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Spot diagnosis: An ominous rash in a newborn

Italian Journal of Pediatrics volume 35, Article number: 10 (2009) Cite this article

Abstract

Purpura fulminans (PF) is an ominous cutaneous condition usually associated with meningococcemia. PF in the newborn is rarely reported. We report the case of a female preterm infant with extensive PF due to group B streptococcus (GBS) septicemia. She developed multi-organ system failure despite neonatal intensive care support and succumbed 9 days later. GBS, sensitive to penicillin, was isolated from the blood cultures of the mother and the infant. Invasive early GBS infection is common in the newborn and is empirically treated with prompt institution of intravenous antibiotics. PF associated with GBS is a rare cutaneous sign that must not be missed. Mortality remains high despite aggressive treatment and ICU support.

Introduction

Purpura fulminans (PF) is an ominous cutaneous condition usually associated with meningococcemia [1–9]. PF in the newborn is rarely reported [6–12] We report the case of a female preterm infant with extensive PF due to group B streptococcus (GBS) septicemia and discussion issues of management of this rare but often fatal condition.

Case

Purupura fulminans (PF) was immediately evident in a moribund 2.7 kg newborn girl delivered by emergency caesarean section for fetal tachycardia (200/minute by cardiotocography) at 35 week gestation 1. There was no family history of bleeding disorder. The membranes were ruptured 3 hours prior to delivery. The mother developed intrapartum fever (38.9°C) with chills and rigors and was given intravenous ampicillin and gentamicin 23 minutes before delivery by emergency caesarean section. At birth, the baby was apneic with heart rate of 80/minute. She cried and the heart rate responded upon bag and mask ventilation for 1 minute. Apgars were 8 and 10 at 1 and 5 minutes, respectively. On arrival at the NICU, the baby developed further apneas with cyanosis followed by tachypnea, insucking chest and grunting. Her mean arterial blood pressure was 30 mmHg and heart rate 190/minute. Arterial blood gas analysis showed a pH of 7.19, pCO2 8.03 kPa, pO2 2.25 kPa, and base excess of -6.9 mmol/L. Respiratory support (nasal continuous positive airway pressure of 5 cm H2O with 8 L/min of oxygen), normal saline bolus, and intravenous penicillin plus gentamicin were administered within the first hour of resuscitation. In the next 2 hours, she remained hypotensive despite further saline boluses, dopamine infusion and mechanical ventilation. Group B streptococcus, sensitive to penicillin, was isolated from the blood cultures of the mother and the infant. She was aggressively treated with broad antibiotic coverage, cardiopulmonary support with mechanical ventilation and multiple inotropes, and peritoneal dialysis (Table 1). The purpuric rash became more extensive and she developed progressive multi-organ system failure despite full intensive care support and succumbed 9 days later

Figure 1
figure1

Purupura fulminans (PF) was immediately evident in a moribund 2.7 kg newborn girl delivered by emergency caesarean section for fetal tachycardia (200/minute by cardiotocography) at 35 week gestation.

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Table 1 Multi-organ system failure in the neonate with group B streptococcal septicemia
Full size table

Discussion

This report illustrates that PF is an ominous cutaneous sign of fulminant neonatal GBS septicemia. Childhood PF is often associated with meningococcaemia [1–3]. In the neonatal period group B streptococcus is the major cause of PF but gram negative organisms such as Escherichia coli and Enterobacter have been described [7–11].

Nolan et al reported two cases of PF associated with meningococcal and chickenpox, respectively, and reviewed various treatment modalities [1]. Protein C and antithrombin III have been given if these factors are deficient [1, 5]. In PF associated with meningococcemia and septic shock, Rivard et al described severe acquired protein C deficiency successfully treated with conventional therapy and high-volume plasma exchange as a source of protein C [2]. Many other therapies have been described in case reports that claimed to arrest the progression of neonatal PF, such as the use of heparin [8], Protein C [5, 12], Antithrombin III, recombinant tissue plasminogen activator (rtPA), epoprostenol (prostacyclin) [6], topical nitroglycerin, intravenous dextran, and plasmapheresis [1–13]. Nevertheless, there is no strong evidence in favor of one particular therapy due to the small number of cases.

One limitation of our case was that the coagulopathy was immediately treated empirically before the endogenous activities of the anticoagulant factors protein C, protein S, and Antithrombin III (AT III) were assessed. In case any of these factors are found to be affected, human protein C or recombinant human activated protein C may be considered, and protein C, protein S, and AT III genes should have been analyzed in the patient and her parents. There was no family history of coagulopathy and group B streptococcus was identified to be the pathogen, making syndromes of congenital anticoagulant factor deficiency unlikely in this patient.

Regardless of the pathogen, neonatal PF must be promptly recognized and aggressively treated in children [1–6]. Early goal-directed therapy provides significant benefits with respect to outcome in adult patients with severe sepsis and septic shock [14]. The therapy involves adjustments of cardiac preload, afterload, and contractility to balance oxygen delivery with oxygen demand. Guidelines were proposed through the Surviving Sepsis Campaign to improve outcome in septic patients [15]. They are difficult to apply routinely and validate in neonates. As Claessens et al has commented, attempts to apply all of the procedures recommended by the experts, despite the apparent pragmatism of those procedures, have varied widely; diagnosis may be problematic because of atypical or unspecific presentations, biomarkers are of little help at the start of treatment and are unspecific, supportive treatment often depends on local supply of resources, and specific devices are often absent for initial therapy and monitoring [15]. Resuscitation policy for septic shock in neonates generally includes prompt treatment of the underlying infection with broad spectrum antibiotics, replacement of fluids or blood for preload, appropriate usage of inotropes for cardiac contractility and afterload, support of oxygenation and ventilation with mechanical ventilation, and support of individual multi-organ system failure [16].

Despite full intensive care support, PF is often associated with multiple organ failure and high mortality in children [4]. PF has a reported mortality of 50 per cent secondary to multiple organ failure which commonly accompanies the syndrome and is associated with major long-term morbidity in those who survive [4]. In 3 cases of early neonatal PF, all the babies survived but had markedly compromised neurologic outcomes [7].

Consent

Written informed consent was obtained from the patient's next of kin for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.

References

  1. 1.

    Nolan J, Sinclair R: Review of management of purpura fulminans and two case reports. Br J Anaesth. 2001, 86: 581-6. 10.1093/bja/86.4.581.

    CAS  Article  PubMed  Google Scholar 

  2. 2.

    Rivard GE, David M, Farrell C, Schwarz HP: Treatment of purpura fulminans in meningococcaemia with protein C concentrate. J Pediatr. 1995, 126: 646-52. 10.1016/S0022-3476(95)70369-1.

    CAS  Article  PubMed  Google Scholar 

  3. 3.

    Darmstadt GL: Acute infectious purpura fulminans: pathogenesis and medical management. Pediatr Dermatol. 1998, 15: 169-83. 10.1046/j.1525-1470.1998.1998015169.x.

    CAS  Article  PubMed  Google Scholar 

  4. 4.

    Sheridan RL, Briggs SE, Remensnyder JP, Tompkins RG: Management strategy in purpura fulminans with multiple organ failure in children. Burns. 1996, 22: 53-6. 10.1016/0305-4179(95)00078-X.

    CAS  Article  PubMed  Google Scholar 

  5. 5.

    Gerson WT, Dickerman JD, Bovill EG, Golden E: Severe acquired protein C deficiency in purpura fulminans associated with disseminated intravascular coagulation: treatment with protein C concentrate. Pediatrics. 1993, 91: 418-22.

    CAS  PubMed  Google Scholar 

  6. 6.

    Stewart FJ, McClure BG, Mayne E: Successful treatment of neonatal purpura fulminans with epoprostenol. J R Soc Med. 1991, 84: 623-4.

    PubMed Central  CAS  PubMed  Google Scholar 

  7. 7.

    Lynn NJ, Pauly TH, Desai NS: Purpura fulminans in three cases of early-onset neonatal group B streptococcal meningitis. J Perinatol. 1991, 11: 144-6.

    CAS  PubMed  Google Scholar 

  8. 8.

    Issacman SH, Heroman WM, Lightsey AL: Purpura fulminans following late-onset group B beta-hemolytic streptococcal sepsis. Am J Dis Child. 1984, 138: 915-916.

    CAS  PubMed  Google Scholar 

  9. 9.

    Atay E, Akin M, Yuzkoller E: Neonatal purpura fulminans associated with early-onset gram-negative enterobacter septicemia: a case report. Int Pediatr. 2003, 18: 162-163.

    Google Scholar 

  10. 10.

    Chu DZ, Blaisdell FW: Purpura fulminans. Am J Surg. 1982, 143: 356-362. 10.1016/0002-9610(82)90106-4.

    CAS  Article  PubMed  Google Scholar 

  11. 11.

    Gurses N, Ozkan A: Neonatal and childhood purpura fulminans: review of seven cases. Cutis. 1988, 41: 361-363.

    CAS  PubMed  Google Scholar 

  12. 12.

    Sen K, Roy A: Management of neonatal purpura fulminans with severe protein C deficiency. Indian Pediatr. 2006, 43: 542-545.

    PubMed  Google Scholar 

  13. 13.

    Edlich RF, Cross CL, Dahlstrom JJ: Modern concepts of the diagnosis and treatment of purpura fulminans. J Environ Pathol Toxicol Oncol. 2008, 27: 191-6.

    Article  PubMed  Google Scholar 

  14. 14.

    Rivers E, Nguyen B, Havstad S: Early goal-directed therapy in the treatment of severe sepsis and septic shock. N Engl J Med. 2001, 345: 1368-1377. 10.1056/NEJMoa010307.

    CAS  Article  PubMed  Google Scholar 

  15. 15.

    Claessens YE, Dhainaut JF: Diagnosis and treatment of severe sepsis. Crit Care. 2007, 11 (Suppl 5): S2-10.1186/cc6153.

    PubMed Central  Article  PubMed  Google Scholar 

  16. 16.

    Gupta S, Sinha SK, Donn SM: Shock and hypotension in the newborn. eMedicine. [http://emedicine.medscape.com/article/979128-overview]

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  1. Department of Paediatrics, The Chinese University of Hong Kong, Prince of Wales Hospital, Shatin, Hong Kong

    Kam-Lun Hon, King-Woon So, William Wong & Kam-Lau Cheung

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  1. Kam-Lun Hon
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Correspondence to Kam-Lun Hon.

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The authors declare that they have no competing interests.

Authors' contributions

All authors participated in the care of the patient. KH is the principal author who drafts the manuscript. KS, WW and KC ensure that the clinical data is accurate. WW provides dialysis care. All authors read and approved the final manuscript.

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Hon, K., So, K., Wong, W. et al. Spot diagnosis: An ominous rash in a newborn. Ital J Pediatr 35, 10 (2009). https://doi.org/10.1186/1824-7288-35-10

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Keywords

  • Continuous Positive Airway Pressure
  • Epoprostenol
  • Emergency Caesarean Section
  • Nasal Continuous Positive Airway Pressure
  • Recombinant Tissue Plasminogen Activator

Italian Journal of Pediatrics

ISSN: 1824-7288

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