A 42 year old diabetic woman from North Western Province of Sri Lanka presented with abdominal pain, vomiting and intermittent fever for one month. She had recurrent skin abscesses 6 months before presentation which was treated with incision and drainage and a short course of antibiotics for which there was only partial improvement. Three months before presentation to us she was found to have multiple liver abscesses which were aspirated in a local hospital. The aspirate grew “coliforms” resistant to gentamicin and sensitive to ceftazidime. She was treated with cefuroxime, meropenem, co-amoxyclav and metronidazole; further identification of the organism was not done at the local hospital.\nOn presentation to us she had high fever and tender hepatomegaly. Her body weight was 40 kg. Rest of the examination including nervous system was normal.\nHer full blood count showed leukocytosis [15.7×109/L] and granulocytosis [11.3 × 109/L], ESR was 100 mm in the 1st hour and C-reactive protein was 98.4 mg/L. Ultra sound scan of abdomen showed multiple liver and splenic abscesses.\nPresence of recurrent multiple liver and splenic abscesses in a diabetic patient who had recurrent skin abscess in the past made us consider melioidosis in the differential diagnosis. Intravenous meropenem 2g eight hourly was started empirically and aspirate was sent with a sample of serum to Faculty of Medicine, University of Colombo which serves as the National Reference Laboratory, to confirm the diagnosis. There the aspirate yielded pinpoint colonies on blood, chocolate and Macconkey agar after overnight incubation. Since biochemical panels are not very accurate in diagnosis of Burkholderia pseudomallei, isolate was subjected to PCR which confirmed the diagnosis.\nSerum antibodies to melioidin antigen using an in-house indirect haemagglutination (IHA) test based on that described by Alexander et al. with antigen prepared from local strains of B.pseudomallei [] were positive at a titre of 1:10240.\nAfter confirmation of melioidosis intravenous meropenem was continued. However after a week of treatment the disease course was complicated by bilateral lower limb weakness which progressed over the next 4 days. Her lower limbs were flaccid and deep tendon reflexes were absent. There was no sensory sign or bladder/bowel involvement. Cerebro-spinal fluid analysis showed protein of 114.5 mg/dl, glucose 62 mg/dl, white cells <10 mm9/L and red cells < 10 mm9/L. MRI scan of the brain was normal. The nerve conduction study showed evidence of severe peripheral neuropathy with axonal degeneration; compatible with a primary axonal type inflammatory neuropathy such as acute motor axonal neuropathy or acute sensory motor axonal neuropathy.\nA diagnosis of Guillan-Barre syndrome was made based on Brighton case definition (Table ) () and she was treated with 4 cycles of plasmapharesis on alternate days for which there was marked improvement of neurological deficit.\nWith intravenous meropenem patient further improved and her ESR and CRP became normal. Repeat ultrasound scan of abdomen showed reduction of the size of liver and splenic abscesses.\nEradication therapy with cotrimoxazole 1920 mg 12 hourly and co-amoxyclav 625 mg 8 hourly was started on the seventh week and overlapped with intravenous antibiotic for further two weeks. Patient was discharged to outpatient clinic after two weeks of oral antibiotics with a plan to continue them for12 weeks. At the end of 12 weeks she had no residual neurological weakness and there was no sign of a relapse.
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.