Malacoplaquia del colon: Rara enfermedad que puede presentarse como un sindrome consuntivo

La malacoplaquia del colon es una causa inusual de diarrea crónica y puede presentarse como una enfermedad consuntiva. A nivel de colon produce lesiones ulceradas y nodulares erosionadas, similares a las de otras enfermedades infecciosas o granulomatosas más comunes. El diagnostico se basa en el estudio anatomopatológico que muestra acúmulos de histiocitos en la lámina propia, en cuyo interior se observan unas inclusiones redondeadas, positivas a la tinción de Von Kossa, denominadas cuerpos de Michaelis-Gutmann. Presentamos el caso de un varón 55 años, sin otras enfermedades asociadas, que debutó con diarrea, baja ponderal y anemia, mostrando una respuesta clínica muy favorable con el uso de antibióticos.

Colonic malacoplakia is an unusual cause of chronic diarrhea, and it may present as a consumptive disease. At the colon, it can induce ulcerative and erosive nodular lesions, that mimic other common granulomatous or infectious diseases. Diagnosis is support in biopsies showing groups of histiocytes, with typical Michaelis-Gutmann inclusions, which are positive with the Von Kossa stain. We present the case of a 55-year-old male, without associated diseases, who presented with diarrhea, weight loss and anemia, showing a very good clinical response to antibiotics.

Malacoplaquia en un adolescente: a propósito de un caso / Malacoplakia in a teenager: A case report

Resumen La malacoplaquia es una enfermedad inflamatoria rara, granulomatosa y crónica, caracterizada por infiltrados agregados de histiocitos conocidos como inclusiones de Michaelis-Gutmann. El sistema urinario es el sitio afectado más común, seguido del tracto gastrointestinal. El pronóstico de la enfermedad depende de la extensión, la localización y la salud subyacente del paciente. Caso: se describe un caso clínico de un paciente masculino de 15 años de edad, sin antecedentes de salud de importancia, que inicialmente presenta la enfermedad a partir de los 7 años con hematoquecia y dolor abdominal de leve intensidad que mejoraba después de la defecación. Se realizó una colonoscopia en la que se visualizaron lesiones pseudopolipoideas en el colon sigmoide, las cuales fueron biopsiadas y reportaron pólipos inflamatorios con focos de agudización, numerosos histiocitos (cuerpos de Michaelis-Gutmann) y cambios reactivos del epitelio que diagnosticaron malacoplaquia. Conclusión: la malacoplaquia es una enfermedad muy rara en personas jóvenes sin enfermedad subyacente. Su prevalencia es desconocida, pero se han descrito más de 700 casos. Puede darse en todas las edades, con edad media al diagnóstico de 50 años. Los casos pediátricos son raros. Existe una mayor incidencia en hombres y no tiene predilección por la raza. El 60% a 80% de los casos afecta al tracto urinario (vejiga, riñones y uretra); el 15%, al tracto digestivo (colon izquierdo, sigma, recto y estómago); y en menor porcentaje, otras zonas menos frecuentes como piel, pulmones y sistema nervioso central. El pronóstico de la enfermedad es generalmente bueno. Presentamos un caso de un joven sano de 15 años de edad sin enfermedad asociada evidente, que ilustra la rareza de esta presentación y la necesidad de un alto nivel de sospecha clínica para diagnosticar la enfermedad.

Abstract Malacoplakia is a rare, granulomatous, chronic inflammatory disease characterized by foamy histiocytic infiltrations known as Michaelis-Gutmann inclusions. The urinary system is the most commonly affected site, followed by the gastrointestinal tract. The prognosis of the disease depends on the extent, location, and underlying health status of the patient. This is the clinical case of a 15-year-old male patient with no significant health history. At age 7, the patient presented with hematochezia and mild abdominal pain that improved after defecation. A colonoscopy was performed, finding pseudopolypoid lesions in the sigmoid colon. Biopsy samples were taken, and the report showed inflammatory polyps with exacerbation foci, numerous histiocytes (Michaelis-Gutmann bodies), and reactive epithelial changes that lead to diagnose malacoplakia. Conclusion: Malacoplakia is a very rare disease in young people with no underlying disease. Its prevalence is unknown, but more than 700 cases have been reported. It can occur at all ages, with a mean age at diagnosis of 50 years. Pediatric cases are rare. There is a higher incidence in men, and it has no racial predilection. Between 60% and 80% of cases involve the urinary tract (bladder, kidneys, and urethra); 15% involve the digestive tract (left colon, sigmoid colon, rectum, and stomach); and, to a lesser extent, other areas such as skin, lungs, and central nervous system may be affected. The prognosis of the disease is usually good. This is the case of a healthy 15-year-old male with no apparent associated disease that illustrates the rarity of this presentation and the need for a high level of clinical suspicion to diagnose the disease.

Malacoplaquia renal. Presentación de caso / Renal Malakoplakia. Case report

Introducción: La malacoplaquia es una enfermedad granulomatosa multisistémica crónica caracterizada por una o múltiples placas blancas en varios órganos del cuerpo, precedida por alguna infección crónica bacteriana. En la mayoría de los casos estudiados se encuentra que la E. Coli contribuye en aproximadamente el 80 % de ellos. Objetivo: Dar a conocer la existencia de esta enfermedad y sus características mediante una revisión de tema, y exponer un caso de malacoplaquia renal en una mujer de 65 años de edad con múltiples comorbilidades asociadas a dicha entidad; se describe la utilización de los métodos diagnósticos, así como su desenlace. Discusión: La malacoplaquia es una patología que se encuentra frecuentemente en el tejido urinario, a pesar de que puede desarrollarse en otros órganos. Su etiopatología no ha sido totalmente explicada; sin embargo, se asume que se da por un defecto en la actividad fagolisosomal, al parecer por alteración en la concentración de guanosín monofosfato cíclico intracelular (cGMP) en macrófagos y monocitos. Los pacientes que cursan con esta patología pueden ser asintomáticos o presentar síntomas inespecíficos. Conclusión: La malacoplaquia es una entidad que, aunque poco frecuente en la población general, debe tenerse en cuenta cuando existe compromiso uni o multifocal del parénquima renal en pacientes con factores de riesgo. Las imágenes pueden no ser concluyentes y hacer sospechar patología tumoral; sin embargo, el estudio histopatológico de la muestra permite un diagnóstico definitivo, lo cual les da la oportunidad a los pacientes de recibir manejo oportuno con excelentes resultados.

Introduction: Malakoplakia is a chronic multisystemic granulomatous disease characterized by the presence of one or multiple white plaques in various organs of the body, preceded by some chronic bacterial infection. In most reported cases it is found that E. coli contributes in approximately 80% of cases. Objective: The objective of this article is to show a case report of this disease and its characteristics through a review of the subject. We present a case of renal Malakoplakia in a 65-year-old woman with multiple comorbidities associated with this entity. We emphasize on the use of the diagnostic methods, as well as the outcome. Discussion: Malakoplakia is a pathology that is usually found in the urinary tissue, although it may develop in other organs. Its cause has not been fully explained and it is assumed to be due to a defect in phagolysosome activity, apparently by alteration in the concentration of intracellular cyclic guanosine monophosphate (Cgmp) in macrophages and monocytes. Patients with this pathology may be asymptomatic or have non-specific symptoms. Conclusion: Malakoplakia is an entity that although rare in the general population should be taken into account when there is uni or multifocal involvement of the renal parenchyma in patients with the potential risk where the role of imaging is to provide a first work-up of the extension, with the final work-up taking place through histopathological studies, giving patients the opportunity to receive timely treatment with excellent results.

Cutaneous malakoplakia: case report and review / Malacoplaquia cutânea: relato de caso com revisão da literatura

Malakoplakia is a rare acquired disease that can affect many systems but is more common in the urogenital tract. Cutaneous malakoplakia is even rarer. It is far more frequent in immunodeficient patients. We report a case of cutaneous malakoplakia in a kidney transplant patient who had recently stopped receiving immunosuppressive therapy to illustrate a review of the relevant recent literature.

Malacoplaquia é uma doença adquirida rara que pode afetar diversos órgãos e sistemas, mas é mais comum no trato urogenital. O acometimento cutâneo é ainda menos frequente. Atinge principalmente imunodeficientes. Relatamos caso de malacoplaquia cutânea em um paciente transplantado renal que havia recentemente deixado de receber a terapia imunossupressora, a fim de ilustrar uma revisão da literatura recente relevante.

Malacoplakia of the Epididymis

Malacoplakia is a chronic inflammatory disease. The disease mainly affects the urinary bladder, although involvement of extravesical sites is increasingly being documented. Most frequently involved is the urinary tract, particularly the urinary bladder, although the testis, epididymis, lungs, bone, colon, prostate, female genital organs, and retroperitoneum can also be involved. Here we report the case of a 61-year-old man with a scrotal mass with histology that was specific for malacoplakia of the epididymis. The histologic workup demonstrated extensive involvement of the epididymis by diffuse infiltrates of large histiocytes with eosinophilic granular cytoplasm and numerous Michaelis-Gutmann bodies, which were diagnostic of malakoplakia. This is the first case of epididymal malacoplakia in our country and the first case of epididymal malacoplakia without concurrent involvement of the testis. There have been few reports of this condition worldwide.

Malacoplaquia diseminada: evoluciónexcepcional de una rara patología / Disseminated malakoplakia: exceptional development of an inusual pathology

La malacoplaquia es una enfermedad granulomatosa crónica que afecta principalmente el tracto genitourinario, si bien puede asentar en cualquier órgano de la economía, habitualmente autolimitada y benigna. Las formas extravesicales presentan un curso másagresivo. El diagnóstico es histopatológico, siendo patognomónicos los cuerpos de Michaelis-Guttmann. Se vincula a infecciones crónicas, enfermedades sistémicas, inmunodepresión y neoplasias. El tratamiento médico es inespecífico y en caso de masas voluminosas se plantea conducta quirúrgica. El pronóstico está íntimamente relacionado a la localización del proceso y a la presencia de enfermedades concomitantes.El caso de malacoplaquia reportado es excepcional por la presentación clínica (HDA reiteradas), por el origen topográfico, el compromiso ganglionar y la diseminación de la afección, así como por el curso evolutivo maligno de la misma.

Malakoplakia is a chronic disease which primarily affects the Genitourinary tract, although it can appear in any body,usually self-limited and benign. Extravesical forms evidencea more aggressive course. Diagnostic is done through histopatology, being Michaelis-Guttmann bodiespathognomonic. It is associated to chronic conditions, systemic diseases, immune depression and neoplasia. Medical treatment is non-specific and in the case of voluminous masses surgery is recommended. Prognosis is closelyrelated to the localization of the process and the present of concomitant diseases.The malakoplakia case reported is unusual given its clinical presentation (repeated high digestive hemorrhage),the topographical origin, lymph node commitment and the dissemination of the condition, as well as its malignevolution.

A malacoplaquia é uma doença granulomatosa crônica que afeta principalmente o trato geniturinário embora possa se desenvolver em outros órgãos; geralmente é autolimitada e benigna. As formas extravesicais são mais agressivas. O diagnósticoé histopatológico, sendo patognomônicos os corpos de Michaelis-Guttmann. Está vinculada a infecções crônicas, doenças sistêmicas, imunodepressão e neoplasmas.O tratamento médico é inespecífico e quando se observam massas voluminosas se indica cirurgia. O prognóstico está estreitamente relacionado à localização do processo e a presença de doenças concomitantes.O caso de malacoplaquia que se descreve apresenta características excepcionais pelo quadro clínico (HDA repetidas), por sua origem topográfica, o compromisso ganglionar e a disseminação, como também pela evolução maligna.

Malacoplaquia renal: relato de caso / Renal malakoplakia: a case report

Relata-se caso clássico de malacoplaquia acometendo rim de paciente de 44 anos. Abordam-se aspectos histopatológicos além da patogenia da lesão.

This is a report of 44 years old patient with renal malakoplakia. We study histopathological aspects and pathogenesis of this lesion.

Malacoplakia of the Prostate / 대한비뇨기과학회지

Malacoplakia is a rare granulomatous inflammatory disease, which most frequently involves the urinary tract and especially the urinary bladder. The prostatic involvement of malacoplakia is rare and only two cases have been reported in the Korean literature. Malacoplakia of the prostate may clinically mimic prostate cancer or chronic prostatitis. We report a case of malacoplakia of the prostate in a 71-year-old man, which was initially misdiagnosed as prostate cancer or prostatitis.

Malacoplakia of the Prostate / 대한비뇨기과학회지

Malacoplakia is a rare granulomatous inflammatory disease that most frequently involves the urinary tract, particularly the urinary bladder and may involve the testis, epididymis, lung, bone, colon, prostate, female genital organs, and the retroperitoneum, etc. A malacoplakia of the prostate is a very rare disease that is associated with a chronic urinary tract infection. It is often misinterpreted as granulomatous prostatitis, and may clinically mimic prostate cancer. The major cellular component of malacoplakia is the macrophage and the diagnostic hallmark is a characteristic inclusion, the Michaelis-Gutmann body. We recently experienced a case of malakoplakia of the prostate in a 74-year-old man, that was found incidentally in a TRUS-guided biopsy to confirm a suspected prostate cancer.

El caso de infecciosas: malacoplaquia renal, duodenal y retroperitoneal: presentacion de un caso y revision del tema / The case of infectious diseases: renal malakoplakia, duodenal and retroperitoneal: a case report and review of the topic

Se presenta el caso de una mujer de 52 años, residente en Medellín, quien consultó al Hospital Pablo Tobón Uribe con historia de Diabetes Mellitus y 2 meses de evolución de fiebre, escalofríos, disminución de peso y presencia de masa retroperitoneal, con compromiso de riñón derecho y duodeno. El diagnóstico de malacoplaquia fue hecho por biopsia y confirmado en el Instituto de Patología de las Fuerzas Armadas de Washington. Este caso corresponde al tercero colombiano y el primero de esta localización. El paciente recibió tratamiento con ciprofloxacina y 2 años después continúa en buenas condiciones de salud.

Here we present a case ofa 52 year oldwoman resident in Medellín, Colombia, who carne for a check up to the Hospital Pablo Tobon Uribe with a history ofDiabetes Mellitus and with two-month symptoms offever, chills, weight loss, and a retroperitoneal mass along with a compromise ofa right kidney and duodenum. The diagnosis of Malacoplakia was done with a biopsy and confirmed at the Pathology Institute of the Armed Forces in Washington.This is the third case diagnosed in Colombia and the frrst one located in this site. The patient received treattnent with ciprofloxacine and two years after treattnent she still in good health conditions.

Bilateral Renal Parenchymal Malacoplakia Presenting as Fever and Acute Renal Failure / 대한신장학회잡지

Malacoplakia is a rare, chronic inflammatory disorder characterized by abnormal macrophage function and cell infiltration into normal tissues. We report a 72 year old male with bilateral renal parenchymal malacoplakia who presented with high fever and acute renal failure. The patient had anemia, leukocytosis, thrombocytopenia and bilateral nephromegaly with reduced renal function. Blood and urine cultures showed no micro-organisms. A kidney biopsy revealed diffuse interstitial histiocytic infiltration with intracellular inclusion bodies(Michaelis-Gutmann body). He was treated with antibiotics, and bethanechol, ascorbic acid without improvement. Following treatment with an intravenous methylprednisolone pulse therapy, clinical improvement was made. Renal parenchymal malacoplakia should be considered in the differential diagnosis when presented with high fever, enlarged kidneys and acute renal failure.

Bilateral Renal Parenchymal Malacoplakia Presenting as Fever and Acute Renal Failure / 대한신장학회잡지

Malacoplakia is a rare, chronic inflammatory disorder characterized by abnormal macrophage function and cell infiltration into normal tissues. We report a 72 year old male with bilateral renal parenchymal malacoplakia who presented with high fever and acute renal failure. The patient had anemia, leukocytosis, thrombocytopenia and bilateral nephromegaly with reduced renal function. Blood and urine cultures showed no micro-organisms. A kidney biopsy revealed diffuse interstitial histiocytic infiltration with intracellular inclusion bodies(Michaelis-Gutmann body). He was treated with antibiotics, and bethanechol, ascorbic acid without improvement. Following treatment with an intravenous methylprednisolone pulse therapy, clinical improvement was made. Renal parenchymal malacoplakia should be considered in the differential diagnosis when presented with high fever, enlarged kidneys and acute renal failure.

A Case of Malacoplakia of the Prostate / 대한비뇨기과학회지

Malacoplakia is an uncommon granulomatous inflammatory disease, which predomina- ntly affected the urinary tract, particularly the urinary bladder. The prostatic involve- ment of malacoplakia is extremely rare and may clinically mimic prostate cancer. A correct diagnosis of malacoplakia can be made by histopathologic findings characterized by accumulations of macrophages containing typical intracytoplasmic inclusions(Michael is-Gutmann bodies). We report a case of prostatic malacoplakia, which was incidentally found in prostate biopsy performed to confirm clinically suspected prostate cancer.

Prostatic malacoplakia. An ultrastructural and immunohistochemical study

A case of malacoplakia of the prostatic gland associated with prostatic nodular hyperplasia from a 69 years old man was presented, and its light and electron microscopic and immunohistochemical features were discussed along with its pathogenesis. This lesion was incidentally found in a transurethral prostatectomy specimen, and consisted of large number of epithelioid cells in which were typical cytoplasmic inclusions known as Michaelis-Gutmann bodies. Ultrastructurally, these inclusions showed a dense, central calcified bodies of various developmental stages. Immunohistochemical study using antilysosomal antibody revealed no lysosomal activity. Based on these findings, we could suspect that main problem for this development of malacoplakia is altered intracellular digestion process of foreign biologic materials.