A 42-year-old Sri Lankan man presented to the Colombo North Teaching Hospital, Ragama, Sri Lanka (CNTH) with a febrile illness of 6 days, accompanied by headache and constitutional symptoms. He was a grocer from Minuwangoda, a suburban area in the Western Province situated 44 km from Colombo. He was initially investigated and treated at a regional hospital for 4 days, and was transferred to the CNTH for specialized care. He gave a history of type 2 diabetes mellitus for 5 years without microvascular or macrovascular complications, and was a nonsmoker and a teetotaler. There were no specific symptoms suggesting a source of infection such as cough, abdominal pain, urinary symptoms, etc. He was febrile with a temperature of 39.10 C, and examination of the heart, lungs and abdomen was unremarkable. There was no papilloedema, focal neurological signs, pyramidal signs or neck stiffness. Initial laboratory work up revealed features of a bacterial infection, with neutrophil leukocytosis and elevated inflammatory markers (erythrocyte sedimentation rate – 101 mm/1st hour, C-reactive protein - 220 mg/dl). Initial blood cultures done at the regional hospital had yielded an isolate, which was reported as a Pseudomonas species; this was sensitive to ceftazidime, imipenem and meropenem and resistant to gentamicin and ceftriaxone. Other basic laboratory investigations including renal and liver function tests, electrolyte panel and urinalysis were normal. Chest x-ray and ultrasound scan of the abdomen were normal, and the trans-thoracic 2-D echo did not show any vegetations. As the unusual antibiotic sensitivity pattern suggested the possibility of melioidosis, blood was sent for serological testing to a specialized Melioidosis Research Laboratory at the Faculty of Medicine, University of Colombo.\nHe had been initially treated with intravenous ceftriaxone, and later with ceftazidime according to the antibiotic sensitivity pattern. Although the frequency and intensity of fever spikes reduced with treatment, he continued to have low grade fever and complain of anorexia, malaise and lethargy. On the 4th day after admission to the CNTH (day 10 of the illness), he developed simple partial seizures involving the left lower limb, progressing to persistent numbness of the left side of the body. An urgent CT scan of the head revealed a subdural collection over the right fronto-parietal region with gas locules and obliteration of sulci and gyri, without definite evidence of abscess formation (Fig. ). Contrast enhanced MRI scan of the brain demonstrated a subdural collection in the right fronto-temporo-parietal region with possible abscess formation in the right parietal region (Fig. ). Seizures were treated with oral sodium valproate and phenytoin sodium. He was referred for neurosurgical opinion, and the subdural collection was managed conservatively. Results of the indirect hemagglutination assay (IHA) for melioidosis antibodies were received on the following day; an antibody titre of more than 1/10,240 was strongly suggestive of an acute infection with Burkhoderia pseudomallei and a diagnosis of cerebral melioidosis was made.\nAntibiotics were changed to intravenous meropenem and oral trimethoprim/sulfamethoxazole (Co-trimoxazole/TMP-SMX). Initial intensive therapy with these antibiotics was continued for 8 weeks, until clinical improvement was evident with resolution of inflammatory markers and radiological improvement confirmed by repeat MRI scan of the brain. (Fig. ) Repeat blood cultures were sterile after 2 weeks of treatment with antibiotics. There were no further seizures, and the fever and the neurological symptoms resolved completely. He was discharged home with oral TMP-SMX and doxycycline, which were continued for 6 months, and the antiepileptics were gradually tailed off. At 6 months follow up he was asymptomatic. (Fig. ).
A 26-year-old female with B. cenocepecia presented with a history of recurrent small joint pains in association with intermittent painful erythematous nodular rash on the lower legs. This came on at the onset of an infective exacerbation of her chest. Biopsy confirmed erythema nodosum. Other causes of erythema nodosum were excluded, and she did not respond to Non-steroidal anti-inflammatory medications. Her symptoms continue to relapse in association with infective exacerbations of her chest.\nOf the remaining patients (n = 3), one presented with an erythematous rash on the lower legs and another had a purpuric rash on the lower legs and both were diagnosed clinically as leucocytoclastic vasculitis by a dermatologist. The third patient presented with purpuric areas and blistering of the lower legs which was confirmed as leucocytoclastic vasculitis on biopsy.\nOverall, two patients had biopsy proven leucocytoclastic vasculitis, three were clinically diagnosed with vasculitis but did not have biopsy confirmation and two had biopsies, which revealed other pathologies (erythema nodosum and capillaritis), giving an overall prevalence of cutaneous vasculitis in patients with Burkholderia cepacia complex of 16.7%. Out of the five patients with vasculitis, three had a raised ESR/CRP ratio.\nThere was no evidence of renal involvement in any patient – all had a negative urine dipstick and normal serum creatinine. The antinuclear antibody and antineutrophil cytoplasmic antibody were negative in all patients apart from one with biopsy proven leucocytoclastic vasculitis who had a transiently positive antineutrophil cytoplasmic antibody. Antistreptolysin O titre was tested in only two out of the five patients and was negative.\nMean forced expiratory volume1 was 30% (range: 18–55) of the predicted value. In four patients (57%), the forced expiratory volume1 was lower at the time of presentation with rash compared to the baseline forced expiratory volume1 values for the previous two years.\nAll patients received antibiotics for a presumed pulmonary exacerbation at onset of the rash. Three patients received specific treatment for vasculitis (prednisolone ± additional immunosuppressive) after non-resolution of the rash.