced care.

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.

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.

Idiopathic purpura fulminans associated with anti-protein S antibodies in children: a multicenter case series and systematic review.

Idiopathic purpura fulminans (IPF) is a rare but severe prothrombotic coagulation disorder that can occur after chickenpox or human herpesvirus 6 (HHV-6) infection. IPF leads to an autoantibody-mediated decrease in the plasma concentration of protein S. We conducted a retrospective multicenter study involving patients with IPF from 13 French pediatric centers and a systematic review of cases in published literature. Eighteen patients were included in our case series, and 34 patients were included as literature review cases. The median age was 4.9 years, and the diagnostic delay after the first signs of viral infection was 7 days. The lower limbs were involved in 49 patients (94%) with typical lesions. In all, 41 patients (78%) had a recent history of varicella-zoster virus infection, and 7 patients (14%) had been infected by HHV-6. Most of the patients received heparin (n = 51; 98%) and fresh frozen plasma transfusions (n = 41; 79%); other treatment options were immunoglobulin infusion, platelet transfusion, corticosteroid therapy, plasmapheresis, and coagulation regulator concentrate infusion. The antithrombin level and platelet count at diagnosis seemed to be associated with severe complications. Given the rarity of this disease, the creation of a prospective international registry is required to consolidate these findings.

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.

Refractory gram-negative septic shock complicated by extended purpura fulminans and multiple organ failure in a 23-year-old puerpera -a case report.

BACKGROUND: Pregnancy-related infections are the third most common cause of maternal death worldwide. The aim of this report is to present a case of pregnancy-related infection, which progressed into refractory septic shock accompanied by purpura fulminans and multiple organ failure. CASE: A 23-year-old woman in the postpartum period developed fulminant, refractory septic shock complicated by purpura fulminans and multiple organ failure syndrome (acute respiratory distress syndrome, acute kidney injury, and encephalopathy). Management included antibacterial therapy, fluid and transfusion therapy, nutritional support, protective mechanical ventilation, hydrocortisone, a large dose of ascorbic acid, and thiamine. There were no neurological consequences and all organ functions returned to normal, although the predicted hospital mortality based on the Sequential Organ Failure Assessment (SOFA) score was more than 90%. CONCLUSIONS: Septic shock is a significant, yet not completely understood life-threatening condition, which can be associated with purpura fulminans, multiple organ dysfunction, disseminated intravascular coagulation, and massive tissue necrosis.

Acute infectious purpura fulminans: a case series from India.

Acute infectious purpura fulminans is a serious, potentially fatal condition. We present a case series of 11 patients from March 2005 to March 2017, whose clinical symptoms were fever (100%), confusion (63.6%) and headache (55%), and whose common laboratory abnormalities were thrombocytopenia (100%), elevated alkaline phosphatase (70%) and anaemia (63.6%). Three patients (27%) developed gangrene and two presented in shock. Only one grew Neisseria meningitidis in cerebrospinal fluid (CSF) culture and another confirmed by latex agglutination and polymerase chain reaction in CSF. Five others had serology confirmed spotted fever rickettsioses (SFG). All received broad spectrum antibiotics; in 9/11 patients, this included doxycycline or azithromycin. The mean hospital stay was 10.2 days and overall mortality was 18.2%.

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.

Management of purpura fulminans skin lesions in a premature neonate with sepsis: a case study.

Purpura fulminans is a severe and rapidly progressive septic process characterised by the development of haemorrhagic and ecchymotic lesions and skin necrosis. It can appear on any part of the body but predominantly affects the limbs. Purpura fulminans is a rare but possible complication in paediatric patients, especially neonates. It can increase their risk of morbidity and mortality if not treated early and cause a severe long-term condition in survivors of the infectious episode, including amputation. For professionals involved in wound healing, purpura fulminans poses a major challenge. This report describes the case of a premature neonate with extensive purpura fulminans of the legs and arms. Topical treatment of the limbs and purpuric areas with hyperoxygenated fatty acids (HOFAs) every two hours produced an improvement in the lesions. Complete healing was achieved using moist wound healing products. Early topical application of HOFAs appears to be a safe treatment that improves tissue microcirculation in paediatric patients with Purpura fulminans, minimising sepsis-related skin damage.

Protein C Deficiency.

Protein C (PC) deficiency is a heritable or acquired risk factor for thrombophilia, with presentations varying from asymptomatic to venous thromboembolism to neonatal purpura fulminans, a life-threatening disorder. Hereditary PC deficiency is caused by mutation in the PC (PROC) gene located on chromosome 2q14.3. Heterozygous and acquired PC deficiencies are more common than homozygous deficiency. The recommended initial laboratory test measures PC activity using either clot-based or chromogenic methods. There are numerous potential interferences in PC activity testing that may result in either false-positive (falsely low activity) or false-negative (falsely normal or elevated activity) results. In the present review, we discuss common clinical presentations; laboratory testing, with a focus on potential assay interferences; treatment options; and prognosis in patients with PC deficiency.

Streptococcal toxic shock syndrome in a returning traveller.

Background: A patient presenting with fever and purpura after a stay in the tropics tempts a physician to make a differential diagnosis mainly focusing on imported diseases. Although the importance of considering a tropical disease is obvious, the fact that cosmopolitan infections account for one third of the cases in a febrile returning traveler must not be overseen. Toxic Shock Syndrome is amongst the most notorious diseases due to the high mortality when inappropriately managed and the association with necrotizing fasciitis. Methods : We present a 60-year old female with fever, shock syndrome and progressive appearance of painful purpura on the lower legs after a 2-week holiday in Zanzibar. Results : The patient was diagnosed with Streptococcal Toxic Shock Syndrome. Treatment focusing on aggressive fluid resuscitation, prompt administration of antibiotics (ceftriaxon, doxycycline and one dose of amikacin) and adjunctive treatment by clindamycin and immunoglobulin was initiated. She was also immediately taken into surgery for a bilateral fasciotomy and surgical exploration of the lower legs. Histology appeared compatible with purpura fulminans, thereby excluding necrotizing fasciitis. No source of infection could be identified.  Conclusion: Toxic Shock Syndrome remains a challenging diagnosis and even more in a returning traveler with an extensive differential diagnosis containing both tropical and cosmopolitan diseases. Cornerstones for the treatment of Streptococcal Toxic Shock Syndrome are abrupt administration of antimicrobial therapy comprising beta-lactam antibiotics and clindamycin and surgical exploration to apply source control when indicated.