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CASE REPORT
Year : 2018  |  Volume : 19  |  Issue : 2  |  Page : 164-166

Neonatal purpura fulminans caused by rare Citrobacter species


Department of Dermatology, Venerology and Leprosy, Acharya Vinoba Bhave Rural Hospital, Jawaharlal Nehru Medical College, Sawangi, Maharashtra, India

Date of Web Publication26-Mar-2018

Correspondence Address:
Sanjiv Vijay Choudhary
28-Modern Nagpur Society, Chattrapati Square, Nagpur - 440 015, Maharashtra
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijpd.IJPD_121_16

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  Abstract 


A 23-day-old neonate, born of nonconsangious marriage, admitted to Neonatal Intensive Care Unit for hypernatremic dehydration with petechiae and ecchymotic patches and necrotic skin lesions for 10–12 days was referred to dermatology department. On the general examination, pulse was 158/min, and respiratory rate was 52/min, and systemic examination was normal. Hematological investigations showed pancytopenia. Bleeding time was normal but prothrombin time and activated partial thromboplastin time reports were prolonged. D-dimer levels were elevated. Urine and stool were normal. Blood culture and sensitivity report revealed the growth of Citrobacter species with sensitivity to ciprofloxacin, amikacin, tetracycline, and resistance to Imipenem. Histopathology revealed epidermal hyperkeratosis with epidermal-dermal splitting, vessels showing fibrin occlusion with red blood cell extravasation into the perivascular areas in dermis along with dermal necrosis. To the best of our knowledge, this might be the first case of purpura fulminans in a neonate caused by rare Citrobacter species.

Keywords: Citrobacter species, neonate, purpura fulminans


How to cite this article:
Choudhary SV, Dhande SS, Aghi T, Mahajan P. Neonatal purpura fulminans caused by rare Citrobacter species. Indian J Paediatr Dermatol 2018;19:164-6

How to cite this URL:
Choudhary SV, Dhande SS, Aghi T, Mahajan P. Neonatal purpura fulminans caused by rare Citrobacter species. Indian J Paediatr Dermatol [serial online] 2018 [cited 2019 Dec 7];19:164-6. Available from: http://www.ijpd.in/text.asp?2018/19/2/164/206058




  Introduction Top


Purpura fulminans is a rare rapidly progressive disease caused by microvascular thrombosis of skin vessels manifesting as characteristic skin lesions.

Three different subtypes have been described: neonatal (congenital mutations in either protein C or S genes), idiopathic, and acute infectious.

Acute infectious purpura fulminans, the most common form of purpura fulminans, occurs superimposed on a bacterial infection. In this illness, the balance between anticoagulant and procoagulant endothelial cell activity is disturbed. This disturbance is precipitated by bacterial endotoxin and mediated by various factors, including the inflammatory cytokines interleukin (IL)-12, interferon gamma, tumor necrosis factor-α, and IL-1, which consume antithrombin III as well as proteins C and S.[1]

Pathogenesis of purpura fulminans includes disseminated intravascular coagulation (DIC) which leads to small vessels thrombosis and tissue necrosis.

Appropriately, 60%–70% cases of purpura fulminans have been reported among children below 2 years of age. The species commonly causing acute infectious purpura fulminans are Streptococcus pneumoniae, Group A and B streptococci, Staphylococcus aureus,  Neisseria More Details meningitidis. To the best of our knowledge, this might be the first case of neonatal purpura fulminans caused by rare Citrobacter species.

Treatment of purpura fulminans depends on subtype and may include antibiotic therapy, supportive treatment, and transfusions of fresh frozen plasma.


  Case Report Top


A 23-days-old neonate, born of nonconsangious marriage, admitted to Neonatal Intensive Care Unit (NICU) for hypernatremic dehydration was referred to Dermatology Department for skin lesions for 10–12 days. They were progressive in nature and were associated with a cough and cold. On general examination, pulse was 158/min, and respiratory rate was 52/min. Systemic examination was normal. Cutaneous examination revealed multiple erythematous, petechiae and ecchymotic patches over upper and lower extremities [Figure 1] and trunk. Single, circular, well-defined necrotic eschar of size approximately 3 cm × 4 cm with surrounding erythematous border over right shoulder and a necrotic patch over right great toe were present [Figure 2]a and [Figure 2]b.
Figure 1: Multiple erythematous petechiae and ecchymotic patches over both lower extremities

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Figure 2: (a) Single, circular, well-defined necrotic eschar of size approximately 3 cm × 4 cm with surrounding erythematous border over the right shoulder. (b) Necrotic patch over right great toe

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Hematological investigations showed pancytopenia (hemoglobin [Hb] - 4.6 g%, total leukocyte count - 19,400 cumm, platelet - 95,000/mm 3). Bleeding time (BT) was normal but prothrombin time (PT) and activated partial thromboplastin time (aPTT) reports were prolonged (PT - 15.6 s, APTT - 60 s in a term infant). D-dimer levels were elevate (1 mg/ml). Protein C level was normal (41 IU/dl). Serum creatinine was 2.62 mg%, and blood urea was 304 mg%. Serum electrolytes mainly sodium and potassium were 176 and 4.19 mEq/L, respectively. C-reactive protein was present. Urine and stool were normal. Lumbar puncture for cerebrospinal fluid report was normal. Blood culture and sensitivity report revealed the growth of Citrobacter species with sensitivity to ciprofloxacin, amikacin, tetracycline, and resistance to Imipenem.

Histopathology revealed epidermal hyperkeratosis with epidermal-dermal splitting, vessels showing fibrin occlusion with extravasation of red blood cell (RBC) into the perivascular areas in dermis, fibrin thrombin formation along with dermal necrosis [Figure 3]a-c].
Figure 3: (a) Epidermal hyperkeratosis with epidermal-dermal splitting, vessels showing fibrin occlusion with extravasation of red blood cell into the perivascular areas in dermis (H and E, ×10). (b) Epidermal-dermal splitting, extravasation of red blood cell into the perivascular areas (H and E, ×40). (c) fibrin thrombin formation (H and E, ×40)

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In treatment intravenous (IV) antibiotics were given. Injection ciprofloxacin 28 mg IV for 14 days, injection amikacin 7 mg IV OD for 14 days and injection metrogyl 10 mg IV 12 h for 10 days. Two platelet rich concentrates were also given. The adequate hydration was also given. The patient recovered completely after 2 weeks of NICU stay.


  Discussion Top


The close differential diagnosis in the present case was idiopathic thrombocytopenic purpura (ITP). In ITP, episodes of hematuria and malena might be present. Cutaneous examination might show petechiae and ecchymosis but no necrotic lesions. There is isolated thrombocytopenia with normal Hb and white blood cell count. Other laboratory investigations show prolonged BT with normal clotting time, aPTT, and PT.

In our case, cutaneous examination revealed multiple petechiae and ecchymotic patches with necrotic lesions and eschar formation. No episodes of malena or hematuria were noted. The patient had pancytopenia, normal BT but prolonged aPTT; PT. D-dimer test levels were elevated. Protein C level was normal.

Blood culture report revealed the growth of Citrobacter species, indicating infective pathology. Citrobacter species are straight, facultative anaerobic, Gram-negative bacilli. Citrobacter species are commonly found in water, soil, food, and the intestinal tracts of animals and humans. In a patient with Citrobacter infections, the bacteria can be transmitted vertically from mother or horizontally from carriers or other hospital sources.[2]

On histopathological examination, classical findings of epidermal keratosis with epidermal-dermal splitting, fibrin thrombin, and extravasation of RBCs with necrosis were seen. On the basis of clinical history, laboratory investigations which were similar to DIC and histopathological examination, we confirmed the diagnosis of purpura fulminans due to infective pathology.

Appropriately, 60%–70% cases of purpura fulminans have been reported among children below 2 years of age. Neonatal purpura fulminans has a high morbidity and mortality (approximately 43%). The species commonly causing purpura fulminans are S. pneumoniae, Group A and B streptococci, S. aureus, and N. meningitidis.

Till date, there is only one case report of purpura fulminans with septic shock due to bacterial translocation by Citrobacter by Hiromi et al. in a 50-year-old male patient.[3] To the best of our knowledge, this might be the first case of purpura fulminans in a neonate caused by rare Citrobacter species.

If an infant presents with purpuric and necrotic lesions, consider the possibility of congenital or infective etiology of purpura fulminans and investigate thoroughly by doing protein S and C levels, blood tests (D-dimer, aPTT, PT, complete blood count) and blood culture for early diagnosis and proper management of this fatal condition.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Madden RM, Gill JC, Marlar RA. Protein C and protein S levels in two patients with acquired purpura fulminans. Br J Haematol 1990;75:112-7.  Back to cited text no. 1
[PUBMED]    
2.
Doran TI. The role of Citrobacter in clinical disease of children: Review. Clin Infect Dis 1999;28:384-94.  Back to cited text no. 2
[PUBMED]    
3.
Hiromi S, Susuma T, Takashi S. A case of purpura fulminans with septic shock possibly due to bacterial translocation of Citrobacter freundii. Nishi Nihon Hifuka 2009;71:48-52.  Back to cited text no. 3
    


    Figures

  [Figure 1], [Figure 2], [Figure 3]



 

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