We present the case of a 15-year-old female heterozygous for ΔF508del/N1303K, with poorly controlled lung disease determined by forced expiratory volume in one second (FEV1) 44% of predicted value, who presented with cutaneous vasculitis and arthritis in association with an infective exacerbation of CF. The cutaneous involvement consisted of a palpable purpuric rash distributed over the tibial surfaces, ankles, and dorsa of the feet (Figure ). At this admission in 2015, infection and colonization with BCC and methicillin-resistant Staphylococcus aureus (MRSA) later in the year were also diagnosed. Blood work at admission is presented in Table ; acute phase reactants (C-reactive protein and fibrinogen) and rheumatoid factor were elevated. ANCAs were negative. Antinuclear antibodies (ANAs) were negative initially, but became positive five years later. Over the next several years, the patient required on average three to four hospitalizations every year due to IECF. Cutaneous vasculitis was present in approximately every other episode of IECF and improved with treatment of the pulmonary exacerbations. Bacterial colonization together with respiratory function is summarized in Figure - of note, the FEV1 decreased from 65% at 14 years of age (before appearance of the vasculitis) to 24% by 19 years of age.\nFrom the patient’s medical history, we consider eloquent the following facts: at the age of two years, she received nine-month isoniazid prophylaxis due to contact with her father who had tuberculosis. At the age of three years, she was diagnosed with bronchiectasis via CT. At the age of nine years, colonization with Pseudomonas aeruginosa was diagnosed through bronchoalveolar lavage and she had an FEV1 of 75% predicted. At the age of 13 years, the patient was diagnosed with a latent pulmonary tuberculosis infection which was again treated with nine months of isoniazid monotherapy. At the age of 14 years, she was diagnosed with allergic bronchopulmonary aspergillosis, via elevated total IgE and Aspergillus-specific IgE. At this time, the patient had an FEV1 of 65% predicted.\nAt the age of 15 years (2015), colonization with BCC was noted, she had an FEV1 of 44% predicted. At this time, the patient showed first signs of the cutaneous vasculitis, in form of a palpable purpuric, nonvesicular maculopapular rash localized to the lower legs. The lower extremities were slightly swollen, painful, and as the episodes of the vasculitis continued to erupt, grey discoloration in the place of the rash began to set in and over the years became permanent. At the age of 18 years (2018), colonization with Achromobacter xylosoxidans and Candida parapsilosis was noted, she had an FEV1 of 24% during the exacerbation, which improved to 38% of predicted after treatment. The chest x-ray comparison from 2015 to 2017 is presented in Figure and B, respectively. The CT scan done at this time showed cystic dilations in the right apex (Figure ) and severe bilateral middle lung field cylindrical bronchiectasis with signet-ring signs (Figure ).\nTreatment was started with long-term oral prednisone. Colonization with Pseudomonas, BCC, MRSA, Achromobacter xylosoxidans, and Candida parapsilosis was treated with multiple courses of inhaled, IV and oral antibiotics (linezolid, ceftazidime, ofloxacin) and antifungals (fluconazole and voriconasole). In addition, the following was administered: azithromycin (three days a week for three months), tiotropium, an antihistamine, and periodic cough assist with antibiotic aerosol treatment. Starting from 2019, the patient’s nebulization with colistin and tobramycin was supplemented with nightly positive-pressure ventilation (PPV), but the ventilation was discontinued six months later due to slight hemoptysis that was predominantly present in the morning. Throughout the six months, the patient was on PPV, she experienced twp IECF, which required hospitalization. The two episodes of IECF, together with the extensive bronchiectasis, may have contributed to the appearance of the hemoptysis, which may have predisposed certain areas of the lung to barotrauma. After the PPV was discontinued, the hemoptysis disappeared and did not require any more treatment.
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.