Fatal necrotizing Candida esophagitis in a patient with leukocytoclastic cutaneous vasculitis and ankylosing spondylitis

Esophageal infection by Candida spp. is a common opportunistic entity in immunocompromised hosts; however, systemic fungal dissemination due to perforation or transmural necrosis, also known as necrotizing Candida esophagitis (NCE), is rare. We report the case of a 61-year-old male patient with diagnosed ankylosing spondylitis, severe arteriosclerosis, and vasculitis under immunosuppressive therapy who presented NCE with fungal and bacterial septicemia diagnosed at autopsy. Necrotizing esophagitis is a rare manifestation of Candida infection, which may be a final complication in severely ill patients. Unfortunately, it may be underdiagnosed, and we call attention to this devastating complication in patients with leukocytoclastic cutaneous vasculitis and ankylosing spondylitis.

Bullous systemic lupus erythematosus ­ a case report

Skin involvement in systemic lupus erythematosus (SLE) occurs in more than 75% of patients with this condition. Vesicles and blisters in lupus erythematosus (LE) may be present in SLE secondary to interface vacuolar changes in the epidermis, in discoid LE also secondary to vacuolar epidermal changes, and in bullous LE secondary to antibodies anti-collagen VII deposits with neutrophilic aggregates. In addition, blisters can occur due to the association of SLE with other autoimmune blistering diseases (e.g. bullous pemphigoid). BSLE is a rare blistering disease that mainly occurs in females (30­40 years old), and less frequently in children and adolescents. The most common presentation is rapid and widespread development of tense vesicles and bullae over erythematous macules or plaques. Preferential sites are: superior trunk, proximal superior limbs, and face (lips) with symmetrical distribution. Mucosal involvement is common on perioral, pharyngeal, laryngeal, and genital areas. The involvement of sun-exposed areas is not mandatory. The lesions usually progress with no scarring, but hypo or hyperchromia may be present. We report an 18-year-old female patient with blistering lesions at admission, who was diagnosed with BSLE. She was initially treated with systemic prednisone and hydroxychloroquine. Her condition evolved with relapsing lesions, which required the introduction of Dapsone. The authors emphasize the relevance of recognizing BSLE­a rare presentation of SLE­which may evolve with marked clinical presentation

Pulmonary histoplasmoma: a disguised malady

Histoplasmosis is a mycosis caused by the dimorphic fungus, Histoplasma capsulatum, which is transmitted via dust and aerosols. Lung involvement is the most common, with a varied clinical presentation. Although it is not the only source of infection, H. capsulatum is frequently found in bat guano, which is the reason why it is highly prevalent among caving practitioners. The solitary histoplasmoma of the lung is an unusual and chronic manifestation of this entity, which mimics, or at least is frequently misconstrued, as a malignancy. Almost invariably, the diagnosis of this type of histoplasmosis presentation is achieved after lung biopsy. The authors present the case of a young woman who sought medical care because of chest pain. The diagnostic work-up revealed the presence of a pulmonary nodule. She was submitted to a thoracotomy and wedge pulmonary resection. The histologic analysis rendered the diagnosis of histoplasmoma. Thisreport aims to call attention to this diagnosis as the differential diagnosis of a pulmonary nodule.

Celiac Crisis: an unusual presentation of gluten-sensitive enteropathy

Celiac disease (CD)­also known as gluten-sensitive enteropathy­is a chronic, genetically predisposing and autoimmune entity with a wide range of clinical manifestations triggered by gluten ingestion, which affects 1% of the general population. Currently, up to 60% of the diagnosis of CD is in adults due to the atypical course of the disease. The severe acute onset of CD­also called celiac crisis­is very uncommon and is still not well documented in adults. We report the case of a 58-year-old man who presented a 45-day history of subtle-onset diarrhea followed by malabsorption syndrome with progressive weight loss, anasarca, and electrolyte disturbances. The diagnostic work-up included an upper digestive endoscopy, which showed scalloping of the duodenal mucosa with pathological features confirmed on biopsies. Specific antibodies were positive, and a satisfactory clinical response was obtained once a gluten-free diet was started. Celiac crisis is a rare initial presentation of CD characterized by severe diarrhea, dehydration, weight loss, hypoproteinemia, and metabolic and electrolyte disturbances. Although rare, it should be considered in patients with apparently unexplained chronic diarrhea.

Bone marrow necrosis and fat embolism syndrome: a dreadful complication of hemoglobin sickle cell disease

Sickle cell disease encompasses a wide range of genotypic presentation with particular clinical features. The entity affects millions of people, particularly those whose ancestors came from sub-Saharan Africa and other countries in the Western Hemisphere, Saudi Arabia, and India. Currently, the high frequency of S and C genes reflects natural selection through the protection of heterozygotes against severe malaria, the high frequency of consanguineous marriages, improvement of some public health policies and the nutritional standards in the poorer countries where newborns are now living long enough to present for diagnosis and management. Although there is a high burden of the disease, in many countries, the new-born sickle cell screening test is being performed and is rendering an early diagnosis; however, it is still difficult for sickle cell patients to find proper treatment and adequate follow-up. Moreover, in many countries, patients are neither aware of their diagnosis nor the care they should receive to prevent complications; also, they do not receive adequate genetic counseling. Hemoglobin SC (HbSC) disease is the most frequent double sickle cell heterozygosis found in Brazil. The clinical course tends to be more benign with fewer hospitalizations compared with double homozygotic SS patients. However, HbSC patients may present severe complications with a fatal outcome. We report the case of a 36-year-old man who presented to the emergency care facility with symptoms consistent with the diagnosis of sickling crisis. The outcome was unfavorable and death occurred just hours after admission. The autopsy revealed a generalized vaso-occlusive crisis by sickled red cells, bone marrow necrosis, and fat embolism syndrome.

Severe pulmonary hypertension due to combined pulmonary fibrosis and emphysema: another cause of death among smokers

In 2005, the combined pulmonary fibrosis and emphysema (CPFE) was first defined as a distinct entity, which comprised centrilobular or paraseptal emphysema in the upper pulmonary lobes, and fibrosis in the lower lobes accompanied by reduced diffused capacity of the lungs for carbon monoxide (DLCO). Recently, the fibrosis associated with the connective tissue disease was also included in the diagnosis of CPFE, although the exposure to tobacco, coal, welding, agrochemical compounds, and tire manufacturing are the most frequent causative agents. This entity characteristically presents reduced DLCO with preserved lung volumes and severe pulmonary hypertension, which is not observed in emphysema and fibrosis alone. We present the case of a 63-year-old woman with a history of heavy tobacco smoking abuse, who developed progressive dyspnea, severe pulmonary hypertension, and cor pulmonale over a 2-year period. She attended the emergency facility several times complaining of worsening dyspnea that was treated as decompensate chronic obstructive pulmonary disease (COPD). The imaging examination showed paraseptal emphysema in the upper pulmonary lobes and fibrosis in the middle and lower lobes. The echo Doppler cardiogram revealed the dilation of the right cardiac chambers and pulmonary hypertension, which was confirmed by pulmonary trunk artery pressure measurement by catheterization. During this period, she was progressively restricted to the minimal activities of daily life and dependent on caregivers. She was brought to the hospital neurologically obtunded, presenting anasarca, and respiratory failure, which led her to death. The autopsy showed signs of pulmonary hypertension and findings of fibrosis and emphysema in the histological examination of the lungs. The authors highlight the importance of the recognition of this entity in case of COPD associated with severe pulmonary hypertension of unknown cause.

Bicytopenia and leukoerythroblastosis: a rare initial presentation of signet ring cell gastric adenocarcinoma

Gastric adenocarcinoma is a common neoplasia and is responsible for up to 30% of the overall deaths due to cancer. Advanced disease is mostly characterized by peritoneum, liver, and lung involvement. The spread of the disease to the bone is rare, and bone marrow dissemination is even rarer. In this setting, leukoerythroblastosis may be the initial manifestation of the disease. The authors report the case of a 64-year-old Caucasian man who sought medical care complaining of back pain, weakness, and weight loss. The physical examination revealed pallor, and the laboratory work-up depicted severe anemia and thrombocytopenia; the peripheral blood smear was consistent with leukoerythroblastosis. The ongoing investigation through a bone marrow biopsy showed massive involvement of the bone marrow by a signet ring cell adenocarcinoma. During hospitalization, the patient presented melena, and an upper digestive endoscopy depicted an ulcerated and infiltrative lesion in the cardia, upon which the histological examination revealed a signet ring cell adenocarcinoma. This case highlights the bone marrow invasion represented by bicytopenia and leukoerythroblastosis as the initial manifestation of this histological type of gastric cancer. Although treatment attempts were made with chemotherapy and radiotherapy, the patient died early on, showing the aggressive behavior of this form of tumoral presentation.

Academic autopsies in Brazil - a national survey / Autópsias acadêmicas no Brasil - um inquérito nacional

Objective: To investigate the number and rate of academic autopsies, general organization, educational and research in Brazilian academic services. Methods: Standardized questionnaires were sent to Brazilian medical schools (n=177) and active pathology residency programs (n=53) from March to June 2009. Data were collected for years 2003 to 2008. Results: Thirty-two academic services in 11 Brazilian states answered the survey. Twenty-one (65.6%) perform less than a hundred autopsies for natural causes and less than fifty pediatric or fetal autopsies/year. Twenty-four (75%) perform less than a hundred adult autopsies/year. Many institutions (46.9%) reported a drop in the number of autopsies in a six-year period. The total autopsy count and autopsy rate in 2008 ranged 1-632 (median = 80), and 0-66% (mean = 10.6%), respectively. A steady decrease in the total count of autopsies in a pool of 19 institutions was observed (p<0.01). Median autopsy rates have fallen from 19.3%, in 2003, to 10.6%, in 2008 (p=0.07). Significant discrepancies at autopsies led to changes in institutional healthcare practice in 37.5% of the services. The low number of autopsies was a limiting factor in undergraduate education for 25% of respondents. A minimum number of autopsies is required to complete the pathology residency program in 34.6% of the services. Conclusion: The total number and the rate of academic autopsies have decreased in Brazil between 2003 and 2008. The number of autopsies and the general organization of academic services must be enhanced to improve medical education, research, and the quality control of patient care. .

Objetivo: Investigar o número e a taxa de autópsias acadêmicas, organização geral, ensino e pesquisa em serviços acadêmicos brasileiros. Métodos: Questionários padronizados enviados para escolas médicas brasileiras (n=177) e programas de residência em patologia ativos (n=53) de março a junho de 2009. Dados coletados referentes ao período de 2003 a 2008. Resultados: Trinta e dois serviços em 11 estados responderam à pesquisa. Vinte e um (65,6%) realizam menos de cem autópsias de causas naturais e menos de cinquenta autópsias fetais ou pediátricas/ano. Vinte e quatro (75%) realizam menos de cem autópsias de adultos/ano. Muitas instituições (46,9%) relataram queda no número de autópsias em seis anos. A contagem total e a taxa de autópsias em 2008 variaram, respectivamente, de 1 a 632 (mediana=80) e de 0 a 66% (média=10,6%). Foi observada uma redução contínua no total de autópsias em um grupo de 19 instituições (p<0,01). A mediana da taxa de autópsias caiu de 19,3%, em 2003, para 10,6%, em 2008 (p=0,07). Discrepâncias significativas observadas em autópsias levaram a mudanças na prática institucional de saúde em 37,5% dos serviços. O baixo número de autópsias foi limitante no ensino de graduação para 25% dos entrevistados. Um número mínimo de autópsias é necessário para completar o programa de residência em patologia em 34,6% dos serviços. Conclusão: O número total e a taxa de autópsias acadêmicas diminuíram no Brasil entre 2003 e 2008. O número de autópsias e organização geral dos serviços acadêmicos deve ser melhorado para fortalecer a educação médica, pesquisa e controle de qualidade prestado ao paciente. .

Frequência de anemia infecciosa equina em equinos nos estados da Paraíba, Rio Grande do Norte e Ceará durante o ano de 2010 / Frequency of equine infectious anemia in equine in the states of Paraíba, Rio Grande do Norte and Ceará, Northeastern Brazil during 2010

A frequência de equinos soro-reagentes para o vírus da Anemia Infecciosa Equina (AIE) foi investigada, durante o ano de 2010, em 5615 animais originários de 209 municípios de três estados do nordeste do Brasil: Paraíba, Rio Grande do Norte e Ceará. Os soros foram examinados com o emprego do teste de imunodifusão em gel de ágar (IDGA) produzido com o antígeno p26. Dos 5615 animais examinados, 151 (2,69%) foram soropositivos. As proporções de animais reatores positivos por estado foram: Paraíba (2,86%), Rio Grande do Norte (1,29%) e Ceará (3,10%). Tomando-se por base a menor proporção de resultados positivos encontrada no estado do Rio Grande do Norte, o estado da Paraíba apresentou uma odds ratio de 2,64 (IC 95% = 1,33-5,36; p = 0,004) e o Ceará de 2,87 (IC 95% = 1,48-5,71; p = 0,001). Na Paraíba houve o registro de animais soropositivos em todos os meses do ano, com frequência mínima (0,58%) em julho e máxima (5,82%) em junho; no Rio Grande do Norte de março a maio e de julho a novembro, com variação de 0% em janeiro, fevereiro, junho e dezembro a 3,61% em maio; e no Ceará em todos os meses com frequência mínima (1,10%) em agosto e máxima (7,29%) no mês de dezembro.

During the year of 2010 the frequency of equines sero-reactants to the Infectious Anemia vírus (EIA) was investigatedin 5,615 animals originated from 209 counties of three northeastern brazilian states: Paraíba, Rio Grande do Norte and Ceará. The serum samples were examined by an agar gel immunodiffusion test (AGID) produced with p26 antigen. Of the 5,615 animals examined 151 (2.69%) were seropositive to EIA. The proportion of seroreactant animals per state were: Paraíba (2.86%), Rio Grande do Norte (1.29%) and Ceará (3.10%). Paraíba presented an odds ratio of 2.64 (95% CI = 1.33-5.36; p = 0.004), while Ceará had an odds ratio of 2.87 (95% CI = 1.48-5.71; p = 0.001) both of them compared with Rio Grande do Norte. Paraíba had seropositive animals in all months of the year, with frequency ranging from 0.58% in July to 5.82% in June; in Rio Grande do Norte from March to May and from July to November ranging from 0% in January, February, June and December to 3.61% in May; and in Ceará in all months ranging from 1.10% in August to 7.29% in December.

Teníase na população do bairro Nossa Senhora Aparecida, município de Correia Pinto - SC em 2003 e 2004 / Taeniasis in the population of Nossa Senhora Aparecida district, county of Correia Pinto - SC in 2003 and 2004

O presente trabalho foi realizado entre dezembro de 2003 e janeiro de 2004. Teve como objetivo avaliar a ocorrência de teníase nessa população. Participaram do estudo 628 pessoas, dessas, 16 (2,5%) eram portadores de teníase. Praticamente a metade dos casos de teníase ocorreram em indivíduos na faixa etária entre os 30 a 49 anos (49,9%). Ocorreu associação entre existência dehortas e adubação, com os casos de teníase (p < 0,001). O consumo de carne de porco “mal passada”, também apresentou significância estatística (p = 0,003). A presença de sintomas estava associada aos casos com teníase (p = 0,004). Constatou-se ainda, que as pessoasque apresentaram teníase tinham maior chance de possuir outras enteroparasitoses (p = 0,038). O maior problema do bairro é a falta de infra-estrutura sanitária, possibilitando a exposição das pessoas e dos animais, ao esgoto a céu aberto, favorecendo a manutenção do complexo teníase/cisticercose. Desta forma, para interromper essa cadeia de transmissão faz-se necessária à melhoria da infra-estrutura sanitária e intensificação das ações preventivas e educativas em saúde pública.

The present study was done from december, 2003 to january, 2004. The main objective of this research was to evaluate the occurrence of taeniasis among its population. 628 people took part in the study, being 16 (2,5%) of the participants carriers of taeniasis. Almost half of the cases of teniasis were found among individuals around the age of 30 to 49 years (49.9%). A correlation between the existence of vegetable gardens and adubation and the cases of taeniasis was found (p=0.001). The consumption of raw pork meat was found to be statistically significant (p=0.003).The symptons were associated with cases of taeniasis (p = 0.004). It was also found that those who had taeniasis had a higher chance of having other enteroparasites (p=0.038). The most important problem in the neighborhood is the lack of sanitary infrastructure, forcing people and animals to the open contact with landfills and sewage contamination, and therefore favouring the maintenance of the complex taeniasis/cysticercose. In order to end this transmission chain, it is necessary that a sanitary infra-structure is implemented and that preventive and educational actions in public health be intensified.

Multiple brain abscesses due to Penicillium spp infection

Apresentamos um caso de infecção do sistema nervoso central (SNC) por Penicillium spp em paciente do sexo masculino, HIV-negativo no Brasil. O paciente apresentou-se ao Serviço de Urgência do Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo queixando-se de alteração visual e dificuldade na fala. Exames de neuroimagem mostraram lesões múltiplas, compatíveis com abscessos. A biópsia esterotáxica revelou infecção fúngica, iniciando-se o tratamento com anfotericina B com sucesso inicial. O paciente morreu poucos dias depois, vítima de uma hemorragia digestiva maciça devido a varizes de esôfago. A necropsia e a análise microbiológica final da biópsia cerebral revelaram infecção por Penicillium spp. Exixtem centenas de espécies de fungos do gênero Penicillium. A peniciliose sistêmica é causada pelo P. marneffei e costumava ser uma doença rara, mas atualmente é uma das infecções oportunistas mais comuns em associação com AIDS no Sudeste Asiático. Infecção pelo Penicillium spp de espécie diferente do P. marneffei normalmente causa apenas doenças superficiais ou alérgicas mas doenças invasivas também ocorrem raramente. Nós relatamos o quarto caso de infecção do SNC por Penicillium spp.

Cirrose hepática: aspectos morfológicos relacionados às suas possíveis complicações. Um estudo centrado em necropsias / Hepatic cirrhosis: morphologic aspects related to its possible complications. A study focused on necropsies

INTRODUÇAO: A cirrose hepática é muito prevalente em nosso meio, cursando freqüentemente com complicações como insuficiência e carcinoma hepatocelulares e hipertensão portal. OBJETIVOS: Analisar os aspectos clínicos e morfológicos das cirroses, relacionando-os com os principais padrões histológicos e suas complicações. MATERIAL E MÉTODO: Entre 35 fígados cirróticos provenientes de necropsias realizadas no Serviço de Verificação de Obitos da Capital (SVOC-SP), estudados em cortes seqüenciais a cada centímetro, dois padrões anatomopatológicos predominaram: os associados a hepatites crônicas e os decorrentes de esteato-hepatites. RESULTADOS E DISCUSSAO: A alta incidência de cirrose em necropsias (7,1 por cento), com freqüente detecção de macronódulos (62,8 por cento) e de carcinoma hepatocelular (CHC) (37,1 por cento), aponta para a relevância dessas questões em saúde pública. A análise histológica demonstrou o encontro de neoductos, agressão biliar e siderose com maior freqüência na hepatite crônica que na esteato-hepatite. CONCLUSÕES: Essa abordagem sistemática nos espécimes necroscópicos permitiu-nos a identificação de elevada freqüência de cirrose em necropsias do SVOC e, em especial, daquelas decorrentes de hepatites crônicas, aspecto de interesse sanitário. A freqüente detecção de CHC, além de constituir modelo para futuros estudos sobre o surgimento dessas neoplasias, demonstra ser uma das principais complicações da cirrose avançada, qualquer que seja sua causa.

The value of cytology and pleural biopsy in the differential diagnostic of nonspecific pleural effusions

A significant percentage of pleural effusions remains without a diagnostic explanation. In such circumstances, the anatomical-pathological result of nonspecific chronic pleuritis should be revised. This is an important issue, not only for the pathologist, but mainly for the pneumologist, who usually makes the clinical-pathological correlation during the routine practice. Although the existence of established criteria is accepted, they are certainly subjective and make the communication by means of a common language difficult, mainly among pathologists. The authors recently studied 311 pleural biopsies with histo-pathological diagnoses of chronic nonspecific pleuritis. All specimens were reviewed and the histo-pathological parameters quantified by stereology. The patients were stratified according to the final diagnosis of their disease, including cases of chronic renal failure, vasculitis, pancreatitis, tuberculosis, cancer, and congestive cardiac failure, in this case considering the pleura as normal. This procedure allowed us to obtain a discriminating model, whose morphological subcriteria classified almost 90% of the nonspecific chronic pleuritis biopsies, according to their final clinical diagnoses, which included true nonspecific chronic pleuritis or tuberculosis pleuritis, paraneoplastic pleuritis, or even normal pleura. By adding to this model the biochemistry or differential cytology of the pleural liquid, its classificatory power reaches 99% of correctness. This study represents the result of the experience acquired over several years in the histo-pathological interpretation of pleural biopsies, based on the correlation between morphology and biochemistry and cytology of the pleural fluid.

Interstitial granulomatous pulmonary diseases: a diagnostic approach for the general pathologist

Some kinds of interstitial pneumonia present a histopathological pattern dominated by sarcoid - necrotizing or non-necrotizing - granulomas, which can be divided into two main groups: infectious and non-infectious. The infectious causes include tuberculosis, histoplasmosis, fungi in general, paracoccidioidomycosis, ascaridiasis, echinococcosis and dirophilariosis. The non-infectious causes include histiocytosis-X, hipersensitivity pneumonia, vasculitis, lymphomas, sarcoidosis, and pneumoconioses such as silicosis and berylliosis. The purpose of this review is to provide a practical guideline to enable general pathologists to make the differential diagnosis of granulomatous pulmonary diseases. For this purpose, anatomical-clinical-radiological correlations will be presented and targeted to each diagnosis discussed. Whenever a granulomatous inflammatory process is in progress, the search for infective agents by direct observation, by culture, and by histochemical methods should be mandatory. The histological aspects of infectious granulomas to be analyzed should include their random histo-anatomical location, the type of inflammatory reaction, and necrosis. A panel of complementary reactions (immunohistochemistry and PCR) should identify the infectious agent and, whenever their results and the culture are negative, the possibility of non-infectious granulomatous diseases has to be evaluated. In such cases, the histo-anatomical distribution (bronchocentric, lymphangitic, angiocentric, random), the qualitative characteristics of the lesions (type of necrosis and inflammatory reaction), and the correlation with the X-ray findings will help the diagnosis.