Zigomicosis y síndrome hemofagocítico en un paciente inmunocompetente: informe de caso / Zygomycosis and hemophagocytic lymphohistiocytosis in an immunocompetent patient: a case report

Introducción: la zigomicosis es una infección fúngica poco frecuente, con alta tasa de mortalidad y de mal pronóstico. Afecta principalmente a pacientes inmunocomprometidos. La asociación con el síndrome hemofagocítico es extremadamente inusual, más aún en pacientes inmunocompetentes, con pocos ejemplos registrados en la literatura. Caso clínico: se presenta el caso de un paciente masculino inmunocompetente de 40 años con diagnóstico de mucormicosis y síndrome hemofagocítico que evoluciona desfavorablemente, con fallo multiorgánico, a pesar de los esfuerzos médicos. Conclusión: la asociación de mucormicosis con síndrome hemofagocítico en un paciente inmunocompetente es extremadamente rara; existen pocos casos informados en Latinoamérica. Debemos tener presente esta asociación, ya que requiere un tratamiento agresivo y soporte vital avanzado. (AU)

Introduction: zygomycosis is a rare fungal infection that carries with high mortality rates. This poor prognosis, rapidly progressive infection mainly affects immunocompromised patients. The association with hemophagocytic lymphohistiocytosis is extremely unusual, even more in immunocompetent patients, with few cases reported. Case: we present the case of an immunocompetent male patient who was diagnosed with zygomycosis and hemophagocytic lymphohistiocytosis. Despite medical efforts he developed multiorganic failure. Conclusion: the association of mucormycosis with hemophagocytic lymphohistiocytosis in an immunocompetent patient is exceptional with few cases reported in Latin America. We must always suspect this association considering they require aggressive treatment and advanced life support. (AU)

Conidiobolomicose causada por conidiobolus lamprauges em ovinos no estado de Santa Catarina / Conidiobolomycosis caused by conidiobolus lamprauges in sheep in the state of Santa Catarina, Brazil

Descreve-se um surto de conidiobolomicose em ovinos no Estado de Santa Catarina. O surto ocorreu entre os meses de dezembro e março de 2006, no município de Santo Amaro da Imperatriz, região litorânea do Estado. A propriedade possuía 75 ovinos da raça Santa Inês e seis desses animais adoeceram. Clinicamente os animais doentes apresentavam dificuldade respiratória, corrimento nasal seroso a mucossanguinolento e, por vezes exolftalmia. Na necropsia verificou-se uma massa amarelada na região etmoidal e adjacências que, às vezes, atingia os linfonodos regionais, cérebro, globo ocular e pleura. Microscopicamente a massa caracterizava-se por infiltrado inflamatório granulomatoso com áreas necróticas associadas a hifas largas pouco ramificadas. Através de exame molecular detectou-se DNA de Conidiobolus lamprauges. Os aspectos clínicos, epidemiológicos, macroscópicos, microscópicos e moleculares caracterizam a conidiobolomicose causada por Conidiobolus lamprauges em ovinos.

An outbreak of conidiobolomycosis affecting sheep in the State of Santa Catarina, Southern Brazil is reported. The disease occurred in six Santa Inês breed sheep from a flock of 75 during the rainy season. Common clinical signs were noisy respiration and dyspnea, serous to mucosanguineous nasal discharge and exophthalmus. At necropsy there was a dense yellow mass in the nasopharyngeal area affecting the ethmoidal region, turbinate bones and occasionally limph nodes, central nervous system and pleura. Histopathologycally there was multifocal granulomas whith an eosinophilic necrotic reaction containing ribbon type hyphae similar to zygomycetous fungi. At molecular examination Conidiobolus lamprauges DNA was detected. The clinical, epidemiological, macroscopical, microscopical and molecular aspects characterize conidiobolomycosis caused by Conidiobolus lamprauges in sheep.

Primary cutaneous zygomycosis from a tertiary care centre in north-west India.

Background & objectives: Zygomycosis is highly invasive fungal infection, with high mortality rate. In most of patients, diabetes mellitus is an underlying factor but in primary cutaneous zygomycosis, presentation may be different. Here we present the description of clinical presentation, fungi isolated and management of cases with cutaneous zygomycosis seen in a tertiary care hospital in north India during 2001-2007. Methods: All patients diagnosed with primary cutaneous zygomycosis between November 2001 and September 2007 presenting with clinical diagnosis of necrotizing fasciitis were included. Detailed history of each patient was taken, clinical presentation, site of involvement, underlying illness and risk factor, if any were noted. The diagnosis was established by direct microscopic evidence of broad, aseptate or sparsely septate ribbon-like hyphae with right angle branching in KOH wet mount and histopathological examination of stained sections. Cultures were put up for fungal isolation and species identification. Outcome of the therapy was analysed. Results: Of the nine patients reviewed, only one had diabetes mellitus. Commonest risk factor was injection abscess (33.3%). Apophysomyces elegans was isolated in four cases, Saksenaea vasiformis and Absidia corymbifera in one each. The fungal culture was sterile in three cases. Mortality rate was high with only four patients responded well to surgical and/or medical therapy. Interpretation & conclusion: Primary cutaneous zygomycosis appears to be on rise in India that calls for high index of clinical suspicion and an early biopsy of the affected area for timely diagnosis. The standard treatment is a combination of amphotericin B therapy, surgical debridement, and reversal of the underlying disease or immunosuppression.

Conidiobolomicose em ovinos no Estado de Mato Grosso / Conidiobolomycosis in sheep in the state of Mato Grosso, Brazil

Relatam-se os achados epidemiológicos, clínicos, patológicos e micológicos de conidiobolomicose em ovinos no Estado de Mato Grosso. A doença ocorreu em uma propriedade no município de Nobres, em um rebanho com 40 ovelhas adultas, entre os meses de janeiro a junho de 2007. Aproximadamente 30 por cento dos ovinos da propriedade adoeceram e todos os doentes morreram em curso clínico de 2-5 semanas. O quadro clínico foi caracterizado por apatia, emagrecimento, dificuldade respiratória com dispnéia, respiração ruidosa e oral, secreção nasal mucosa ou sero-sanguinolenta, exoftalmia unilateral, por vezes com cegueira, e morte. Havia desaparecimento de etmoturbinados com substituição por tecido bran-cacento, finamente granular, multilobulado e friável infil-trando-se na lâmina cribiforme, no septo nasal e nas coa-nas em todos os ovinos necropsiados. Lesões similares foram encontradas em linfonodos regionais (2 casos), pulmões (3), encéfalo (2) e em linfonodos do abomaso (1). Microscopicamente havia inflamação granulomatosa da região rinocerebral, caracterizada por necrose, proliferação de tecido conjuntivo, infiltrado de neutrófilos, eosinófilos, células epitelióides e células gigantes multinucleadas freqüentemente circundando material Splendori-Hoeppli, onde havia imagens negativas de hifas. Na impregnação pela Prata-Metenamina, as hifas tinham septos e ramificações escassas e irregulares, com dilatação balonosa terminal e com forte demarcação de contornos. Iso-lou-se de tecido nasal de quatro ovinos Conidiobolus sp.

The epidemiological, clinical, pathological and mycological findings of an outbreak of conidiobolomycosis in a flock of 40 Santa Ines sheep, in the state of Mato Grosso, Brazil, are reported. The illness occurred in the municipality of Nobres during January-June, 2007, resulting in death of about 30 percent of the affected sheep within 2-5 weeks. The clinical signs were characterized by apathy, weight loss, labored and noisy breathing with dyspnea, and mucous or serohemorrhagic nasal discharge. In three sheep there was unilateral exophthalmia, keratitis and corneal ulceration. A firm whitish, multi-lobulated, friable growth was evident in the ethmoturbinate region at the coronal sections of the head from six affected sheep. In all sheep the choana had similar nodular infiltration which resulted in local obstruction. In three of those there was exophthalmia. There was infiltration in the cribiform plate and brain (2 cases), regional lymph nodes (2), lungs (3), and abomasums (1). Microscopic findings were granulomatous inflammation of the rhinoetimoidal region, with necrosis, lymphocytic infiltration, epithelioid multinucleated giant cells and fibrovascular tissue, surrounding Splendore-Hoeppli material wich contained unstained ghost images of hyphae. The methenamine-silver stain uncover fungi hyphae, rarely ramified with bulbous dilatation in their extremities. Conidiobolus sp. was isolated from nasal tissue lesions of four sheep.

Epidemiologia e sinais clínicos da conidiobolomicose em ovinos no Estado do Piauí / Epidemiology and symptoms of conidiobolomycosis in sheep in the State of Piauí, Brazil

Foi realizado um estudo de ocorrência da conidiobolomicose ovina em 25 rebanhos no Estado do Piauí, de janeiro de 2002 a dezembro de 2004. A enfermidade acometeu apenas ovinos e ocorreu principalmente em abril-julho. A incidência média anual foi de 2,80 por cento e a incidência semestral foi significativamente mais alta (P <0,05) no primeiro semestre (2,10 por cento), durante a época chuvosa, do que no segundo semestre (0,69 por cento), durante a seca. A incidência entre rebanhos variou de 0,1-14,3 por cento e a letalidade foi de 100 por cento. Ao exame clínico, os animais apresentavam apatia, emagrecimento progressivo, secreção nasal serosa, mucosa e/ou hemorrágica, dificuldade respiratória, respiração ruidosa, febre e na maioria dos casos assimetria crânio-facial e exoftalmia. Alguns ovinos permaneciam com a cabeça baixa ou a pressionavam contra objetos. O curso clínico foi de 1-5 semanas. As lesões macroscópicas, microscópicas e ultrastructurais e a identificação do agente são descritas separadamente. Este é o primeiro registro de conidiobolomicose em ovinos no Brasil, enfermidade endêmica no Estado do Piauí, associada à alta pluviosidade (1000-1600mm anuais) e alta temperatura (19-36ºC).

Conidiobolomycosis is reported in 25 farms, from January 2002 to December 2004, in the state of Piauí. The disease affects only sheep, mainly in April-June. The mean morbidity rate was 2.80 percent, but was higher in the first semester (2.1 percent), during the raining period, than in the second one (0.69 percent), during the dry period. Morbidity rate among flocks varied from 0.1-14.3 percent. Case fatality rate was 100 percent, and the clinical manifestation period varied from 1-5 weeks. Clinical signs were serous, mucous and/or bloody nasal secretion, respiratory distress, snoring respiration, cranium-facial asymmetry, exophthalmia, fever and progressive emaciation. Marked depression, sometimes with the head down or head pressing was observed in some cases. Gross, microscopic and ultrastructural lesions and identification of the agent are reported elsewhere. This is the first report of conidiobolomycosis in Brazil, which is endemic and has a high frequency in sheep in the State of Piauí, associated with high rainfalls (1000-1600mm annually) and high temperature (19-36ºC).

Experiencia en mucormicosis Hospital Clinico Universidad de Chile / Experience in mucormycosis Hospital Clínico Universidad de Chile

Revisamos los hallazgos clínicos, patológicos y tratamiento en 12 pacientes con mucormicosis en el Hospital Clínico de la Universidad de Chile entre los años 1990 y 2004. Del total de pacientes estudiados, 7 eran hombres y 5 mujeres. La edad promedio fue de 54 años, con un rango de 29 a 79 años. El diagnóstico de mucormicosisse realizó por biopsia en 10 pacientes y por estudio directo en 2 pacientes. Los factores predisponentes presentes en los pacientes estudiados fueron, diabetes mellitus, HIV en etapa SIDA y enfermedades hematológicas en tratamiento oncológico. La histología demostró necrosis e infarto con invasión de vasos sanguíneos y trombosis micótica. Todos los pacientes estudiados recibieron tratamiento con Anfotericina B y 6 fueron intervenidos quirúrgicamente. Del total de 12 pacientes, 4 fallecieron (33 por ciento) y 8 permanecen vivos (66 por ciento).

We made a review of the clinical and pathological findings and the treatment in 12 patients presenting mucormycosis in the Clinical Hospital of the University of Chile between 1990 and 2004. Of the whole studied patients, 7 were men and 5 women. The average age was 54 years, with a range from 29 to 79 years. The diagnosis of mucormycosis was performed by biopsy in 10 patients andby direct study in 2 patients. The predisposing factors in the studied patients were: Diabetes Mellitus, HIV at AIDS stage and hematological diseases in oncologic treatment. The histology demonstrated necrosis and infarction with blood vessels invasion and mycotic thrombosis. All the studied patientsreceived treatment with Anphotericine B and 6 required surgical intervention. Of the total of 12 patients, 4 died (33 percent) and 8 remain alive (66 percent).