The clinical triad of peripheral cutaneous erythematous nodules, oligo or polyarthritis with intraosseous fat necrosis in the setting of pancreatic disease defines a rare entity called pancreatitis-panniculitis-polyarthritis syndrome. The early recognition of this triad is critical due to its high mortality rate and the rapid onset of osseous and articular disabilities. We describe the clinical course of a 54-year-old patient with complaints of weight loss and fever who presented to our hospital with signs of polyarthritis and appendicular erythematous cutaneous nodules. Clinical investigation revealed high inflammatory and pancreatic enzymes levels. Cutaneous biopsy and articular MRI showed evidence of peripheral necrosis. After a thorough investigation, a diagnosis of panniculitis-polyarthritis-pancreatitis syndrome was established. Treatment was initiated, and a slow but steady improvement was observed. Further complications of the disease process were observed. This case highlights the importance of recognising the association between panniculitis and polyarthritis with pancreatic disease in order to improve outcomes.
RESUMOO cancro de primário desconhecido é responsável por cerca de 3-5% de todas as neoplasias malignas e é, portanto, um dos 10 diagnósticos de cancro mais frequentes. A tecnologia de imagem moderna e o estudo anatomopatológico desenvolvido (imuno-histoquímica, microscopia eletrónica e diagnóstico molecular) resultaram na melhoria do diagnóstico; no entanto, os primários permanecem ocultos na maioria dos doentes, mesmo pós-autópsia. Os locais mais comuns de origem são os tumores do pulmão e pâncreas. O cancro de origem desconhecida é diagnosticado num estadio metastático, conferindo um prognóstico desfavorável. O fíga-do é um dos órgãos mais comumente envolvido na doença metastática, que surge com mais frequência de primários no cólon. A colonoscopia é o gold standard para avaliação da mucosa do cólon e reto. É apresentado um doente de 64 anos, sem história médica de relevo, que recorreu ao serviço de urgência por dor no flanco direito. A ecografia abdominal revelou metastização hepática difusa. A tomografia computadorizada referia suspeita de espessamento do cólon ascendente mas a endoscopia e colonoscopia (com uma boa visibilidade da válvula ileocecal) não tinham alterações. Palavras-chave: Colonoscopia; cancro colorretal; metástases hepáticas; cancro de primário desconhecido. ABSTRACTMetastatic Cancer of Unknown Primary Site (CUP) accounts for approximately 3-5% of all malignant neoplasms and is therefore one of the 10 most frequent cancer diagnoses. Modern imaging technology and extensive work-up with specific pathology investigations (immunohistochemistry, electron microscopy and molecular diagnosis) have resulted in some improvements in diagnosis; however, the primary site remains unknown in most patients, even on autopsy. The most prevalent sites of primary tumors origin are the lung and pancreas. CUP is diagnosed at a metastatic stage, conferring an unfavorable prognosis. The liver is one of the most common organs to be involved with metastatic disease, which arises most frequently from primary sites in the colon. A total colonoscopy is the gold standard for the evaluation of the colon. We present a 64-year--old man with no remarkable past medical history, which presented to the Emergency Service with right flank pain. The abdominal ultrasound revealed diffuse lesions suggestive of hepatic metastases. A computerized tomography (CT) scan showed thickening of ascending colon, but upper and lower endoscopies (with a good view of the ileocecal valve) and other diagnostic procedures were unremarkable.
ResumoOs Enterococos pertencem à flora comensal humana e têm sido reconhecidos como importantes agentes causadores de infeções. As infeções causadas por estes microrganismos têm vindo a aumentar, especialmente entre os idosos, doentes com doenças cardíacas degenerativas e válvulas cardíacas protésicas, bem como em manipulações dos tratos gastrointestinal e urinário. São responsáveis por cerca de 8-17% AbstractEnterococci belong to the commensal human flora and have been recognized as important causative agents of infections. The infections caused by these organisms have been increasing, particularly among the elderly, patients with degenerative heart disease and cardiac prosthetic valves, as well as manipulation of the gastrointestinal and urinary tracts. They are responsible for about 8-17% of infectious endocarditis, being the Enterococci responsible for the main third -90% of cases due to Enterococcus faecalis and less than 5% caused by Enterococcus faecium. The morbidity and mortality of enterococcal endocarditis is high. The pneumatosis intestinalis is usually asymptomatic, and complications are present in only 3% of cases, including intestinal obstruction, perforation and hemorrhage. Once in circulation, Enterococcus have high affinity for thickened or prosthetic heart valves. . Ainda que variáveis como a idade avançada, insuficiência cardíaca e êmbolos cerebrais têm sido reconhecidas como fatores de risco para elevada mortalidade, estudos multicêntricos têm avaliado o papel de outras variáveis, como a aquisição de infeção nosocomial, a presença de comorbilidades, ou a mudança de suscetibilidade antimicrobiana dos Enterococos como fatores determinantes do prognóstico 2,3,4 . Estes microrganismos estão na patogenia de uma importante percentagem de infeções nosocomiais a nível mundial, sendo cada vez mais associados com infeções do trato urinário, endocardites, infeções intra-abdominais e pélvicas, infeções relacionadas com cateteres, infeções de feridas cirúrgicas e infeções do sistema nervoso central 5,6,11 . As duas espécies de Enterococos mais comuns são Enterococcus faecalis e Enterococcus faecium, sendo o primeiro mais comum e responsável por 80-90% das EI por Enterococos 7 . Ambos são capazes de formar biofilmes 4,6 -complexos de moléculas ligadas irreversivelmente nas várias superfícies bióticas e mecânicas 7,8 . Gradualmente, as infeções contraí-das por Enterococos estão associadas a instrumentação dos tratos urinário e gastrointestinal (GI), bem como em situações de falta de integridade ou trauma da mucosa 7,11,12 . As complicações cardíacas por EI são propensas quando ocorrem enfarte agudo do miocárdio ou bacteriemia, devido à necrose das fibras papilares e do processo inflamatório envolvente 4,10,11 . A complicação mais comum é a formação de abcessos 14,15 . As fístulas intracardíacas podem desenvolver-se devido à extensão da infeção da válvula do miocárdio adjacente 14,21 . Raramente, podem levar à formação de aneurismas, disseção aórtica e perfuração do miocár-dio 5,14,22 . Habitualmente, o Es...
Takayasu arteritis (TA) is a rare type of vasculitis that affects mainly the aorta and its major branches. It is highly similar to giant cell arteritis (GCA), and differentiation between them may not be achieved even by histological examination. Arterial hypertension is typical of TA and is caused by stenosis of the renal arteries. Here we report the case of a 59-year-old woman, with a history of dyslipidemia and anemia, seen in the Internal Medicine department for resistant hypertension. Evaluation of secondary causes led to stenosis of the renal arteries. Assessment of target organ involvement was performed by computed tomography angiograph which revealed ectasia of the aortic arch and ascending aorta, tortuous course of the brachiocephalic trunk and the proximal portion of the right common carotid artery; positron-emission tomography which showed diffuse increased uptake in the ascending aorta, compatible with large vessels vasculitis. The patient was submitted to aortic valve replacement with a biological prosthesis combined with myocardial revascularization (Bentall-De Bono procedure). Aortic biopsy specimens showed anatomical and pathological features of GCA and TA. Due to persistently uncontrolled hypertension, prednisone 60 mg was initiated,with significant improvement in patient's condition.
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