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Purpura fulminans secondary to rickettsia in a child: a case report.

Purpura fulminans (PF) is a life-threatening hematologic emergency in which there is skin necrosis and disseminated intravascular coagulation (DIC). It was first described by Guelliot in 1884. There is a tissue necrosis, small vessel thrombosis, and DIC. PF may be categorized as: PF due to inherited abnormalities of protein C or other coagulation systems, acute infectious PF, and idiopathic PF (1). There have been many case reports both in adults as well as in pediatric patients with life-threatening fatal outcomes. Here, we report a case of PF in a child secondary to rickettsial infection, who was managed efficiently with timely intervention.

Case report

Eighteen month old female child presented to our outpatient department with 3-days history of low grade intermittent fever, and two episodes of non-bilious, blood stained, projectile vomiting. At the time of admission she developed generalized tonic clonic convulsions which lasted for 2 min followed by post-ictal drowsiness. On examination, the glasgow coma scale was 5/15 and she was in septic shock. Petechial and ecchymotic rashes were seen on the limbs and trunk. There was hepatosplenomegaly. Over the following days, the child developed mottled skin pigmentation and diffused purpuric rashes. She developed symmetrical patchy skin necrosis on both upper and lower limbs and also gangrene of tips of three fingers, tip of the nose and helical borders of both ears. (Figs. 1-4).

On investigation, complete blood count was unremarkable initially, except for hemoglobin 6 g%. But on the following days her platelet count decreased to 22,000/[mm.sup.3]. Widal test, blood and urine culture, X-ray chest and peripheral smear were done to find out the cause of fever and were found negative. Culture of the skin swab showed no growth. So, Weil-Felix (WF) test was done and was found positive for OX19 with a titre of >1 : 160 titre. The routine urine test, the liver function tests, the renal function tests and the serum electrolytes were normal except for hyperkalemia of 7 meq/l. Acid blood gas analysis was normal. Based on the above clinical findings and investigations, a diagnosis of acute infectious type of PF secondary to rickettsia was made. Due to financial constraints, confirmatory tests for rickettsia and its speciation, and other investigations for the hypercoagulable states, like proteins C and S, and fibrinogen degradation products were not done.

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The child was managed with immediate heparinization, and infusion of fresh frozen plasma (15 ml/kg every 12 h) along with the resuscitation measures for septic shock. Multiple units of platelets were transfused. Initially was started on crystalline penicillin, and dexamethasone injections. Later on she was put on azithromycin and meropenem. She was also given low molecular weight dextran intravenously.

Over next two weeks, her general condition improved, blood parameters returned to normal, skin lesions healed with hypertrophic scars and there was autoamputation of the gangrenous parts of the digits, tip of the nose and helical borders of both ears.

DISCUSSION

Neonatal PF is the most common form which is due to inherited or acquired abnormalities of the proteins C and S anticoagulant pathways. They usually manifest within first 72 h of birth. Idiopathic PF follows a bacterial or viral illness (2) and usually begins 7-10 days after the onset of the infection. The pathogenesis involves acute transient decreases in proteins C and S, or antithrombin III levels.

The most common form, acute infectious PF, occurs superimposed on a bacterial infection. The bacterial endotoxins consume antithrombin III as well as proteins C and S, thus, disturbing the anticoagulant and procoagulant endothelial cell activity (3). Meningococci (4), group A streptococci (5), Pneumococci (6) and Varicella are the various bacterial and viral pathogens implicated. The present case which we have reported is the acute infectious form of PF secondary to rickettsia infection.

Irrespective of the cause of PF, its cardinal manifestations are presence of circumscribed ecchymosis of skin and symmetrical gangrene of the extremities with coagulation abnormalities suggestive of disseminated intravascular coagulation (7). Typical hematological findings include low concentrations of fibrinogen, clotting factors and platelets, and prolonged prothrombin and partial thromboplastin times (8). Fibrinogen degradation products tend to be raised and concentrations of proteins C, S, and anti-thrombin III reduced (9).

Rickettsial diseases are some of the most covert reemerging infections and are prevalent throughout the world. They are vector borne diseases, vectors being ticks, fleas and louse. They commonly affect the travelers to endemic areas. In India, they are reported from Maharashtra, Tamil Nadu, Karnataka, Kerala, Jammu and Kashmir, Uttarakhand, Himachal Pradesh, Rajasthan, Assam and West Bengal (10). These infections present with vague signs and symptoms mimicking viral infections, thus making diagnosis difficult.

Various serological tests that can be done to diagnose rickettsial infections are microimmunoflorescence, immunoperoxidase assay, latex agglutination, indirect hemagglutination, enzyme-linked immunosorbent assay, dot blot immunoassay (including dipstick test) and Weil-Felix test.

Of these, only WF test is easily available in India. As all these tests detect antibodies, they would be able to make diagnosis only after 5-7 days of onset of disease (10). The sharing of antigens between rickettsia and proteus is the basis of this heterophile antibody test. It demonstrates agglutinins to Proteus vulgaris strain OX 19, OX 2 and OX K. The poor sensitivity of the WF test is now well demonstrated but a good correlation between the results of the WF test and detection of IgM antibodies by an indirect immunofluorescence assay (IFA) is often observed (11). This can be used as a screening test, when positive, is reasonably specific. Thus, this test can replace the more definitive investigations to diagnose rickettsial infections. In our patient, WF test was positive to OX19 antigen, and since other causes of fever were ruled out, and based on clinical signs and symptoms, we made a diagnosis of acute infectious PF secondary to rickettsia.

Irrespective of the type and etiology, management includes supportive therapy with aggressive broad spectrum antibiotics, resuscitation with fluids and rectification of acid base and electrolyte imbalance. Fresh frozen plasma must be given 10-20 ml/kg every 8-12 h in order to replace the procoagulant and anticoagulant proteins consumed in the disease process. Platelets and cryoprecipitates must be transfused to manage thrombocytopenia and hypofibrinogenemia respectively. Anticoagulation with heparin is important to manage large vessel venous thrombosis. Prompt excision of necrotic tissue is recommended and escharotomies and amputations may be indicated (12). Multidisciplinary involvement may be required to facilitate rehabilitation.

Correct diagnosis, timely intervention and appropriate management are important in PF in order to prevent morbidity and mortality associated with this condition.

Key words Ischemic necrosis; purpura fulminans; rickettsia

ACKNOWLEDGEMENT

We thank the parents of the child in this case report for consent and cooperation

REFERENCES

(1.) Adcock DM, Bronza JP, Marlar RA. Proposed classification and pathologic mechanism of purpura fulminans and skin necrosis. Semin Thromb Hemost 1990; 16: 333-10.

(2.) Brown DL, Greenhalgh DG, Warden GD. Purpura fulminans and transient protein C and S deficiency. Arch Dermatol 1998; 124: 119-23.

(3.) 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.

(4.) Rosenstein N, Perkins BA, Stephens DS, Popovic T, Hughes JM. Meningococcal disease. N Engl J Med 2001; 344: 1378-88.

(5.) Dhodapkar K, Corbacioglu S, Chang MW, Karpatkin M, DiMichelle D. Purpura fulminans caused by group A beta-hemolytic Streptococcus sepsis. J Pediatr 2000; 137: 562-7.

(6.) Noguera A, Fortuny C, Pons M, Cambra F, Palomeque A. Pneumococcal-associated purpura fulminans in a healthy infant. Pediatr Emerg Care 2004; 20: 528-30.

(7.) Hautekeete ML, Berneman ZN, Bieger R, Stevens WJ, Bridts C, Buyssens N, et al. Purpura fulminans in pneumococcal sepsis. Arch Intern Med 1986; 146: 497-9.

(8.) Nolan J, Sinclair R. Review of management of purpura fulminans and two case reports. Br J Anaesth 2001; 86: 581-6.

(9.) Smith OP, White B. Infectious purpura fulminans: Diagnosis and treatment. Br J Haematol 1999; 104: 202-7

(10.) Rathi N, Rathi A. Rickettsial infections: Indian perspective. Indian Pediatr 2010; 47(2): 157-64.

(11.) La Scola B, Raoult D. Laboratory diagnosis of rickettsioses: Current approaches to diagnosis of old and new rickettsial diseases. J Clin Microbiol 1997; 35: 2715-27.

(12.) Herrera R, Hobar PC, Ginsburg CM. Surgical intervention for the complications of meningococcal-induced purpura fulminans. Pediatr Infect Dis J 1994; 13: 734-7.

Received: 18 January 2014

Accepted in revised form: 30 April 2014

Leelavathy Budamakuntla, Eswari Loganathan, Sarala Sabapathy & Parul Thakur

Bowring and Lady Curzon Hospital and Research Institute, Bangalore Medical College and Research Institute, Bengaluru, India

Correspondence to: Dr B. Leelavathy, No. 21, Sri Mookambika, IV Cross, 1st Stage (Opposite IDIOM), Michael Palya, Indira Nagar,

Bengaluru-560 038, India.

E-mail: drleelaskincare@rediffmail.com
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Title Annotation:Case Reports
Author:Budamakuntla, Leelavathy; Loganathan, Eswari; Sabapathy, Sarala; Thakur, Parul
Publication:Journal of Vector Borne Diseases
Article Type:Clinical report
Date:Sep 1, 2014
Words:1446
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