SYMMETRIC PERIPHERAL GANGRENE IN A CASE OF FALCIPARUM MALARIA – A RARE PRESENTATION

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ABSTRACT

          Symmetric peripheral gangrene (SPG) is a rare clinical presentation in case of  falciparum malaria. Few cases have been so far reported from India and abroad. Here we report such a case from this hospital and discuss the pathophysiology and management.

KEY WORDS

Symmetric peripheral gangrene (SPG)

Falciparum malaria

Disseminated intravascular coagulation (DIC)

INTRODUCTION

          SPG is an uncommon clinical condition characterized by sudden onset of symmetric dry gangrene of acral distribution without any evidence of vasculitis or arterial obstruction. This has been described in conditions like infections most commonly bacterial (meningococcal, streptococcal, Pneumococcal, E.coli septicaemia) and viral (varicella and viral gastroenteritis), low output states like myocardial infarction, shock, CCF or use of drugs like vasopressin and ergot and other conditions like polymyalgia rheumatica,cryoglobinaemia. In all these conditions DIC has been the basic pathogenesis (90%)⁹.

           The incidence of DIC and SPG in cases of falciparum malaria has rarely been reported as one of its multiple complication¹.

CASE REPORT

          A 15 years boy from Raigarh (C.G.) admitted to this hospital in last week of December 2005 with history of fever associated with chill and rigor (20 days), sudden blackening  of distal parts of fingers, toes of all limbs , tip of nose and lateral part of pinna of both ears (15 days) and altered sensorium for 3 days. He was treated at the local hospital with I/V fluid, antibiotic and decadron , without any improvement.

          There was no history of joint pain, trauma, sore throat, spontaneous bleeding, thromboembolic episode or any relevant disease like SCD, HTN, DM. He was a student in a residential school without habit of smoking or alcohol. There was no relevant family history.

           On examination the patient was stuporous with Temperature 100⁰F , pulse – 100/min , regular low volume and all the peripheral pulsations well felt without brachiofemoral delay. BP was 110/70 mm of Hg (both U/L), with moderate pallor and features of dehydration. There was no icterus , clubbing, lymphadenopathy or thyromegaly. Cardiovascular and Respiratory system revealed no abnormality. There was no hepatosplenomegaly. Neurological examination revealed mild neck rigidity with diminished DTJ and plantar was flexor. Examination of skin and digits showed blackening of tip of nose (Fig.1), both ear pinna on lateral aspects (Fig 2) and distal parts of toes and fingers of all limbs (dry gangrene) (Fig 3 & 4). There was clear line of demarcation between the healthy and affected parts.

Investigation on the day of admission showed

Hb: 6.2 gm%

DC : N - 48, E-2, L-48, M-2, B-0

TLC: 9800/mm³

TPC: 60,000/mm³

RBS : 116mg%

B. Urea : 96mg%

Serum Creatinine : 1.5mg%

Blood smear : Showed P.falciparum rings and gametocytes

LFT :

Serum Bilirubin :              Total 0.78mg%

                                      Direct 0.26mg%

SGOT:                             39U/L

SGPT :                            36U/L

Alkaline phosphatase:       50U/L

Electrolytes:           Serum Na+ - 138meq/L

Serum K+ - 3.6meq/L

Serum Ca++ - 8.5 mg/dl

Prothrombin time (PT) – 16 second (control 12.5)

Activated partial thromboplastin time (aPTT) 29.5 seconds (control – 28)

Fibrinogen : 187mg/dl (N : 250-450mg/dl) 

          The patient was treated with injection Artesunate, injection Ceftriaxone  2gm I/V BID,injection Omnacortil, Injection Ranitidin 8 hourly, I/V fluid and tablet Zilast (Cilostazol 100mg BID) with care of the skin , bladder, bowel and maintaining nutrition. He was stabilised on 2^nd day and gained consciousness and there was clinical improvement. Two units of whole blood transfusion given.

          On further investigation urine analysis was normal and haemoglobin electrophoresis was AA (to exclude SCD – common in this belt). USG of abdomen and pelvis was normal. Test for antinuclear factor, LE cell, Rheumatoid factor, VDRL were negative, Test for cryoglobulin was normal. Coagulation profile (Antineutrophil cytoplasminc antibodies, Antinuclear antibodies, anti double standard DNA, complement –3 , complement – 4) was normal. Serum uric acid was 3.9 mg/dl.

          Echocardiography did not reveal any thrombi or vegetation.  The Doppler study of the vessels demonstrated normal flow pattern upto digital arteries in all four limbs.

          Biopsy of an affected area of the skin showed thrombi in dermal capillaries without any evidence of vasculitis.

          Blood could not be tested for fibrin degradation products (FDPs). However evidence of DIC was seen in skin biopsy.

          Gradually the patient improved. Full anti-malarial course was given and antibiotic was given for 7 days. Zilast was continued and omnacortil was gradually tapered. Slowly the gangrenous parts improved and patient was discharged on 12^th day. No surgical intervention was required.

DISCUSSION

SPG has been reported in various medical conditions including falciparum malaria^{1, 7, 4, 2, 6,12}. Our patient had no clinical or laboratory evidence of other causes like sepsis, vasospastic condition, ergot or other drugs. There was no evidence of vasculitis, cryoglobulinaemia, polycythemia or thrombocythaemia. The common pathogenic mechanisms of SPG is DIC⁹. All the cases reported had evidence of DIC.

          Reduced fibrinogen level, thrombocytopenia, prolonged PT, prolonged aPTT and histopathological evidence of microvascular thrombi indicate the presence of DIC in this patient¹.

          Alteration of coagulation and fibrinolytic system in falciparum malaria is well recognized⁵. A functionally active but controlled coagulatory state exists in falciparum malaria even in uncomplicated cases⁵. Elevation of FDPs reflecting the ongoing fibrinolysis have been  documented¹¹. Heavy parasitaemia triggering the coagulation pathway¹⁰, alteration  in the lipid distribution across the surface membrane of the parasitized  erythrocytes activating the intrinsic coagulation cascade⁸ and activation of complement system⁵ have been postulated as possible mechanism for DIC in falciparum malaria. Sequestration of the parasitised erythrocytes in the microcirculation by molecular interactions with endothelial receptors, mainly intracellular adhesion molecule¹⁰ – 1(ICAM – 1) may occur. Rosetting of the healthy erythrocytes around parasitised red cells may occur and these multicellular aggregates further exacerbate the vascular obstruction caused by sequestration³.

          DIC is encountered in less than 10% of patients with cerebral malaria³ and manifest as spontaneous bleeding from gum and GIT. But in a review of 71 cases of SPG and DIC significant bleeding complications were not recorded⁹.

          Our patient who had no other cause of peripheral gangrene made satisfactory  recovery on specific antimalarial therapy supporting the observation that falciparum malaria was the triggering mechanism for the DIC and subsequent SPG.

CONCLUSION

In conclusion we report that falciparum malaria may present as peripheral dry gangrene and this possibility though uncommon must be taken into consideration while encountering patients in endemic areas 

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