Mucormicosis periostomal posoperatoria, a propósito de un caso / Mucormycosis periostomal: case report

Resumen Introducción: La mucormicosis en una enfermedad infrecuente y oportunista que afecta, principalmente, a pacientes inmunocomprometidos. Pocas veces se han reportado casos de afectación periostomal. Clínicamente puede ser confundida con otras patologías, pudiendo tener una evolución fulminante, por lo que un adecuado y pronto diagnóstico son necesarios para una instauración precoz del tratamiento. Caso Clínico: Se presenta el caso de una paciente de 62 años inmunocomprometida, que tras complicaciones quirúrgicas evoluciona con mucormicosis periostomal de la pared abdominal. A pesar de un tratamiento quirúrgico con múltiples resecciones de tejido asociado a antifúngico local y sistémico, la paciente fallece, concordante a la letalidad expresada en la literatura.

Introduction: Mucormycosis is a rare and opportunistic disease that mainly affects immunocompromised patients. Few cases of peristomal involvement have been reported. Clinically it can be confused with other pathologies and may have a fulminant evolution, so an adequate and prompt diagnosis is necessary for an early establishment of treatment. Clinical Case: We present the case of a 62-year-old immunocompromised patient who, after surgical complications, evolves with periostomal mucormycosis of the abdominal wall. Despite surgical treatment with multiple tissue resections, associated with local and systemic antifungal agents, the patient died, consistent with the lethality expressed in the literature.

Mucormicosis asociada a COVID-19: revisión de la literatura para odontólogos / COVID-19 associated mucormycosis: a literature review for dentists

La mucormicosis es una infección fúngica rara, con alta morbilidad y mortalidad. Se presenta principalmente en pa- cientes con diabetes mellitus no controlada, inmunocompro- metidos, con tratamiento crónicos con esteroides, entre otros. Actualmente, se cree que la pandemia de COVID-19 y los tratamientos con corticosteroides podrían estar implicados en el aumento de casos de esta micosis. Este hongo invade el sistema vascular, ocluyendo el flujo sanguíneo arterial y generando una rápida trombosis e isque- mia, lo que provoca la necrosis de los tejidos duros y blandos, con invasión rápida a los tejidos circundantes. Hay varias formas clínicas. En la cavidad bucal se presenta la variante rino-orbito-cerebral, que afecta el paladar en forma de lesión eritematosa o grisácea que puede progresar hacia la formación de una masa necrótica o ulceración con muy escaso sangrado de mucosa. Se manifiesta con síntomas típicos de una rinosinusitis con fiebre y dolor en las piezas dentarias superiores. El tratamiento consta de tres pilares fundamentales: el diagnóstico, un manejo adecuado de las comorbilidades y la combinación de las terapias antifúngica y quirúrgica. Desde el año 2020, la mucormicosis asociada a COVID-19 pasó a ser un evento de notificación obligatoria inmediata al Sistema Nacional de Vigilancia de la Salud (SNVS2.0) me- diante el Sistema Integrado de Información Sanitaria Argen- tina (SISA). Es importante destacar que se han reportado casos de mu- cormicosis luego de extracciones dentales; lo que impulsa a afianzar los conocimientos sobre esta enfermedad, extremar las medidas preventivas e incentivar el diagnóstico precoz en la atención odontológica, debido a la rapidez en la evolución de la patología (AU))

Mucormycosis is a rare fungal infection, with high mor- bidity and mortality. It occurs mainly in patients with uncon- trolled diabetes mellitus, immunocompromised, on chronic treatment with steroids, among others. Currently, it is believed that the COVID-19 pandemic and the corticosteroid treatments could be one of the causes of increased cases. This fungus invades the vascular system, occluding arteri- al blood flow and generating rapid thrombosis and ischemia, which causes necrosis of hard and soft tissues, with rapid in- vasion to the surrounding tissues. There are several clinical forms. In the oral cavity, the rhino-orbito-cerebral variant presents itself affecting the pal- ate in the form of an erythematous or grayish lesion that can progress towards the formation of a necrotic mass or ulcera- tion with very little mucosal bleeding. It manifests itself with typical symptoms of rhinosinusitis, with fever and pain in the upper teeth. The treatment consists of three fundamental pillars: diag- nosis, proper management of comorbidities and the combina- tion of antifungal and surgical therapies. Since 2020, COVID-19 associated mucormycosis became an event of mandatory immediate notification to the National Health Surveillance System (SNVS2.0,) through the Argentina Integrated Health Information System (SISA). It is important to emphasize that mucormycosis cases had been reported following tooth extractions, which drives to strengthen knowledge about this disease, extreme preventive measures and encourage early diagnosis in dental care, due to the speed of the evolution of the pathology (AU))

How could hypoglycemia-inducing glycogen storage disease lead to hyperglycemia-induced mucormycosis?

Mucormycosis is an increasingly frequent, difficult to diagnose, difficult to treat, often fatal infection, especially in patients with hyperglycemia from uncontrolled diabetes. Type I (von Gierke) glycogen storage disease is due to inherited deficiency of enzymes in glycogen metabolism, which causes hypoglycemia. This report is the case of a patient with von Gierke disease and a missed diagnosis of pulmonary mucormycosis. This report illustrates the importance of having a high index of suspicion for mucormycosis in the appropriate clinical context.

An Emergent Entity: Indolent Mucormycosis of the Paranasal Sinuses. A Multicenter Study

Abstract Introduction Indolent or chronic mucormycosis is a rare entity that affects both immunosuppressed and immunocompetent individuals. Additionally, its clinical evolution is nonspecific and there is no standardized treatment for this condition. Objective To describe the clinical characteristics and management of patients with indolent mucormycosis. Methods In the project of study with chart review in the Interinstitutional secondary care centers, patients with evidence of indolentmucormycosis, defined as pathological confirmation of nasal/paranasal sinus mucormycosis for more than 1 month, were included. All patients underwent complete laboratory workup, imaging studies, surgical treatment and adequate follow-up. No evidence of disease status was defined when patient had subsequent biopsies with no evidence of mucormycosis. Results We included seven patients, three female and four male subjects. The mean age was 53.14 years. Four patients were immunosuppressed and three immunocompetent. Among the immunosuppressed patients three had diabetes and one had dermatomyositis. The symptomswere nonspecific: facial pain/headache, mucoid discharge and cacosmiawere the ones most frequently reported. Maxillary sinus involvement was present in all patients. Two immunosuppressed subjects received amphotericin. Posaconazole was the only treatmentinoneimmunosuppressedpatient. Allimmunocompetent patientshadsingleparanasal sinus disease and received only surgical treatment. All patients are alive and free of disease. Conclusion Indolent mucormycosis is a new and emerging clinical entity in immunosuppressed and immunocompetent patients. Single paranasal sinus disease is a frequent presentation and should not be overlooked as a differential diagnosis in these patients. Immunocompetent patients should only be treated surgically. (AU)

Tricodisplasia espinulosa asociada a síndrome mielodisplásico e inmunosupresión / Trichodysplasia spinulosa: report of one case

Trichodysplasia spinulosa is a rare disease that occurs in the setting of immunosuppression, associated with tolerogenic therapy used in allograft recipients or patients with hematologic malignancies. Clinically, it is characterized by a centrofacial cutaneous eruption of erythematous papules with a central keratinous spicule, often associated with variable degrees of alopecia. Histologic findings are characteristic, and electron microscopy reveals the presence of trichodysplasia spinulosa associated polyomavirus. We report a 47-year-old woman with idiopathic autoimmune pancytopenia refractory to diverse immunosuppressant regimens, with clinical and pathologic findings compatible with the disease, in whom complementary studies were required to exclude other differential diagnoses.

Mucormycosis and chromoblastomycosis occurring in a patient with leprosy type 2 reaction under prolonged corticosteroid and thalidomide therapy / Mucormicose e cromoblastomicose em um paciente com reação hansênica tipo II sob terapia prolongada com corticosteróide e talidomida

Mucormycosis is an uncommon fungal infection caused by Mucorales. It frequently occurs in patients with neutropenia, diabetes, malignancy and on corticoid therapy. However, it is rare in patients with AIDS. Clinical disease can be manifested in several forms. The case reported illustrates the rare occurrence of chromoblastomycosis and mucormycosis in an immunosuppressed patient with multibacillary leprosy, under prolonged corticosteroid and thalidomide therapy to control leprosy type 2 reaction. Neutrophil dysfunction, thalidomide therapy and work activities are some of the risk factors in this case. Chromoblastomycosis was treated by surgical excision and mucormycosis with amphotericin B. Although the prognosis of mucormycosis is generally poor, in the reported case the patient recovered successfully. This case should alert dermatologists to possible opportunistic infections in immunosuppressed patients.

Mucormicose é uma infecção fúngica incomum causada por Mucorales. Ocorre frequentemente em pacientes com neutropenia, diabetes, corticoterapia e condições malignas. Porém, é rara em pacientes com AIDS. A doença pode apresentar-se em diferentes formas. Este caso ilustra a rara ocorrência de mucormicose e cromoblastomicose em um paciente com hanseníase multibacilar, que estava sendo tratado com prednisona e talidomida devido a eritema nodoso (reação hansênica tipo II). Disfunção de neutrófilos, uso de talidomida e atividades profissionais são alguns fatores de risco neste caso. A cromoblastomicose foi tratada por excisão cirúrgica e a mucormicose com anfotericina B. Embora o prognóstico da mucormicose seja ruim, neste caso o tratamento foi bem sucedido. Este caso alerta dermatologistas para a possibilidade de infecções oportunistas em pacientes imunossuprimidos.

Mucormicosis: Una micosis emergente / Mucormycosis: An emergent mycosis

El objetivo de este trabajo es presentar la incidencia, frecuencia, características clínicas y evolución de los pacientes con mucormicosis atendidos en el Hospital de Clínicas José de San Martín, Universidad de Buenos Aires, entre los años 1982 y 2010. Durante ese período se diagnosticaron 10 casos de mucormicosis. Los tres primeros entre 1982 y 2004 y los últimos 7 entre 2005 y 2010. La incidencia y frecuencia de esta enfermedad, para el período 1980-2004 fue 0.13 pacientes/año y 0.1 casos/10 000 egresos (IC 95%: 0.00 a 0.3) respectivamente. En el período 2005-2010 la incidencia fue 0.86 pacientes/año y la frecuencia de 1.1 casos/10 000 egresos (IC 95%: 0.5 a 2.4). Hubo nueve casos de mucormicosis rinosinuso-orbitaria, siete en pacientes con diabetes mellitus, uno en una paciente con una hemopatía maligna y neutropenia, y el restante en un paciente con HIV/sida que además estaba neutropénico y con un síndrome hemofagocítico. En una paciente se realizó el diagnóstico post mortem de mucormicosis pulmonar. El diagnóstico se efectuó por la observación de filamentos cenocíticos en los diez casos. Hubo desarrollo de mucorales en los cultivos de 8/9 pacientes; cinco Rhizopus spp y tres Mucor spp. Todos los pacientes recibieron un tratamiento inicial con anfotericina B deoxicolato, que en tres de ellos fue continuado con anfotericina B liposomal, y cirugía. Tres enfermos recibieron además un tratamiento adyuvante con oxigeno hiperbárico. La mortalidad fue 30%.

Mucormycosis is an opportunistic infection caused by fungi of the order Mucorales. It is characterized by rapid progression and high morbidity and mortality in the absence of early diagnosis and prompt treatment. It was an infrequent disease, but in recent years, its incidence appears to have increased. The aim of this paper is to report the cases of mucormycosis diagnosed from 1982 to 2010 at the Hospital de Clinicas José de San Martín, University of Buenos Aires. We diagnosed 10 cases of mucormycosis; the first three between 1982 and 2004 and the last 7 between 2005 and 2010. The incidence from 1980 to 2004 was 0.13 patient-years and the frequency 0.1/10 000 discharges (95% CI 0.00- 0.3). In the period 2005 to 2010, the incidence was 0.86 patients per year with 1.1/10 000 discharges (95% CI 0.5-2.4). There was a pulmonary mucormycosis case (in a patient treated with corticosteroids) and nine rhinocerebral cases, two in neutropenic and seven in diabetic patients. The diagnosis was made by observation of cenocytic hyphae in 10/10 patients. Mucorales were recovered in 8/9 cultures (5 Rhizopus spp and 3 Mucor spp.). In one case diagnosis of pulmonary mucormycosis was made post-mortem. Nine patients were treated with amphotericin B deoxycholate (in 3 patients supplemented with liposomal amphotericin B) and surgery. Three patients underwent hyperbaric chamber. Seven patients had favorable outcome. In conclusion, mucormycosis is a rare disease, but its incidence has increased over the past five years. A good evolution of the patients is linked to early diagnosis and treatment.

Mucormicosis cutánea primaria: a propósito de dos casos. Revisión de la literatura / Primary cutaneous mucormycosis: two case reports and literature

Mucormycosis or zygomycosis is a rare opportunistic infection caused by aerobic saprophytic fungus that belongs to the class of Zygomycetes Mucorales family. These organisms live in the environment and enter the body by air, gastrointestinal or skin routes, through solutions of continuity of the skin. This microorganism is generally not pathogenic for immunocompetent hosts, being the development of the disease linked with the immune status of the subject. Its mortality is around 50-60 percent; sometimes in spite of early diagnosis and treatment initiation it has a fatal course. Six clinical forms of mucormycosis are described: rhinocerebral, cutaneous, pulmonary, disseminated, gastrointestinal and miscellaneous form. Two cases of patients with primary cutaneous mucormycosis diagnosed in the Pathology Unit of Hernan Henriquez Aravena Hospital of Temuco, Chile are presented here.

La mucormicosis o zigomicosis es una infección oportunista poco frecuente, causada por un hongo sapró-fito aeróbico que pertenece a la clase Zygomycetes de la familia Mucorales. Estos microorganismos viven en el ambiente y penetran en el organismo por vía aérea, gastrointestinal o cutánea a través de soluciones de continuidad de la piel. Este agente generalmente no es patógeno para el hospedero inmunocompetente estando relacionado el desarrollo de la enfermedad con el estado inmune del sujeto. Su mortalidad es cercana a 50-60 por ciento; en ocasiones, a pesar del precoz diagnóstico e instauración del tratamiento, tiene un curso fatal. Se describen seis formas clínicas de mucormicosis: rinocerebral, cutánea, pulmonar, diseminada, gastrointestinal y una forma miscelánea. Se presentan dos casos de pacientes con mu-cormicosis cutánea primaria diagnosticados en la Unidad de Anatomía Patológica del Hospital Hernán Henríquez Aravena de Temuco.

Fatal disseminated mucormycosis in a patient with mantle cell non-hodgkin's lymphoma: an autopsy case

A patient with mantle cell non-Hodgkin's lymphoma presented herself with fever, nausea, right upper quadrant pain on the 7th day of R-CHOP chemotherapy. After hospitalization with the suspicion of acute cholecystitis, she received antibiotherapy with G-CSF because of emerging neutropenia at the 10th day of chemotherapy. Abdominal computed tomography revealed small infarcts in the spleen and kidneys. The echymotic lesion which developed on her right lateral malleolus, became bullous in the following days and treated as ecthyma gangrenosum. Altough the patient was afebrile with a normal neutrophil count on the third day of antibiotherapy, she developed acute renal failure and deteriorated rapidly. The patient underwent hemodialysis but expired on the 10th day of hospitalization. Post mortem autopsy findings showed ischemic infarction and necrosis of parenchyma due to mycotic thrombosis of arteries and veins of many organs (heart, lung, diaphgram, kidneys, spleen, gut mucosa) as well as invasion of vessel walls and parenchyma by mucor. We reviewed mucormycosis in the light of this case.

Mucormicosis: infección necrotizante como complicación de dermolipectomía abdominal / Mucormycosis: necrotizing infection as a complication of abdominal dermolipectomy

Las infecciones necrotizantes de los tejidos blandos varían en su presentación clínica, pero en general son de curso grave y alta mortalidad. El cuadro clínico incluye: fiebre, celulitis, edema, crepitación, necrosis y sepsis; con frecuencia existe un antecedente o traumático quirúrgico. Como hallazgo operatorio se encuentra necrosis de la piel y tejido subcutáneo, con o sin mionecrosis. El tratamiento es una inmediata y amplia debridación acompañada con antibioticoterapia de amplio espectro. Se presenta un caso clínico de infección necrotizante de tejidos blandos por mucor, en una mujer con antecedente de abdominoplastia y lipoescultura con progresión crítica y evolución fatal. La mucormicosis es una patología agresiva, cuyo diagnostico clínico no es fácil por lo cual debe ser considerada seriamente por el cirujano plástico.

Hepatic and small bowel mucormycosis after chemotherapy in a patient with acute lymphocytic leukemia

Mucormycosis is a rare but invasive opportunistic fungal infection with increased frequency during chemotherapy-induced neutropenia. The clinical infections due to Mucor include rhinocerebral, pulmonary, cutaneous, gastrointestinal and disseminated diseases. The first two are the most common diseases and all entities are associated with a high mortality rate. Still hepatic involvement of Mucor is rarely reported. We experienced a case of hepatic and small bowel mucormycosis in a 56-year-old woman after induction chemotherapy for B-cell acute lymphocytic leukemia. Initial symptoms were a high fever unresponsive to broad spectrum antibiotics and pain in the left lower abdominal quadrant. It was followed by septic shock, deterioration of icterus and progressively elevated transaminase. An abdominal CT demonstrated multiple hypodense lesions with distinct margins in both lobes of liver and pericolic infiltration at small bowel and ascending colon. Diagnosis was confirmed by biopsy of the liver. The histopathology of the liver showed hyphae with the right-angle branching, typical of mucormycosis. The patient was managed with amphotericin B and operative correction of the perforated part of the small bowel was performed. However, the patient expired due to progressive hepatic failure despite corrective surgery and long-term amphotericin B therapy.

Micosis Nasosinusal. Espectro de la Enfermedad / Rhino-sinusal mycosis: range of the disease

Introducción. Las micosis de senos paranasales se han clasificado en invasiva y no invasiva; el tipo de micosis y su comportamiento clínico depende de la interacción entre la competencia inmunológica del paciente y la patogenicidad del microorganismo, en un determinado micro ambiente. Material y Métodos. Se realizó un estudio clínico prospectivo para evaluar las características de pacientes con sinusitis micótica. Se presentan todos los pacientes diagnosticados y tratados como sinusitis micótica en el periodo de 1994-1997. Resultados. Se encontraron 10 pacientes que correspondieron al 6 por ciento de los casos de sinusitis crónica tratados quirúrgicamente; Todos los pacientes con mucormicosis aguda invasiva recibieron tratamiento medico con anfotericina B, Insulina y refección quirúrgica con debridación extensa del tejido necrótico, resultando en control de la enfermedad subyacente y sobrevida del 50 por ciento de los pacientes. Conclusión. Es necesario diagnosticar precozmente, y dar tratamiento multi disciplinario médico y quirúrgico para lograr una mejor supervivencia de los pacientes

Mucormicosis: estudio de cinco casos hallados en los hospitales San Juan de Dios y Sor María Ludovica, La Plata, Argentina / Mucormicosis: study of five cases found in San Juan de Dios and Sor María Ludovica Hospitals of La Plata, Argentina

Se estudiaron cinco casos de micromicosis en los hospitales San Juan de Dios y Sor María Ludovica de La Plata entre los años 1980 y 1997. La mucormicosis se presenta como una enfermedad oportunista, grave, frecuentemente mortal, que afecta a individuos con una afección primaria que favorece la invasión del microorganismo (diabetes, inmunodepresión, linfomas, leucemia, quemados, etc.). En este trabajo presentamos tres casos de mucormicosis cutáneas en niños de 3, 10 y 11 años de edad, secundarias a politrautamismos y fracturas expuestas sufridas en accidentes automovilísticos; un caso de mucormicosis pulmonar en un adulto de 28 años de edad que había recibido corticoterapia prolongada y una mucormicosis rinocerebral en un paciente de 38 años de edad, cuya enfermedad primaria era una leucemia linfoide aguda