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 Table of Contents  
CASE REPORT
Year : 2022  |  Volume : 2  |  Issue : 4  |  Page : 245-248

Neonatal Purpura Fulminans by an Unusual Pathogen: Elizabethkingia meningoseptica


1 Consultant Neonatologist, Surya Hospital, Jaipur, Rajasthan, India
2 Consultant Neonatologist, MJM Hospital, Pune, Maharashtra, India
3 Consultant Neonatologist, Department of Pediatrics, Division of Neonatology, KEM Hospital, Pune, Maharashtra, India

Date of Submission09-Jun-2022
Date of Decision28-Jul-2022
Date of Acceptance31-Oct-2022
Date of Web Publication29-Nov-2022

Correspondence Address:
Dr. Tushar Parikh
Department of Pediatrics, Division of Neonatology, KEM Hospital, TDH Building. Rasta Peth, Pune - 411 011, Maharashtra
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ipcares.ipcares_133_22

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  Abstract 

Background: Neonatal purpura fulminans (PF) is a rare disorder characterized by the formation of dermal microvascular thrombosis associated with disseminated intravascular coagulation (DIC). It can be caused by inherited protein C or protein S deficiency or severe sepsis with DIC due to organisms such as Streptococcus pneumoniae and Gram-negative bacteria. Clinical Description: A preterm boy of 31-week gestation and weighing 1480 g was delivered by cesarean section. There were no risk factors for sepsis. He presented with respiratory distress after birth, was shifted to the neonatal intensive care unit (NICU), was diagnosed as respiratory distress syndrome, and was managed as per standard protocol. Management: On the 6th day of life, the neonate developed pulmonary hemorrhage, multiple purpura on his upper and lower extremities, and shock. Raised D-dimer (>400 ng/ml), increased prothrombin and activated partial thromboplastin time, and thrombocytopenia (6000/μL) were indicative of DIC. The blood culture isolated Elizabethkingia meningoseptica. Meningitis was ruled out. Supportive care included fresh frozen plasma and platelet transfusion, antibiotics as per drug sensitivity, and granulocyte colony-stimulating factor. The baby improved and the lesions healed with scarring. Protein S and protein C deficiency was excluded on follow-up. On follow-up, at corrected age of 6 months, the baby was developmentally normal. Three additional cases were identified in the unit around the same time, however outbreak investigation could not identify origin of the pathogen. Conclusion: We could not find any earlier publications of neonatal PF due to E. meningoseptica septicemia. This organism is a cause of sepsis and meningitis in preterm babies and outbreaks in NICU settings. Early identification, meticulous assessment, and prompt specific antimicrobial treatment are important for survival.

Keywords: Disseminated intravascular coagulation, protein C, protein S, thrombohemorrhagic


How to cite this article:
Kumawat R, Kartikeswar GA, Parikh T. Neonatal Purpura Fulminans by an Unusual Pathogen: Elizabethkingia meningoseptica. Indian Pediatr Case Rep 2022;2:245-8

How to cite this URL:
Kumawat R, Kartikeswar GA, Parikh T. Neonatal Purpura Fulminans by an Unusual Pathogen: Elizabethkingia meningoseptica. Indian Pediatr Case Rep [serial online] 2022 [cited 2023 Jan 30];2:245-8. Available from: http://www.ipcares.org/text.asp?2022/2/4/245/362233

Neonatal purpura fulminans (PF) is a rare thrombohemorrhagic disorder associated with disseminated intravascular coagulation (DIC) in neonates and characterized by the formation of dermal microvascular thrombosis.[1] There are three types: neonatal (due to mutations of genes encoding for protein C or protein S), acute infectious, and idiopathic.[2] The underlying causes may be genetic or acquired. The latter are more common and often associated with acute fulminant sepsis caused by Gram-negative organisms, Staphylococcus species, Streptococcus, and Neisseria meningitidis.[2] This results in consumptive coagulopathy and transient relative deficiency of protein C and/or S.[1] Multiorgan involvement may occur due to thrombosis of both small and large vessels which leads to high mortality and morbidity.[3]

We report a case of neonatal PF with DIC due to Elizabethkingia meningoseptica infection. To the best of our knowledge, we report the first case of PF due to E. meningoseptica.


  Clinical Description Top


A preterm boy of 31-week gestation and weighing 1480 g, appropriate for gestational age (AGA), was delivered by cesarean section (in view of fetal distress). The mother was a 33-year-old multigravida with a previous live issue (a 3-year-old girl) and one abortion. The present pregnancy was by natural conception but had been complicated by pregnancy-induced hypertension and gestational diabetes from the 22nd week of gestation onward, for which she was on labetalol, oral acarbose, and insulin injections. The antenatal ultrasounds performed at 19 and 26 weeks were normal.

The mother received two doses of betamethasone (12 mg) 24 h apart, 2 days before delivery. There was no history of any risk factors for early-onset sepsis such as leaking per vaginum, maternal fever, and prolonged rupture of membranes. APGAR score was 7 and 8 at 1 and 5 min, respectively. The baby displayed signs of respiratory distress with a Silverman–Anderson score of 4/10. He was shifted to the neonatal intensive care unit (NICU) for initiating continuous positive airway pressure (CPAP) and preterm care.

At admission, the baby had a heart rate of 140/min with normal volume pulses, capillary filling time of <3 s, respiratory rate of 76/min associated with nasal flaring, upper chest and xiphoid retraction, and grunt audible with stethoscope. The settings of CPAP were set to maintain a target saturation of 90%–95%. Minimally invasive surfactant therapy technique was initiated at 30 min of life as the oxygen and pressure requirement persisted, and CPAP continued. After surfactant therapy, the condition of the baby gradually improved, with a decrease in oxygen requirement and CPAP pressure. At 6 h of life, the baby was on minimal CPAP support [21% FiO2 (fraction of inspired oxygen) and Positive end-expiratory pressure (PEEP) of 4], the baby had a heart rate of 136/min, capillary filling time <3 s, mean blood pressure was 34 (56/26) mmHg, and both femoral pulses were well felt. The abdomen was soft, with peristaltic sounds present on auscultation. The tone was normal for his gestational age. The baby weighed 1480 g (between the 10th and 50th centiles), his head circumference was 30 cm (between the 50th and 90th centiles), and his length was 41 cm (between the 10th and 50th centiles). The baby was diagnosed clinically as preterm, very low birth weight (VLBW) with respiratory distress syndrome.

Management and outcome

Empiric antibiotics were started after taking sepsis screen and blood culture. Minimal enteral feeds were initiated at 6 h of life and gradually graded up. At 30 h of life, the baby developed poor circulation and worsening respiratory distress with increased O2 requirement. He was intubated and mechanical ventilation started. Preliminary reports revealed hemoglobin – 16.5 gm%, total leukocyte count – 12300/mm3, platelet count – 2.3 lakh/mm3, and C-reactive protein (CRP) – 4.2 mg/L. Bedside echocardiogram screening revealed a 3-mm hemodynamically significant patent ductus arteriosus, which was treated with intravenous paracetamol. After initial improvement in perfusion, and decrease in ventilator requirement on days 3–5, the baby developed pulmonary hemorrhage on the 6th day of life, followed by shock. Multiple well-demarcated bluish patches (identified as purpura) were noted on both upper and lower limbs [Figure 1], which progressively increased over the next 24 h. The centers of the lesions became necrotic and multiple patches developed over the feet, legs, and hands, the largest one measuring 3.5 cm × 3 cm over the left foot.
Figure 1: Cutaneous necrosis involving foot

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A clinical diagnosis of PF was suspected. Pulmonary hemorrhage and shock were managed with ventilatory support, fresh frozen plasma (FFP) transfusion, injection Vitamin K, and dopamine infusion. A repeat sepsis screen showed fall in hemoglobin to 6.9 g/dl, leukopenia (total leukocyte count: 3020/μl) with neutropenia (absolute neutrophil count: 262/μl), thrombocytopenia (platelet count: 6000/μl), and increase in CRP (9 mg/L). Biomarkers for DIC were raised: D-dimer levels >400 ng/ml (normal <200 ng/ml) and prothrombin time (20.4 s, INR: 1.6) with activated partial thromboplastin time 56 s (reference range: 26–42). Fibrinogen levels could not be done due to logistic reasons. Ultrasonographic (USG) color Doppler of the large vessels of the upper and lower limbs showed no evidence of thrombosis. The USG of the cranium and abdomen for asplenia was normal. The blood culture grew E. meningoseptica that was sensitive to levofloxacin and vancomycin, and the antibiotics were modified accordingly. Colony-stimulating factor (CSF) analysis (27 cells with 90% lymphocytes, protein: 87 mg/dl, and sugar: 55 mg/dl) including sterile culture excluded meningitis.

The baby was given supportive management in the form of high-frequency oscillatory ventilation for the pulmonary hemorrhage, FFP, packed cell and platelet transfusion, and granulocyte-CSF injections (for neutropenia). The margins of the purpurae gradually showed well-defined demarcation over the next 3 days. Scab formation was seen over the following 7 days, with scar formation within 3 weeks. Antibiotics were continued for 14 days. The baby was discharged on full oral feeds at 36 days of life at a corrected gestational age of 36 weeks with a weight of 1680 g.

The baby was kept under close follow-up. The lesions had evolved into depigmented macules by 2 months of age [Figure 2]a and [Figure 2]b. We also got protein C and protein S levels, done, to rule out genetic causes of PF, which were normal. Galactosemia was ruled out as part of newborn screening. Three more cases of E. meningoseptica were identified during same time; as per standard protocol an out break investigation was carried out, however, the source of infection could not be localized. All the cases were preterm, VLBW neonates presenting with late-onset sepsis and neonatal seizures. Two of them had concurrent CSF culture-proven meningitis.
Figure 2: Healing with scar on follow-up (a) foot, (b) scar over wrist and hand

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  Discussion Top


Clinicians should keep a high index of suspicion of PF in young infants with purpuric and necrotic lesions. This condition may occur due to inherited mutations of the genes encoding protein C and/or protein S, galactosemia, or acquired causes such as severe sepsis, DIC, severe hepatic dysfunction, antiphospholipid antibodies, or warfarin therapy.[1] It is a hematological emergency in which skin necrosis and DIC may progress rapidly to multiorgan failure caused by thrombotic occlusion of small- and medium-sized blood vessels.[3] Management is based on clinical and laboratory findings. Supportive therapy with blood component therapy should be given to maintain the platelet count >50,000 × 109/L and the fibrinogen level >1 g/L. Specific management depends on the cause. In congenital protein C and S deficiency, the acute phase of replacement therapy should be followed by maintenance therapy and anticoagulation with warfarin. Severe infection is treated by appropriate intravenous antibiotics.[1] Early lesions are reversible with timely intervention, though established lesions often progress to full-thickness skin necrosis within 24–48 h and may require surgical debridement, fasciotomies, or even amputation.[3] PF is associated with more than 50% mortality rate in children and often major long-term morbidity like amputation of the limb, blindness, and cerebral palsy in those who survive, emphasizing the importance of early and aggressive treatment in such conditions.

There are a few case reports of PF due to Streptococcus, Acinetobacter, and Citrobacter sepsis.[2],[4] To the best of our knowledge, PF due to E. meningoseptica has not been reported earlier. E. meningoseptica formerly known as Chryseobacterium meningosepticum is a nonmotile, Gram-negative bacillus found mostly in soil and fresh water that causes severe infection in immunocompromised patients.[5] It causes neonatal meningitis, especially in premature infants during the 1st week of life. Other manifestations include bacteremia, pneumonia, cellulitis, septic arthritis, and urinary tract infection.[6],[7]

This organism has become a potential source of infections in the hospital environment. It can survive in chlorine-treated municipal water supplies, often colonizing sink basins and taps.[8] It has also been isolated from chlorhexidine gluconate solutions that are used in hospital wards for hand hygiene.[9] E. meningoseptica can present with skin manifestation apart from sepsis and meningitis in premature low birth weight infants in NICU. Ghafur et al. reported 29 cases of E. meningoseptica sepsis from a hospital in Chennai, India, out of which 11 cases were in immunocompromised hosts such as stem cell transplant and malignancy patients.[10] A recent review in 2017 included 283 published cases of E. meningoseptica infections in children and neonates, of which 35 cases were from India and more than three-quarters (215 out of 283 cases) were in neonates.[6] As E. meningoseptica can cause an outbreak of sepsis, can survive in water source for a long time, and is difficult to treat, a thorough outbreak investigation in NICU is important. In our case, we sent a culture of swabs from ventilator used, NICU incubator, suction bottle, air conditioner, tap water used for handwashing, distilled water used for humidifier, parenteral nutrition used for the patient, and mother's breast milk.

This organism may be a future threat in NICU due to its multidrug-resistant nature. We report this case to emphasize the importance of prompt diagnosis, early recognition of organism, specific antibacterial treatment, and outbreak investigation in a NICU setting.



Acknowledgment

All authors were thankful to the parent of the child for their cooperation and help of other doctors and nursing staff of KEM Hospital, Pune.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

 
  References Top

1.
Price VE, Ledingham DL, Krümpel A, et al. Diagnosis and management of neonatal purpura fulminans. Semin Fetal Neonatal Med 2011;16:318-22.  Back to cited text no. 1
    
2.
Choudhary SV, Dhande SS, Aghi T, et al. Neonatal purpura fulminans caused by rare Citrobacter species. Indian J Paediatric Dermatol 2018;19:164.  Back to cited text no. 2
    
3.
Chalmers E, Cooper P, Forman K, et al. Purpura fulminans: Recognition, diagnosis and management. Arch Dis Child 2011;96:1066-71.  Back to cited text no. 3
    
4.
Albarrak M, Al-Matary A. Neonatal purpura fulminans manifestation in early-onset group B Streptococcal infection. Am J Case Rep 2013;14:315-7.  Back to cited text no. 4
    
5.
Bloch KC, Nadarajah R, Jacobs R. Chryseobacterium meningosepticum: An emerging pathogen among immunocompromised adults. Report of 6 cases and literature review. Medicine (Baltimore) 1997;76:30-41.  Back to cited text no. 5
    
6.
Hoque SN, Graham J, Kaufmann ME, et al. Chryseobacterium (Flavobacterium) meningosepticum outbreak associated with colonization of water taps in a neonatal intensive care unit. J Hosp Infect 2001;47:188-92.  Back to cited text no. 6
    
7.
Dziuban EJ, Franks JL, So M, et al. Elizabethkingia in children: A comprehensive review of symptomatic cases reported from 1944 to 2017. Clin Infect Dis 2018;67:144-9.  Back to cited text no. 7
    
8.
Ceyhan M, Celik M. Elizabethkingia meningosepticum (Chryseobacterium meningosepticum) Infections in Children. Int J Pediatr 2011;2011:215237.  Back to cited text no. 8
    
9.
Coyle-Gilchrist MM, Crewe P, Roberts G. Flavobacterium meningosepticum in the hospital environment. J Clin Pathol 1976;29:824-6.  Back to cited text no. 9
    
10.
Ghafur A, Vidyalakshmi PR, Priyadarshini K, et al. Elizabethkingia meningoseptica bacteremia in immunocompromised hosts: The first case series from India. South Asian J Cancer 2013;2:211-5.  Back to cited text no. 10
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