Boundaries of a systemic disease: a protean presentation of giant cell arteritis.

A 60-year-old man was hospitalised with persistent fever, arm pain, dry cough and cholestasis. Diagnostic workup was remarkable for elevated inflammatory markers. Infectious diseases and autoimmune screening were negative. Imaging modalities excluded a neoplastic aetiology. Liver biopsy was negative for granulomatous or lymphomatous infiltrations. Giant cell arteritis (GCA) was suspected, but temporal artery Doppler ultrasound and biopsy were non-diagnostic. A positron emission tomography scan showed intense metabolic uptake in large vessels suggesting the diagnosis of GCA. Prednisolone was initiated with clinical and analytical improvement. At 1-year follow-up, there were no relapses and the patient remains symptom free.

Massive organ embolization from primary aortic thrombosis.

A 49-year-old woman was hospitalized for acute left foot arterial ischemia. Arterial Doppler revealed occlusion of the dorsalis pedis and posterior tibial arteries. A computed tomography angiography performed to assess abdominal pain showed hepatic, splenic, renal and pancreatic infarctions. A splenic artery embolism and a small aortic wall thrombus at the celiac trunk were identified. No radiological signs of aortic atherosclerosis were found. No predisposing conditions for secondary aortic thrombosis or intracardiac embolic sources were detected. It was determined that primary aortic thrombosis, a rare though potentially serious condition, was to blame. Isolated aortic mural thrombosis therapy is not well established, although systemic anticoagulation, thrombolysis, thromboaspiration, endovascular stent grafting and surgical thrombectomy have been attempted with varying success. In our patient, systemic anticoagulation therapy was initiated and resulted in aortic thrombus resolution. Close clinical follow-up is crucial, as the aortic thrombus can recur despite anticoagulation and aggressive control of the atherosclerotic risk factors.

Erythema induratum of Bazin and Ponçet's arthropathy as epiphenomena of hepatic tuberculosis.

A 42-year-old black woman presented with fever, polyarthritis, livedo reticularis, subcutaneous calf nodules and hepatomegaly. She had been diagnosed with depression 6 weeks prior. Blood analysis showed anaemia, elevated erythrocyte sedimentation rate and C reactive protein, elevated liver enzymes, and positive antinuclear and antiribonucleoprotein antibodies. Abdominal ultrasound revealed heterogeneous hepatomegaly with necrotic lymphadenopathy around the caeliac trunk and splenic hilum. We considered the following diagnoses: lymphoma, connective tissue disease, tuberculosis and sarcoidosis. Subcutaneous nodule histology was compatible with erythema induratum of Bazin, and liver biopsy evidenced granulomatous hepatitis. Although microbiological examinations were negative in tissue samples, a presumptive diagnosis of hepatic tuberculosis was admitted. Having excluded other causes, erythema of Bazin, livedo reticularis and polyarticular involvement (Ponçet's arthropathy) were accepted as immunological epiphenomena associated with tuberculosis. Empirical antituberculous treatment was started and after 3 weeks the patient improved substantially. This clinical response was a further confirmation of the diagnosis.

A cerebral abscess at first internist glance.

A 73-year-old man was transferred to the neurosurgery ward, with a presumptive diagnosis of cerebral abscess. The case was also discussed with the internists as internal medicine consultants. The neurosurgeons pointed out a right temporal lobe abscedated lesion on CT, but we noticed that the hypodense attenuation that usually surrounds the abscess wall (vasogenic oedema) extended to a broader, well-delimitated area, suggesting medium cerebral artery territory. The patient had left-sided hemiplegia with a confusional state and low-grade fever. Considering possible haematogenous dissemination, an echocardiography was performed, confirming mitral endocarditis. Blood cultures and aspirated pus isolated Escherichia coli. Investigating the patient's medical history, we learned he had been submitted to bladder catheterisation 7 weeks before for acute urinary retention due to benign prostatic hyperplasia, and empirically medicated for urinary tract infection. E. coli had also been isolated in a urine specimen at the time. The clinical history of the patient cancelled the pathogenesis of cerebral abscess.

Nodular mucinosis misdiagnosed as non-responsive secondary syphilis.

A previously healthy 24-year-old man presented with an erythematous, non-pruritic and painless papulonodular skin rash affecting the trunk, upper arms (excluding palms), neck, face, forehead and scalp. He had a penile ulcer for the past 2 weeks, almost healed at the time of observation. The patient tested positive for syphilis and HIV-1; he claimed being negative for HIV 6 months earlier. As the palms were not affected, we performed a skin biopsy for the differential diagnosis between secondary lues and acute HIV seroconversion reaction. Benzathine penicillin (2,400,000 units) was administrated and antiretroviral therapy started. Although the skin biopsy was compatible with secondary syphilis, there was no change in the skin rash 3 weeks after the first penicillin administration. Another 2 doses of penicillin were given but 4 weeks later the rash persisted. A second biopsy revealed a mucinous skin infiltration, compatible with nodular mucinosis.

A chest wall pulsating mass.

A 34-year-old HIV-positive man with intravenous drug addiction presented with a 2-week history of fever and a pulsatile presternal mass; 2 weeks prior he had suffered a traumatic sternal fracture. The CT scan showed a bulky abscess with presternal and retrosternal extension that contacted the heart (which explained its pulsatile effect) and pulmonary necrotising lesions. Drainage of the thoracic wall abscess was performed, and antibiotics (flucloxacillin plus gentamicin) were started on the presumption of staphylococcal bacteraemia given the patient's intravenous drug habit. Blood cultures and culture of the aspirated pus confirmed growth of methicillin-sensitive Staphylococcus aureus; transoesophageal echocardiography showed endocarditis of the tricuspid valve. We therefore assumed pulmonary septic embolisation from right-sided endocarditis and acute sternal osteomyelitis, and the patient was treated for 6 weeks with clinical resolution.

Sustained complete remission of primary effusion lymphoma with adjunctive ganciclovir treatment in an HIV-positive patient.

Primary effusion lymphoma (PEL) is one of the least common of the AIDS-related lymphomas, accounting for less than 1-4% of cases. Clinical manifestations depend on the extent and distribution of disease and, as in the majority of patients no detectable mass lesion is found, symptoms are related to fluid accumulation, dyspnoea (pleural or pericardial effusions), abdominal distension (ascites) or joint swelling. The median survival after diagnosis, even with aggressive chemotherapy, remains poor and remissions are often of short duration. We present the case of a 31-year-old man with AIDS and diagnosis of PEL, in whom sustained and complete remission of the tumour was achieved with adjunctive ganciclovir therapy. Since the disease is so uncommon, there is a paucity of data to guide the treatment of these patients; ganciclovir might be a potential antiviral therapeutic option, as demonstrated by the 2-year remission achieved in our patient.

An uncommon case of hepatopulmonary amoebiasis.

Amoebiasis is an uncommon infection in developed countries caused by the protozoan Entamoeba histolytica. Amoebic liver abscess is the most frequent extraintestinal presentation of the disease; pleuropulmonary involvement is rare, occurring mostly by rupture of the abscess into the pleural space or lung parenchyma. We describe a case of a 48-year-old migrant from São Tomé e Príncipe, with fever, wasting, dry cough and right upper abdominal pain for the past 2 months. The CT scan revealed a voluminous liver abscess with thrombosis of the right suprahepatic and inferior vena cava, right pulmonary lobar abscess and multiple diffuse condensations in both lungs. Aspirated pus resembled anchovy sauce; blood and aspirated material cultures for infectious agents were negative. Serology for E. histolytica was positive, and the diagnosis of hepatopulmonary amoebiasis with infectious phlebitis was confirmed by positive PCR in the liver pus. Treatment with metronidazole+paramomycin led to clinical and radiological resolution.

Pulmonary oedema in the emergency room: what is hidden beyond an apparently common presentation.

Cardiogenic flash pulmonary oedema is a common and potentially fatal cause of acute respiratory distress. Although it often results from acute decompensated heart failure, abrupt-onset aortic regurgitation can sharply rise cardiac filling pressure and, consequently, pulmonary venous pressure, leading to rapid fluid accumulation in the interstitial and alveolar spaces. We report a case of a 64-year-old woman admitted to the emergency department with a flash pulmonary oedema; a careful clinical investigation subsequently revealed a rare aetiology for this 'common' presentation. After a detailed auscultation that unmasked a diastolic cardiac murmur, an acute severe aortic insufficiency was further confirmed by echocardiography, showing inflammation and thickening of the entire aorta wall. The patient was submitted to valve replacement surgery, and histological examination, to our surprise, showed features of aortitis, remarkable for the presence of giant cells. A diagnosis of idiopathic aortitis versus inaugural giant cell arteritis was proposed and treatment started with corticosteroids.