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. [pesquisa.bvsalud.org]

PF presents as a cutaneous manifestation of disseminated intravascular coagulation. We describe a case of PF in a newborn with left leg ischemia and undetectable PC levels soon after birth. [ncbi.nlm.nih.gov]

Albeit very rare, inherited causes leading to severe deficiency of Protein C or S can also present during neonatal period. [omicsonline.org]

• Fever

  PF though common with rocky mountain spotted fever (RMSF) is rarely seen in association with Indian tick typhus, the usual cause of spotted fever in India. [jpgmonline.com]

  (se Scarlet Fever, [[Scarlet Fever]]) Streptococcal Pharyngitis (see Streptococcus, [[Streptococcus]]): Streptococcus species are the most common etiology in adults Upper Respiratory Infection Varicella (see Varicella-Zoster Virus, [[Varicella-Zoster [mdnxs.com]

  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. [pesquisa.bvsalud.org]

  ENTERIC OR TYPHOID FEVER By James C Wilson M D Phila 149 GLANDULAR FEVER By Alfred Stengel M D Philadelphia Pa Phy 157 DIPHTHERIA By Lennox Browne F R C S Ed London England 185 SCARLET FEVER AND MEASLES By Floyd M Crandall M D 236 RUBELLA By William A [books.google.es]

  Case 1 A 6-month-old female infant presented with high-grade fever of 10 days duration associated with a skin rash, which started three days after the onset of fever. [idoj.in]

• Inflammation

  Purpura fulminans is characterized by microvascular thrombosis in the dermis followed by perivascular hemorrhage, necrosis, and minimal inflammation. [synapse.koreamed.org]

  It is characterized by microvascular thrombosis in the dermis followed by perivascular hemorrhage, necrosis, and minimal inflammation. Laboratory findings are consistent with DIC. [ncbi.nlm.nih.gov]

  Histopathology revealed blood vessels with intramural fibrinoid necrosis and transmural inflammation composed of lymphocytes and neutrophils [Figure 3]. Hemorrhagic necrosis of epidermis, dermis, and subcutis was seen. [idoj.in]

  The protein C pathway in tissue inflammation and injury: Pathogenic role and therapeutic implications. Blood 2010;115:1121-30. [ PUBMED ] 6. Dahlbäck B. The protein C anticoagulant system: Inherited defects as basis for venous thrombosis. [ijaweb.org]

• Developmental Delay

  Starting anti-coagulation decisively may also help to prevent developmental delays in rapidly growing infants with this disease. [jmedicalcasereports.biomedcentral.com]

  No intellectual disability or psychomotor developmental delay was demonstrated. The proband, the first child of nonconsanguineous parents, was born at 38 weeks and 4 days of gestation. [nature.com]

• Multiple Organ Dysfunction Syndrome

  “Microvascular thrombosis and multiple organ dysfunction syndrome”. Crit Care Med. vol. 38. 2010. pp. S35-S42. (Discussion of physiologic derangements with extensive microvascular thrombosis.) Silver, RM, Major, H. [dermatologyadvisor.com]

• Gangrene of the Toe

  A few hours later, a purpuric rash developed over the scrotum and upper and lower extremities, with gangrenous fingers and toes ( Figures 1 and 2 ). [ncbi.nlm.nih.gov]

  Gangrene of the toes following pneumococcemia: treatment with sulfanilamide. Am J Dis Child, 56 (1938), pp. 1317-1318 [5] J.D. Coonrod, R.P. Leach. Antigenemia in fulminant pneumococcemia. Ann Intern Med, 84 (1976), pp. 561-563 [6] P. Lanzkowsky, A. [actasdermo.org]

• Easy Bruising

  There is evidence of bleeding under the skin, with easy bruising and the development of petechiae. In the acute form there may be bleeding from any of the body orifices, such as hematuria, nosebleed, vaginal bleeding, and bleeding gums. [medical-dictionary.thefreedictionary.com]

• Diarrhea

  Other symptoms can include malaise, anorexia, nausea, vomiting, abdominal pain, diarrhea, and photophobia. The typical rash seen in 50-90% of cases is maculopapular and spreads centripetally from the extremities. [jpgmonline.com]

  At least 3 out of 10 criteria yields sensitivity and specificity of 82 and 87%: unexplained weight loss > 4 kg livedo reticularis testicular pain myalgias, weakness Abdominal pain,diarrhea,and GI bleeding mononeuropathy or polyneuropathy new onset diastolic [findzebra.com]

  Other side effects are less serious (e.g., nausea, vomiting, abdominal pain, diarrhea). surgical debridement (back to contents) general conceptualization as a burn wound Extensive skin necrosis due to purpura fulminans may mimic burns. [emcrit.org]

  These usually manifest with nausea, vomiting, diarrhea and anorexia. Congestive heart failure manifestations. Lung involvement leads to pneumonitis. Eye changes: retinal hemorrhage. [drmhijazy.com]

  Nonspecific very early symptoms (first 4-6 hours): Fever Headache Myalgia Influenza-like symptoms Early symptoms (median time to onset = 7-12 hours): Leg pains Thirst Diarrhea Abnormal skin color Breathing difficulty Cold hands and feet Classic symptoms [clinicaladvisor.com]

• Blood in Stool

  […] cells or white blood cells) is depressed. [aafp.org]

• Protein S Deficiency

  Abstract Homozygous protein C deficiency or homozygous protein S deficiency are rare genetic diseases with catastrophic and fatal purpura fulminans-like or thrombotic complications occurring during the neonatal period. [ncbi.nlm.nih.gov]

  Available online at http://ghr.nlm.nih.gov/condition/protein-c-deficiency. Accessed October 2014. (Reviewed 2009 October). Protein S deficiency. Genetics Home Reference.Available online at http://ghr.nlm.nih.gov/condition/protein-s-deficiency. [labtestsonline.it]

  The main concern of transfusing FFP is to replace Protein C and Protein S which are severely deficient. 3 Protein C concentrate is recommended in severe heritable deficiency of Protein C after confirming the diagnosis to minimize the use of FFP. [jpma.org.pk]

  […] million births Clinical protein C deficiency: 1 in 20,000 individuals Homozygous protein S deficiency is exceptionally rare. [unboundmedicine.com]

• Visual Impairment

  impairment. [2] [23] For post-infection purpura fulminans, new lesions will occur while neutralising autoantibodies are present (1–2 weeks after presentation). [9] A multi-disciplinary care team is usually required for rehabilitation after purpura fulminans [en.wikipedia.org]

• Purpura

  The authors present a neonate with extensive purpura fulminans due to group B streptoccoccal septicemia and evaluated the attributable clinical mortality and morbidity of this potentially lethal syndrome. [ncbi.nlm.nih.gov]

  Recently, Klebsiella oxytoca and Citrobacter have been reported to cause neonatal purpura fulminans.3 We report here Acinetobacter baumannii, a Gram-negative coccobacillus, as the causative organism for neonatal purpura fulminans. [ijdvl.com]

  Purpura fulminans is characterized by microvascular thrombosis in the dermis followed by perivascular hemorrhage, necrosis, and minimal inflammation. [synapse.koreamed.org]

  Outcome in children with purpura fulminans: report on 16 patients. Am J Hematol. 2005 ;80: 20 - 25. Google Scholar | Crossref | Medline | ISI 7. Price, VE, Ledingham, DL, Krümpel, A, Chan, AK. Diagnosis and management of neonatal purpura fulminans. [journals.sagepub.com]

• Skin Lesion

  A four-day-old neonate presented with multiple necrotic skin lesions with abnormal coagulation profile. Skin lesions responded to repeated plasma transfusions but the neonate developed bilateral retinal detachment. [ncbi.nlm.nih.gov]

  Skin lesions did not progress once definitive therapy was instituted, and started to show gradual resolution over the next few days. [omicsonline.org]

  Case Report A four-day-old male neonate was noted to have multiple necrotic skin lesions in thigh and sole of left leg on day 2 of life. [indianpediatrics.net]

• Petechiae

  Cutaneous examination revealed multiple erythematous, petechiae and ecchymotic patches over upper and lower extremities [Figure 1] and trunk. [ijpd.in]

  […] purpura [ per´pu-rah ] a hemorrhagic disease characterized by extravasation of blood into the tissues, under the skin, and through the mucous membranes, and producing spontaneous bruises, ecchymoses, and petechiae (small hemorrhagic spots) on the skin [medical-dictionary.thefreedictionary.com]

  They generally comprise more or less symmetrical and well-defined ‘lakes’ of confluent ecchymosis, without petechiae. [blackwellreference.com]

  By definition, ecchymoses are 1 centimeter in size or larger, and are therefore larger than petechiae ( less than 2 millimeters in diameter) or purpura (2 millimeters to 1 centimeter in diameter). [findzebra.com]

• Cutaneous Manifestation

  PF presents as a cutaneous manifestation of disseminated intravascular coagulation. We describe a case of PF in a newborn with left leg ischemia and undetectable PC levels soon after birth. [ncbi.nlm.nih.gov]

  To conclude, PF is rarely described as a cutaneous manifestation of GABHS and this report highlights the importance of maintaining a high degree of suspicion when confronted with sepsis with cutaneous necrosis. Financial support and sponsorship Nil. [idoj.in]

• Withdrawn

  However, as we became sure that this was a case of acquired PC deficiency, low molecular weight heparin was withdrawn after 5 days. As our patient was an acquired PC deficiency, we did not discharge him on any anticoagulants. [e-ijd.org]

  After three weeks, the catheter had to be withdrawn because a perforation had occurred. Oral anticoagulant therapy was therefore initiated while the patient was also receiving peripheral-vein infusions of protein C concentrate. [nejm.org]

  However, Activated protein C seems to have a higher rate of hemorrhagic complications when compared to protein C concentrate.(16635072) Regardless, activated protein C was withdrawn from the market and is currently unavailable. [emcrit.org]

Other features include a Chief Complaints section addressing the workup and treatment of 50 signs and symptoms, plus a medication index, syndromes glossary, surgical glossary, laboratory values, and tables all for quick reference saving you time and helping [books.google.com]

The initial workup revealed disseminated intravascular coagulopathy, and both blood and CSF cultures grew GBS. He had normal levels of protein C and protein S for his age. [ncbi.nlm.nih.gov]

See Workup for more detail. [emedicine.medscape.com]

This case highlights the presentation of presumed Indian tick typhus with PF and should be considered in the etiological workup of any patient who presents with a severe purpuric and hemorrhagic rash. :: References 1. [jpgmonline.com]

A diagnosis of protein C deficiency was suspected and thrombophilia workup was done for the baby and her parents. Doppler ultrasound of the head and abdomen was normal. There was no family history of venous thrombotic disease. [ispub.com]

• Capnocytophaga

  Epidemiology History : first described by Guelliot in 1884 Etiology Acute Infectious Purpura Fulminans Capnocytophaga Canimorsus (see Capnocytophaga Canimorsus, [[Capnocytophaga Canimorsus]]) Epidemiologic Exposure : dog bite Risk Factor for Bacteremia [mdnxs.com]

  A total of 12 patients with purpura fulminans, disseminated intravascular coagulation and imminent peripheral necroses in association with sepsis caused by Neisseria meningitidis (n = 5), Streptococcus pneumoniae (n = 2), Capnocytophaga canimorsus (n [insights.ovid.com]

  Leschinger, Marco Maggiorini Volume 29, Number 2 / February, 2003 Correspondence Fibrinolytic therapy in Capnocytophaga canimorsus sepsis after dog bite A. R. T. van de Ven, A. C. M. van Vliet, B. Maraha, H. H. [icmjournal.esicm.org]

  […] ischemic tissue damage. common causes (back to contents) Bacterial infections Neisseria meningitides (~20% of patients develop purpura fulminans) Haemophilus influenzae Streptococcus pneumoniae (especially status post splenectomy) Streptococcus pyogenes Capnocytophaga [emcrit.org]

The treatments given during surgery are shown in Figure 1. [nejm.org]

Make informed clinical choices for each patient, from diagnosis and treatment selection through post-treatment strategies and management of complications, with new evidence-based criteria throughout. [books.google.com]

Prenatal outset of thrombotic events is a rare situation that worsens the prognosis, especially protein C replacement in utero is not available. [chu-fes.ma]

This is crucial considering the poor prognosis and the need for multidisciplinary care to address the ophthalmological and neurological sequelae of this dreaded condition. [indianpediatrics.net]

( i ) it occurs in patients with a history of administering certain medications; ( ii ) and with no prodromal infections; ( iii ) serum protein C and protein S levels may be normal; ( iv ) it responds to corticosteroids; ( v ) generally with a good prognosis [medicaljournals.se]

In severe cases vascular damage can result in purpuric or petechial rash that can progress to purpura fulminans and is generally indicative of bad prognosis. [jpgmonline.com]

Epidemiology History : first described by Guelliot in 1884 Etiology Acute Infectious Purpura Fulminans Capnocytophaga Canimorsus (see Capnocytophaga Canimorsus, [[Capnocytophaga Canimorsus]]) Epidemiologic Exposure : dog bite Risk Factor for Bacteremia [mdnxs.com]

Information Journal Paper Pages  Start Page 34 | End Page 37 Abstract  Neonatal purpura fulminans is a rare and life threatening disease that can be inherited or acquired in etiology. It manifests as DIC and extensive subcutaneous thrombosis. [sid.ir]

If the etiology issecondary to severe infection, appropriate intravenous antibioticsshould be administered.In PC deficiency the initial management is FFP or PC concentrate until all lesions have healed. [ijn.mums.ac.ir]

However, we report a 1 year and 4 months boy, who presented with acquired PC deficiency possibly of postinfectious etiology and developed PF. [e-ijd.org]

Epidemiology History : first described by Guelliot in 1884 Etiology Acute Infectious Purpura Fulminans Capnocytophaga Canimorsus (see Capnocytophaga Canimorsus, [[Capnocytophaga Canimorsus]]) Epidemiologic Exposure : dog bite Risk Factor for Bacteremia [mdnxs.com]

Epidemiology of invasive group B streptococcal disease in the United States, 1999–2005. JAMA. 2008; 299 :2056–65. [ PubMed ] [ Google Scholar ] 18. Chalmers E, Cooper P, Forman K, et al. Purpura fulminans: recognition, diagnosis and management. [ncbi.nlm.nih.gov]

He has since completed further training in emergency medicine, clinical toxicology, clinical epidemiology and health professional education. [lifeinthefastlane.com]

[…] disseminated intravascular coagulation (DIC) Associated with a congenital or acquired protein C and/or protein S deficiency Life-threatening condition that requires prompt diagnosis and judicious replacement therapy to decrease morbidity and mortality Epidemiology [unboundmedicine.com]

Genatlas disease for PROC Gene thrombosis,recurrent (pro-C type I and type II deficiency) Relevant External Links for PROC Genetic Association Database (GAD) PROC Human Genome Epidemiology (HuGE) Navigator PROC Atlas of Genetics and Cytogenetics in Oncology [genecards.org]

This may lead to a transient hypercoagulable state (which is the pathophysiology underlying warfarin-induced skin necrosis). The primary pathophysiologic problem in purpura fulminans is a deficiency of protein C. [emcrit.org]

While three distinct presentations of purpura fulminans are recognized, the underlying pathophysiology stems from a qualitative or quantitative deficiency in protein C, an important regulator of the clotting cascade. [emedicine.medscape.com]

Pathophysiology Common features of purpura fulminans: DIC: Endothelial injury from bacterial endotoxin or other trigger may initiate secretion of inflammatory cytokines or activation of coagulation and complement proteins. [unboundmedicine.com]

(Overview of pathophysiology and assessment of DIC.) Piette, W, Bolognia, JL, Jorizzo, JL, Rapini, RP. “Cutaneous manifestations of microvascular occlusion syndromes”. Dermatology. 2008. pp. 331-45. [dermatologyadvisor.com]

Pathophysiology Purpura fulminans is the acute onset of often rapidly progressing cutaneous hemorrhage and necrosis caused by dermal vascular thrombosis and disseminated intravascular necrosis. [ncbi.nlm.nih.gov]

Prevention of perinatal group B streptococcal disease: a public health perspective. Centers for Disease Control and Prevention. MMWR Recomm Rep. 1996; 45 :1–24. [ PubMed ] [ Google Scholar ] 16. Eberly MD, Rajnik M. [ncbi.nlm.nih.gov]

Rapidly find the answers you need with separate sections on diseases and disorders, differential diagnosis, clinical algorithms, laboratory results, and clinical preventive services, plus an at-a-glance format that uses cross-references, outlines, bullets [books.google.com]

[…] minus' sign Virchow -triad Use Parentheses water AND (cup OR glass) Add an asterisk (*) at end of a word to include word stems Neuro* will search for Neurology, Neuroscientist, Neurological, and so on Use quotes to search for an exact phrase "primary prevention [read.qxmd.com]

Prenatal diagnosis, early recognition, and urgent therapy with protein C replacement and anticoagulant treatment are crucial to improve the prognosis, avoid further damage after delivery, and prevent the devastating consequences of severe protein C deficiency [chu-fes.ma]

Prevention of hypothermia is also of utmost importance in patients suffering from protein C deficiency, as hypothermia may trigger thrombosis. [ijaweb.org]