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 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. ).