Necrotizing Fasciitis of the Serratus Anterior in a Patient Treated With Infliximab and Prednisolone for Ulcerative Colitis and Rheumatoid Arthritis.

Necrotizing fasciitis (NF) is a rapidly progressive bacterial infection with high mortality. Invasive group A Streptococcus (GAS) infection is the leading cause of NF. Our understanding regarding clinicopathological features and pathogenesis of invasive GAS infection is expanding as the incidence of NF in healthy individuals increases. However, clinicopathological features of NF in the presence of autoimmune diseases have been poorly defined. We experienced NF in a patient treated with infliximab and prednisolone for ulcerative colitis and rheumatoid arthritis. Herein, we present time kinetics findings of clinical symptoms and laboratory data of GAS-associated NF in the presence of immunosuppressant-treated immune disorders.

Critical bloodstream infection caused by Chromobacterium violaceum: a case report in a 15-year-old male with sepsis-induced cardiogenic shock and purpura fulminans.

Chromobacterium violaceum (C. violaceum) is a gram-negative bacillus that is widespread in tropical and subtropical areas. Although C. violaceum rarely infects humans, it can cause critical illness with a mortality rate above 50%. Here, we report the successful treatment of a 15-year-old male who presented with bloodstream infection of C. violaceum along with sepsis, specific skin lesions, and liver abscesses. Cardiogenic shock induced by sepsis was reversed by venoarterial extracorporeal membrane oxygenation (VA ECMO). Moreover, C. violaceum-related purpura fulminans, which is reported herein for the first time, was ameliorated after treatment. This case report demonstrates the virulence of C. violaceum with the aim of raising clinical awareness of this disease.

Acute Infectious Purpura Fulminans Complicated by Bacterial Translocation after Rectal Cancer Surgery: A Case Report.

The patient was a man in his 80s who had undergone laparoscopic anterior resection for rectal cancer. Bowel obstruction occurred on the third postoperative day but improved with a decompression tube by the fifth postoperative day. A high fever (in the 38 °C range) was also observed. Blood culture tests detected two sets of the gram-negative bacilli Klebsiella aerogenes within 24 h of collection. On the seventh postoperative day, the patient subsequently went into septic shock with disseminated intravascular coagulation (DIC). On the eighth postoperative day, the fingertips and toes became black, and the palms and dorsal surfaces of both feet were dark purple due to peripheral circulatory failure. This suggested acute infectious purpura associated with sepsis (acute infectious purpura fulminans (AIPF)). Intensive care was provided; however, the necrosis of both middle fingers worsened, both middle fingers were gangrenous, and the patient died on the thirtieth postoperative day. AIPF is rarely reported, especially in early-onset cases after elective surgery. We encountered a rare complication of bacterial translocation from postoperative bowel obstruction, leading to AIPF.

Purpura Fulminans as a Rare Manifestation of Invasive Haemophilus influenzae Disease: A Case Report.

Haemophilus influenzae (Hi) is a bacterium usually found in the upper respiratory tract of humans. Though it is recognized as a naturally occurring element in the human bacterial reservoir, Hi infections have the potential to be severe and even fatal, particularly when they result in conditions such as meningitis or epiglottitis. Because of this, Hi invasive infections are considered a reportable disease in Portugal. We report a case of a 58-year-old female, chronically adrenally suppressed on long-term steroids, who developed an invasive Hi type b infection that led to purpura fulminans and multiorgan failure after an acute episode of epiglottitis. According to our review of the literature, only three previous cases of invasive Hi type b disease-causing purpura fulminans have been described.

Infectious purpura fulminans associated with pneumococcal septicaemia in a patient with unacknowledged functional asplenia.

Purpura fulminans (PF) is a life-threatening complication of septic shock that can occur due to disseminated infections with Streptococcus pneumoniae The spleen is an important organ in the immunisation process against encapsulated bacteria. Patients with asplenia, either functional or anatomical, are therefore at increased risk of developing serious infections and complications, such as PF, if infected with such bacteria.This case report presents a woman in her late 40s with unacknowledged functional asplenia who was admitted to the hospital with signs of an acute disseminated infection causing septic shock, signs of disseminated intravascular coagulation and infectious PF. A few days after admission, the blood cultures showed growth of S. pneumoniae With early sepsis treatment, the patient survived although with some complications. Clinical presentation, investigations, differential diagnosis, treatment and outcome are presented. Treatment and early recognition of PF are presented and discussed. Relevant recognition and preventative treatment strategies for patients with asplenia are also reviewed and discussed.This case demonstrates the importance of early recognition and treatment of PF in septic patients and the importance of preventive treatment strategies for patients with asplenia to avoid serious infections and complications.

Eficacia y seguridad de un compuesto de ácidos grasos hiperoxigenados en la mejora de la microcirculación de la púrpura fulminante en pacientes pediátricos con sepsis: estudio piloto / Efficacy and safety of a hyperoxygenated fatty acid compound in improving the microcirculation of purpura fulminans in paediatric patients with sepsis: a pilot study

Introducción La púrpura fulminante (PF) es una complicación grave de la sepsis resultado de un conjunto de alteraciones caracterizadas por el desarrollo de lesiones hemorrágicas equimóticas y necrosis de la piel. Objetivo Analizar la eficacia y la seguridad de la aplicación tópica de un compuesto de ácidos grasos hiperoxigenados (AGHO) en la microcirculación de la PF en pacientes pediátricos afectados de sepsis. Material y métodos Se llevó a cabo un estudio prospectivo cuasiexperimental pretest-posttest de un solo grupo en una Unidad de Cuidados Intensivos Pediátrica (UCIP). Se incluyeron en el estudio pacientes con PF de 0 a 18 años. Para evaluar la efectividad del producto objeto de evaluación en la microcirculación de las lesiones por PF se determinaron los valores de oximetría somática antes y después de la aplicación de AGHO cada 4h durante los 3 primeros días de ingreso de los pacientes. Resultados Se reclutaron 4 pacientes, con una mediana de edad de 98 meses. Las lesiones purpúricas que se midieron estaban ubicadas, sobre todo, en ambos pies y manos y, en 2 pacientes, también en los maléolos laterales y gemelos de ambas extremidades inferiores. Se obtuvieron un total de 225 mediciones, observando unas puntuaciones medias preintervención de 71,17±15,65% versus los 73,68±14,83% postintervención. Se objetivó significación estadística (p<0,001) al comparar las mediciones pre- y postintervención. Conclusiones La aplicación precoz y continuada de AGHO en el manejo de la PF por sepsis es una práctica eficaz y segura en los casos de población pediátrica analizada. En más de la mitad de los episodios analizados se objetivó un aumento de microcirculación tisular tras la aplicación de los AGHO, sin eventos adversos. (AU)

Introduction Purpura fulminans (PF) is a serious complication of sepsis resulting from a set of alterations characterised by the development of ecchymotic haemorrhagic lesions and skin necrosis. Aim To analyse the efficacy and safety of the topical application of HOFA compound, in the cutaneous microcirculation of PF lesions in paediatric patients affected by sepsis. Material and methods A prospective quasi-experimental pre-test/post-test single-group conducted in a Paediatric Intensive Care Unit of a third level hospital was performed. Paediatric patients aged 0-18 years with sepsis were included. Somatic oximetry values were measured before and after application of HOFAs every 4hours over the first three days of the patients’ hospitalisation. Patient's socio-demographic and clinical variables and somatic oximetry by placing a sensor for measuring tissue perfusion on the area with PF were determined. Results Four patients were recruited, with a median age of 98 months. The purpuric lesions measured were mainly located on both feet and hands and, in two patients, also on the lateral malleoli and calves of both lower extremities. A total of 225 measurements were obtained, with mean pre-intervention scores of 71.17±15.65% versus 73.68±14.83% post-intervention. Statistical significance (p<0.001) was observed upon comparison of the pre- and post-intervention measurements. Conclusions Early and continued application of HOFAs in the management of sepsis-induced PF is an effective and safe practice in the cases analysed. In more than half of the episodes analysed, an increase in tissue microcirculation was observed after the application of HOFAs, with no adverse events. (AU)

Eficacia y seguridad de un compuesto de ácidos grasos hiperoxigenados en la mejora de la microcirculación de la púrpura fulminante en pacientes pediátricos con sepsis: estudio piloto / Efficacy and safety of a hyperoxygenated fatty acid compound in improving the microcirculation of purpura fulminans in paediatric patients with sepsis: a pilot study

Introducción La púrpura fulminante (PF) es una complicación grave de la sepsis resultado de un conjunto de alteraciones caracterizadas por el desarrollo de lesiones hemorrágicas equimóticas y necrosis de la piel. Objetivo Analizar la eficacia y la seguridad de la aplicación tópica de un compuesto de ácidos grasos hiperoxigenados (AGHO) en la microcirculación de la PF en pacientes pediátricos afectados de sepsis. Material y métodos Se llevó a cabo un estudio prospectivo cuasiexperimental pretest-posttest de un solo grupo en una Unidad de Cuidados Intensivos Pediátrica (UCIP). Se incluyeron en el estudio pacientes con PF de 0 a 18 años. Para evaluar la efectividad del producto objeto de evaluación en la microcirculación de las lesiones por PF se determinaron los valores de oximetría somática antes y después de la aplicación de AGHO cada 4h durante los 3 primeros días de ingreso de los pacientes. Resultados Se reclutaron 4 pacientes, con una mediana de edad de 98 meses. Las lesiones purpúricas que se midieron estaban ubicadas, sobre todo, en ambos pies y manos y, en 2 pacientes, también en los maléolos laterales y gemelos de ambas extremidades inferiores. Se obtuvieron un total de 225 mediciones, observando unas puntuaciones medias preintervención de 71,17±15,65% versus los 73,68±14,83% postintervención. Se objetivó significación estadística (p<0,001) al comparar las mediciones pre- y postintervención. Conclusiones La aplicación precoz y continuada de AGHO en el manejo de la PF por sepsis es una práctica eficaz y segura en los casos de población pediátrica analizada. En más de la mitad de los episodios analizados se objetivó un aumento de microcirculación tisular tras la aplicación de los AGHO, sin eventos adversos. (AU)

Introduction Purpura fulminans (PF) is a serious complication of sepsis resulting from a set of alterations characterised by the development of ecchymotic haemorrhagic lesions and skin necrosis. Aim To analyse the efficacy and safety of the topical application of HOFA compound, in the cutaneous microcirculation of PF lesions in paediatric patients affected by sepsis. Material and methods A prospective quasi-experimental pre-test/post-test single-group conducted in a Paediatric Intensive Care Unit of a third level hospital was performed. Paediatric patients aged 0-18 years with sepsis were included. Somatic oximetry values were measured before and after application of HOFAs every 4hours over the first three days of the patients’ hospitalisation. Patient's socio-demographic and clinical variables and somatic oximetry by placing a sensor for measuring tissue perfusion on the area with PF were determined. Results Four patients were recruited, with a median age of 98 months. The purpuric lesions measured were mainly located on both feet and hands and, in two patients, also on the lateral malleoli and calves of both lower extremities. A total of 225 measurements were obtained, with mean pre-intervention scores of 71.17±15.65% versus 73.68±14.83% post-intervention. Statistical significance (p<0.001) was observed upon comparison of the pre- and post-intervention measurements. Conclusions Early and continued application of HOFAs in the management of sepsis-induced PF is an effective and safe practice in the cases analysed. In more than half of the episodes analysed, an increase in tissue microcirculation was observed after the application of HOFAs, with no adverse events. (AU)

Interesting Mucocutaneous Manifestations in COVID-19 Infection or Vaccination Confirmed by Histopathology: A Case Series.

Introduction: Mucocutaneous complications or adverse events due to SARS-CoV-2 infection or vaccination have been well delineated in the literature, respectively. Most eruptions are considered mild and self-limiting; however, for the atypical cases with a tentative clinical diagnosis, performing a biopsy and histopathological assessment is pivotal to confirm the diagnosis and subsequently prescribe a more tailored treatment. Despite the diverse reporting of such incidents globally, most studies restrict the rate of biopsied cases to less than 15%. Case Presentations: This case series elucidates 20 patients referred to the tertiary dermatology clinic, including 14 COVID-19 infection-related eruptions such as lichen planus (LP), cutaneous vasculitis, pityriasis rosea (PR), discoid lupus erythematosus, guttate psoriasis, sarcoidosis, Raynaud's phenomenon, non-specific lesions resembling genital warts, Beau's line, and one severe case of purpura fulminans with a promising outcome. Moreover, we presented six vaccine-induced cases comprising LP, urticarial vasculitis, PR, parapsoriasis, and localized morphea. The diagnosis of all challenging cases has been proven by histopathological evaluation. We included pertaining anamnesis details of each patient and vivid classifying images to pinpoint the morphologic features of each condition. Discussion: In line with our previous studies, the vaccine-induced eruptions were less severe compared to infection-related complications of COVID-19 and are mostly controllable by antihistamines and corticosteroid administration. Therefore, reporting such events should not impede COVID-19 vaccination in the general population.

Neonatal Purpura Fulminans in a Patient With Arterial Thrombosis and Congenital Renal Agenesis.

Neonatal purpura fulminans (PF) is an uncommon skin disorder characterized by acute disseminated intravascular coagulation, tissue necrosis, and small vessel thrombosis. Here, we present a case of a seven-day-old male who was admitted to the Neonatal Intensive Care (NICU) Unit at Gaafar Ibn Auf Tertiary Hospital, in January 2023. He presented with black bullous lesions on the plantar surface of the left foot, deep bluish discoloration over the right buttock and right lower abdomen, and gangrenous changes in the right foot with clear demarcation. Birth history was not significant other than mild pallor and icterus. His blood workup was consistent with severe anemia, thrombocytopenia, elevated prothrombin time, and partial thromboplastin time with decreased protein C and S levels; blood culture yielded no growth. A Doppler ultrasound scan of lower extremities confirmed distal occlusion of the right dorsalis pedis artery. The abdominal ultrasound revealed a free left renal bed and left-sided renal agenesis. We came to a diagnosis of neonatal PF and started administering blood and fresh frozen plasma and subcutaneous heparin injections, but unfortunately, the patient eventually passed away. Hence, we decided to report this case to emphasize the significance of the clinical picture in assisting with early diagnosis, despite limited access to genetic testing. We also want to highlight the importance of a "high index of suspicion" that might be mandatory for better outcomes.

Postinfectious purpura fulminans: A case report. / Púrpura fulminante postinfecciosa: a propósito de un caso.

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.

Efficacy and safety of a hyperoxygenated fatty acid compound in improving the microcirculation of purpura fulminans in paediatric patients with sepsis: a pilot study.

INTRODUCTION: Purpura fulminans (PF) is a serious complication of sepsis resulting from a set of alterations characterised by the development of ecchymotic haemorrhagic lesions and skin necrosis. AIM: To analyse the efficacy and safety of the topical application of HOFA compound, in the cutaneous microcirculation of PF lesions in paediatric patients affected by sepsis. MATERIAL AND METHODS: A prospective quasi-experimental pre-test/post-test single-group conducted in a Paediatric Intensive Care Unit of a third level hospital was performed. Paediatric patients aged 0-18 years with sepsis were included. Somatic oximetry values were measured before and after application of HOFAs every 4h over the first three days of the patients' hospitalisation. Patient's socio-demographic and clinical variables and somatic oximetry by placing a sensor for measuring tissue perfusion on the area with PF were determined. RESULTS: Four patients were recruited, with a median age of 98 months. The purpuric lesions measured were mainly located on both feet and hands and, in two patients, also on the lateral malleoli and calves of both lower extremities. A total of 225 measurements were obtained, with mean pre-intervention scores of 71.17±15.65% versus 73.68±14.83% post-intervention. Statistical significance (p<0.001) was observed upon comparison of the pre- and post-intervention measurements. CONCLUSIONS: Early and continued application of HOFAs in the management of sepsis-induced PF is an effective and safe practice in the cases analysed. In more than half of the episodes analysed, an increase in tissue microcirculation was observed after the application of HOFAs, with no adverse events.

New plasma protein C and protein S concentrate: A synergy for therapeutic purposes.

BACKGROUND AND OBJECTIVES: Deficiencies of protein C (PC) or protein S (PS) are rare diseases, characterized by mutations in the PC or PS genes, which encode plasma serine proteases with anti-coagulant activity. Severe PC or PS deficiencies manifest in early life as neonatal purpura fulminans, a life-threatening heamorrhagic condition requiring immediate treatment. First-line treatment involves replacement therapy, followed by maintenance with anti-coagulants. Replacement therapy with specific protein concentrates is currently only limited to PC, and therefore, a PC + PS concentrate represents a useful addition to therapeutic options, particularly for severe PS deficiency. Further, the production of a PC + PS concentrate from unused plasma fractionation intermediates would impact favourably on manufacturing costs, and consequently therapy prices for patients and health systems. MATERIALS AND METHODS: Several chromatographic runs were performed on the same unused plasma fractionation intermediates using different supports to obtain a PC/PS concentrate. The best chromatographic mediums were chosen, in terms of specific activity and recovery. A full process of purification including virus inactivation/removal and lyophilization steps was set up. RESULTS: The final freeze-dried product had a mean PC concentration of 47.75 IU/mL with 11% of PS, and a mean specific activity of 202.5 IU/mg protein, corresponding to over 12,000-fold purification from plasma. CONCLUSION: The development of a novel concentrated PC/PS mixture obtained from a waste fraction of other commercial products could be used for its potential therapeutic role in the management of neonatal purpura fulminans pathology.

Complete genome sequences of 11 Streptococcus pneumoniae strains isolated from acute infectious purpura fulminans, sepsis, and pneumonia.

The complete genome sequences of 11 Japanese Streptococcus pneumoniae isolates were determined by hybrid assembly of long and short reads, including two strains isolated from patients with acute infectious purpura fulminans, six strains from patients with sepsis, and three strains from patients with pneumonia.

Púrpura fulminante aguda concomitante con proceso infeccioso pulmonar

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.

The c.1243T>C mutation in the PROC gene is linked with inherited protein C deficiency and severe purpura fulminans.

Purpura fulminans is a severe coagulation disorder that often leads to death in neonates. Mutations in the protein C (PROC) gene can cause protein C deficiency, leading to this disorder. This study aimed to investigate a family with a history of coagulopathies, particularly those related to protein C deficiency. The primary objective was to identify any genetic mutations in the PROC gene responsible for the coagulopathies. The study focused on a male neonate with purpura fulminans who ultimately died at 2 months of age. The patient had low protein C activity levels (6%). The entire PROC gene of the patient and his family was analyzed using next-generation sequencing to identify any genetic mutations. Segregation analysis was conducted to determine if the mutation followed an autosomal dominant inheritance pattern. In silico analysis was also conducted to evaluate the pathogenicity of the identified mutation. Analysis revealed a novel homozygous c.1243T>G variant PROC gene. The mutation resulted in a Phe415Val substitution. The mutation was found in at least three generations of the family. Carrier family members had lower protein C activity levels than wild-type homozygotes. Additionally, the mutation may account for the observed reduction in protein C enzyme activity.

Nsaids Linked to IgA-Mediated Hypersensitivity Vasculitis and Purpura Fulminans-Like Eruption.

Background: IgA vasculitis and hypersensitivity reactions following exposure to non-steroidal anti-inflammatory drugs (NSAIDs) are very rarely associated with purpura fulminans (PF). The latter is a coagulation event characterised by decreased levels of protein C and a rapidly progressive purpuric rash, often leading to ischaemia, amputations and death. Case summary: A previously healthy 66-year-old man presented with a vasculitic rash and abdominal pain following exposure to naproxen (NSAID), which quickly deteriorated to purpura fulminans-like eruption and skin necrosis, mainly involving the face and hands. The presence of IgA sediments on skin biopsy and decreased levels of complement as well as protein C pointed to an immune-mediated inflammatory process. Dramatic clinical escalation with immediate risk to organs and life required an aggressive and broad-spectrum therapeutic approach in an intensive care setting. Clinical improvement and complete reconstitution of protein C were achieved following plasma exchange with fresh frozen plasma (FFP) and immunosuppression with glucocorticoids with no persistent organ damage. Conclusions: This rare case illustrates the catastrophic cross links between NSAIDs, IgA-mediated hypersensitivity vasculitis and purpura fulminans-like syndrome. A high index of suspicion is required for the evaluation of environmental exposures such as drugs and infections in patients with vasculitis and/or purpura. A rapid and comprehensive therapeutic approach should be implemented to avoid multi-organ damage, amputations and death. Complete avoidance of the offending agent is key for future prevention of recurrence. LEARNING POINTS: This case illustrates a rare cross link between a commonly used drug (NSAIDs) and severe, life-threatening hypersensitivity reactions (IgA vasculitis and purpura fulminans-like eruption).These events require a high index of suspicion and emphasise the importance of considering environmental exposures such as drugs in the immediate diagnosis of both conditions.In addition to long-term drug avoidance, early and aggressive interventions are required to avoid organ damage, amputations or death.

Development of purpura fulminans by Candida glabrata and Mucormycosis infection post-surgery.

Purpura fulminans (PF) is a disorder with multifactorial causes that lead to acute localize skin microvasculature thrombosis. PF can be classified as one of the manifestations of disseminated vascular coagulation (DIC). Although, there are three types of PF including hereditary (autosomal dominant) due to mutations in single nucleotide polymorphisms (PROC and PROS1) and serpin family C member 1 (SERPINC1) genes. Idiopathic or acquired type of PF is complex and the pathophysiology is ambiguous, however, low levels of protein C and S were observed. The acute infectious form of PF occurs post-bacterial infection (e.g., Neisseria). The clinical presentation is limited to skin findings or systematic manifestation (shock, disseminated intravascular coagulation, or death). We are presenting two cases of PF sharing similar clinical manifestations developed within 12 h post-operatively with distinct micro-organisms infection. The first patient's wound culture grew fluffy mold, and the sequencing confirmed a Mucormycosis, Absidia corymbifera species, while the second patient was infected by cutaneous Candida glabrata which led to the development of PF. Our findings suggest that surgery can trigger local immunological responses in susceptible individuals such as concealed protein C and S deficiency or microorganism toxins that initiated the rapidly developing of PF in those patients.

Preserving finger length in a patient with symmetric digital gangrene under local anesthesia: A case report.

BACKGROUND: Case reports of symmetric digital gangrene resulting from high-dose vasopressors use in patients with alcoholic ketoacidosis, leading to cardiac arrest, are rare. To date, no specific treatment method for autolysis or surgical amputation or guidelines for determining the level of amputation have been established. CASE SUMMARY: In this case report, we describe a treatment method that effectively preserved the function of fingers by surgical treatment under local anesthesia with a minimum operative time, while also preserving finger length to the maximum possible extent. CONCLUSION: Our approach may contribute to improved postoperative quality of life by preserving finger length.

Case report: Blotchy skin in a puffy neonate: is there a new association?

Introduction: Purpura fulminans in the neonatal population is a rare but potentially life-threatening condition complicated by thrombosis, resultant vital organ necrosis, and gangrene of the extremities. Considering the rapid evolution of the pathogenetic mechanism, an index of suspicion, early identification, and prompt intervention are imperative for improved outcomes. The majority of purpura fulminans cases have an infectious etiology, but it is essential to consider other congenital and acquired causes. Case description: We present a clinical case of a female neonate to emphasize the correlation between purpura fulminans, congenital chylothorax, involvement of the PAK2 gene, and the occurrence of retinal detachment in both eyes. After draining the congenital chylothorax, the neonate developed purpura fulminans due to a loss of protein C, S, and antithrombin factors, previously not reported in the literature. The purpuric lesions resolved after the administration of fresh frozen plasma. Subsequently, no recurring purpura fulminans lesions were noted following the normalization of the antithrombotic factor levels in the serum. Subsequently, the child also developed retinal detachment in both eyes.

Overwhelming Post-splenectomy Infection Caused by Escherichia coli 20 Years After Splenectomy: A Case Report.

Post-splenectomy patients are at increased risk of infection. This complication is called overwhelming post-splenectomy infection (OPSI), which is uncommon but has high mortality. We describe a case of a man in his 80s who presented with septic shock with purpura fulminans caused by pyelonephritis. He had undergone a splenectomy in his 50s and had been taking prednisolone for the past six months for suspected immunoglobulin G4 (IgG4)-related disease. He was admitted to the intensive care unit but died the day after admission. OPSI is generally caused by encapsulated bacteria. However, in the present case, the causative agent was Escherichia coli, a bacterium that typically causes urinary tract infections. Post-splenectomy patients are known to have compromised bacterial clearance, and accumulation of bacteria such as E. coli can induce acute sepsis after splenectomy. Thus, physicians must have a high index of suspicion when treating splenectomy patients for the possibility that they may rapidly deteriorate to severe conditions such as OPSI, and the patients must be informed about the risk of severe infections, which can be fatal.