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]

• Disability

  The mortality rate has been decreasing with supportive care, improved management of secondary complications, and some targeted treatments, but it remains a disabling condition often resulting in major amputations in those who survive. [ncbi.nlm.nih.gov]

  Keywords: Purpura fulminans, antithrombin III, coagulation, disseminated intravascular, protein C Introduction Purpura fulminans (PF) is an hemorrhagic potentially disabling and life-threatening skin disorder. [ijdvl.com]

  While the mortality rate of purpura fulminans has decreased with current treatment modalities, it remains a disabling condition with complex wounds complicated by secondary infections, often requiring major amputations. [podiatrytoday.com]

• Dog Bite

  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]

  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]

  bite. ( 29437318 ) Andreu Ruiz A....Moya SA! [malacards.org]

• Pallor

  Fever, lethargy, weight loss, bone pain, joint pain, pallor, lymphadenopathy and hepatosplenomegaly are characteristically absent. [aafp.org]

  He was noted to have pallor and icterus. An extensive purpuric rash with areas of hemorrhage was seen over both the legs, hands, and the trunk [Figure 1]. Rest of the systemic examination was normal. [jpgmonline.com]

• Streptococcal Pharyngitis

  Pharyngitis (see Streptococcus, [[Streptococcus]]): Streptococcus species are the most common etiology in adults Upper Respiratory Infection Varicella (see Varicella-Zoster Virus, [[Varicella-Zoster Virus]]): common etiology Clinical Presentations Neonatal [mdnxs.com]

• Epilepsy

  Other long-term complications included hearing loss in 1.9%, epilepsy in 1.4%, and cerebral palsy or limb plegia in 0.3%. [clinicaladvisor.com]

• 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]

• Pharyngitis

  Neisseria Meningitidis (see Neisseria Meningitidis, [[Neisseria Meningitidis]]): common etiology (most common in infants and adolescents) Rubella (German Measles) (see Rubella, [[Rubella]]) Scarlet Fever (se Scarlet Fever, [[Scarlet Fever]]) Streptococcal Pharyngitis [mdnxs.com]

  S. pyogenes is isolated from the throat of 10–30% children with acute pharyngitis, and healthy carriers are the source of outbreak. [ann-clinmicrob.biomedcentral.com]

• 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]

• Hemorrhagic Bullae

  Later in the course, hemorrhagic bullae may form, which contribute to the classic hard eschars characteristic of NPF. Differential diagnosis with necrotizing fasciitis should be performed. Treatment of NPF should be multidisciplinary. [jpnim.com]

  Hemorrhagic bullae over necrotic skin were present over left arm, right thigh, and right calf (15 × 15 cm, 12 × 11 cm, and 10 × 9 cm, respectively) [Figure 1]. [idoj.in]

  The skin lesions soon enlarge and become vesiculated, producing hemorrhagic bullae with subsequent necrosis and black eschar formation. The margins of the lesions become erythematous and indurated. Thrombocytopenia is often evident. [emedicine.medscape.com]

  bullae -> black necrotic lesions and gangrene May involve heart, kidneys, and lungs Treatment Neonatal Purpura Fulminans Fresh Frozen Plasma (FFP) (see Fresh Frozen Plasma, [[Fresh Frozen Plasma]]): to replace protein C Low Molecular Weight Heparin/Coumadin [mdnxs.com]

  The other two neonates had a relatively less fulminant progression, with acral hemorrhagic bullae turning necrotic [Figures 2 and 3] within 24 hours followed by the development of gangrene. No new lesions appeared after 72 hours of disease onset. [ijdvl.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]

• Blister

  We report the case of a newborn who presented a sudden dermatosis characterized by tense, serous and hematic blisters secondary to a nosocomial infection by Acinetobacter junii, with subsequent progression to necrosis. [medigraphic.com]

  […] bleeding sites Bruising of skin, mucous membranes Internal bleeding Lack of blood supply to tissues ( ischaemia ) Sudden onset of high fever, severe general malaise, and extensive purpura of the extremities Petechiae, purpuric papules, blood-filled blisters [dermnetnz.org]

  Three full-term neonates, born within 12 hours in a tertiary care facility, developed multiple hemorrhagic blisters and ecchymosis over acral areas and thighs within 12–36 hours of birth. All three were born healthy with a normal Apgar score. [ijdvl.com]

  Haemorrhage into the necrotic skin causes purpura fulminans lesions to become painful, dark and raised, sometimes with vesicle or blister (bulla) formation. [16] The distribution of purpura fulminans lesions may be different according to the underlying [en.wikipedia.org]

• Dermatitis

  In addition, the patient developed exfoliative dermatitis, hepatitis, cutaneous vasculitis, and microangiopathic hemolytic anemia. She was successfully treated with adrenal steroids and heparin for her purpura fulminans. [annals.org]

  Clothing dermatitis. Hyperglobulinaemic purpura. Early mycosis fungoides. [drmhijazy.com]

  Examples of the pigmented purpuric dermatosis group include: * Schamberg's disease * Majocchi's disease ( Purpura annularis telangiectodes) * Gougerot-Blum syndrome (Pigmented purpuric lichenoid dermatitis) * Ducas and Kapetanakis pigmented purpura * [findzebra.com]

  He had atopic dermatitis and one history of pneumonia in infancy. The growth and development were normal. There was neither consanguinity nor informative family history. [ann-clinmicrob.biomedcentral.com]

• Agalactia

  (Group B Strep) (see Streptococcus Agalactiae, [[Streptococcus Agalactiae]]) Streptococcus Pneumoniae (Pneumococcus) (see Streptococcus Pneumoniae, [[Streptococcus Pneumoniae]]) Streptococcus Pyogenes (Group A Strep) (see Streptococcus Pyogenes, [[Streptococcus [mdnxs.com]

• Oliguria

  Because of the association with severe septic shock, the patient may experience symptoms of end-organ damage such as hematuria, oliguria, and respiratory distress. Pain out of proportion to exam should always make one consider necrotizing fasciitis. [statpearls.com]

• Seizure

  Despite seizure and necrotic lesions, the general condition of baby was good and she continued to feed well. The seizures were controlled with Phenobarbital administration. [ijn.mums.ac.ir]

  Abstract A patient developed disseminated intravascular coagulation with purpura fulminans 1 month after starting Dilantin® therapy for a seizure disorder. [annals.org]

  The patient had several episodes of generalized tonic-clonic seizures. At about same time after seizure control, mother noticed she could not use the right upper and lower limbs [Figure 2]. [jfmpc.com]

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]

• Staphylococcus Aureus

  Purpura fulminans caused by community-associated methicillin resistant Staphylococcus aureus. Am J Emerg Med. 2012 ;30: 1013.e1 - 1013.e4. Google Scholar | ISI 4. Darmstadt, GL. [journals.sagepub.com]

  Purpura fulminans in a premature neonate resulting from Staphylococcus aureus septicemia is illustrated. Unfortunately, the baby succumbed to septicemia. [ncbi.nlm.nih.gov]

  Although group B streptococcus has been found as common cause of infectious PF, Staphylococcus aureus induced PF has been described [ 1, 3, 4 ]. [pediatric-infectious-disease.imedpub.com]

  Aureus (see Staphylococcus Aureus, [[Staphylococcus Aureus]]): recently identified etiology [ MEDLINE ] Streptococcus Agalactiae (Group B Strep) (see Streptococcus Agalactiae, [[Streptococcus Agalactiae]]) Streptococcus Pneumoniae (Pneumococcus) (see [mdnxs.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]

• Capnocytophaga Canimorsus

  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]

  canimorsus Staphylococcus aureus Gram-negatives (Klebsiella pneumoniae, Escherichia coli, Pseudomonas aeruginosa, Proteus mirabilis) (Less common: Enterococcus faecalis, Leptospira, Rickettsia rickettsii, Vibrio parahaemolyticus) Viral Varicella zoster [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.de]

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]