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.

Malakoplakia of Head and Neck Mimicking Malignant Lymphadenopathy: A Case Report / 대한이비인후과학회지

Malakoplakia is usually found in the genitourinary tract; however, it occurs uncommonly as a chronic inflammatory disease and rarely in the head and neck area, having been reported in the literature only few times. Here, we report, with a review of the related literature, a case of malakoplakia on the posterior neck. A 76-year-old male patient visited our institution presenting a rapidly growing neck mass that had invaded the overlying skin for several weeks. The results of imaging studies strongly indicated a malignant tumor, but an accurate diagnosis was not made until after a fine needle aspiration biopsy was undertaken twice. The lesion was completely excised with an extended radical neck dissection including the overlying skin and scalene muscle upon consent of the patient. The pathological diagnosis was made with various immunohistochemical staining methods including Von Kossa, Periodic acid-Schiff-diastase, CD-68 and CD163. During the 24 months follow-up after the surgery, there was no sign of recurrence.

Reporte de un caso de Malacoplaquia renal: una enfermedad inusual de difícil diagnóstico / Renal malokaplakia: An unusual disease of difficult diagnosis

RESUMEN La malacoplaquia es una enfermedad granulomatosa crónica inicialmente descrita en las vías urinarias, pero posteriormente encontrada en múltiples órganos. La presentación clínica depende del sitio comprometido; en el tracto urinario produce disuria, dolor pélvico o abdominal, fiebre, malestar general y, a veces, sensación de masa. Su etiopatogenia aún no ha sido completamente entendida, pero se cree que es debida a un trastorno en la función lisosomal de los macrófagos que afecta la destrucción de las bacterias. Histológicamente se caracteriza por presentar macrófagos llamados "células de von Hansemann" e inclusiones de Michaelis-Gutmann. Describimos el caso de una paciente con diabetes mellitus de larga evolución y malacoplaquia renal que le produjo síntomas debilitantes crónicos.

SUMMARY Malakoplakia is a chronic granulomatous disease, firstly described in the urinary tract but then it has been reported in many organs. The clinical presentation depends on the affected site, when it affects the urinary tract the symptoms are dysuria, abdominal or pelvic pain, fever, discomfort, and, something mass sensation. The etiopathogenesis is not yet clear, but is believed it is due to lysosomal dysfunction of the macrophages that affects the destruction of bacteria. Histologically it is characterized for the presence of von Hansemann cells and Michaelis-Gutmann bodies. We describe the case of a patient with long-term diabetes mellitus and renal malakoplakia that caused chronic debilitating symptoms.

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.

Malakoplakia of appendix: a case report

We report a case of an extremely rare inflammatory condition of appendix, malakoplakia, in a 22 years old female, who presented with chronic abdominal pain. The clinicopathological features of this condition will be discussed, along with a review of the literature

Bladder Malakoplakia Mimicking Bladder Cancer / 대한내과학회지

Malakoplakia is an extremely rare granulomatous disease caused by infection and known to occur mostly in immune-compromised hosts. The most common site of involvement is the bladder. Patients with malakoplakia of the bladder present with multiple intravesical masses and are often misdiagnosed with bladder cancer. As a result, appropriate treatment is delayed. Here we report a case of a patient with malakoplakia of the bladder that was misdiagnosed as bladder cancer.

Malakoplakia Affecting the Umbilical Cord

No abstract available.

Renal Parenchymal Malakoplakia with Acute Interstitial Nephritis Presented with Acute Kidney Injury / 이화의대지

Malakoplakia is an uncommon chronic granulomatous inflammatory disease which is associated with immunocompromised conditions such as malignancy, autoimmune disease, chronic alcohol intake, poorly controlled diabetes and long-term steroid use. Malakoplakia can occur at various sites, most commonly in the genitourinary tract including urinary bladder and the ureter. Renal parenchymal involvement is relatively uncommon, accounting for 15% of all malakoplakia. A few cases of renal malakoplakia have been reported in Korea, and only one case was accompanied by acute kidney injury. Here we report an 80-year-old female patient with renal parenchymal malakoplakia and acute interstitial nephritis presented as acute kidney injury with literature review.

Malacoplaquia pulmonar associada à imunodeficiência por HTLV-1 e HIV / Pulmonary malakoplakia associated with immunodeficiency by HTLV-1 and HIV

Malakoplakia is a rare chronic inflammatory disease often confused with neoplasia. In this paper we report two cases of pulmonary Malakoplakia, both with typical clinical diagnosis of tuberculosis and lung cancer. A patient with human T-lymphotropic virus type I (HTLV-1) and diagnosis of adult T-cell leukemia/lymphoma, and another patient with human immunodeficiency virus (HIV), which was treated for tuberculosis, but, after pulmonary lobectomy, was evidenced Rodococosis equi, progressed to death...

Malacoplaquia é uma rara doença inflamatória crônica muitas vezes confundida com neoplasia. Neste artigo, relatam-se dois casos de malacoplaquia pulmonar, ambos com quadro clínico sugestivo de tuberculose e neoplasia pulmonar. Uma paciente com vírus T-linfotrópico humano tipo I (HTLV-1) e diagnóstico de leucemia/linfoma de células T do adulto, e um paciente com vírus da imunodeficiência humana (HIV), tratado para tuberculose, mas após lobectomia pulmonar foi evidenciado Rodococose equi, evoluindo para óbito...

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.

Malakoplakia in a healthy young female patient

Malakoplakia is a rare granulomatous disease that occurs commonly in the urinary tract and secondarily in the gastrointestinal tract. Most reported cases of malakoplakia are associated with immunosuppressive diseases or chronic prolonged illness. Here, we report a rare case of malakoplakia in a young healthy adolescent without any underlying disease. A 19-year-old female was referred to our hospital following the discovery of multiple rectal polyps with sigmoidoscopy. She had no specific past medical history but complained of recurrent abdominal pain and diarrhea for 3 months. A colonoscopy revealed diverse mucosal lesions including plaques, polyps, nodules, and mass-like lesions. Histological examination revealed a sheet of histiocytes with pathognomonic Michaelis-Gutmann bodies. We treated the patient with ciprofloxacin, the cholinergic agonist bethanechol, and a multivitamin for 6 months. A follow-up colonoscopy revealed that her condition was resolved with this course of treatment.

Silent Colonic Malakoplakia in a Living-Donor Kidney Transplant Recipient Diagnosed during Annual Medical Examination

Malakoplakia is a characteristic inflammatory condition, which is usually seen in the urogenital tract, and less frequently in the gastrointestinal tract. We present a case of colonic malakoplakia in an immunocompromised patient. A 55-year-old female visited the outpatient clinic for routine cancer surveillance. Her past medical history was significant for kidney transplantation 11 years ago, and she had been taking immunosuppressants. A colonoscopy revealed several depressed flat lesions and elevated polyps, which were 0.3 to 0.4 cm in size and accompanied by whitish exudates. A biopsy revealed an infiltration of histiocytes with ample granular eosinophilic cytoplasm, with some lymphocytes and plasma cells. Many histiocytes had the characteristic morphology, described as Michaelis-Gutmann bodies: one or several round basophilic structures of approximately 1 to 10 microm in size with some being laminated, some appearing homogeneous, and others having a dense central core with a targetoid appearance. These Michaelis-Gutmann bodies were positively stained on von Kossa stain, and were diagnostic for malakoplakia.

Renal Parenchymal Malakoplakia Presenting with Abscesses and Hepatic Extension Misdiagnosed as a Malignant Tumor: A Case Report / 대한내과학회지

Malakoplakia is a chronic inflammatory disorder that usually affects the urinary tract in immunocompromised patients and rarely extends to adjacent organs. Due to its mass-like presentation, malakoplakia is often clinically misdiagnosed as a neoplastic lesion. We describe the case of a 51-year-old female with renal malakoplakia and hepatic extension and large intraperitoneal abscesses that had been misdiagnosed as malignancy. She was diagnosed with myasthenia gravis 12 years prior and had been treated with oral corticosteroids and immunosuppressants. Radical nephrectomy concomitant with abscess drainage was performed. The final pathology was compatible with malakoplakia, and the patient was successfully treated with antibiotics. Although renal malakoplakia is a rare disease, it should be included in the differential diagnosis of patients with a renal mass who have a history of recurrent urinary tract infections or evidence of immunosuppression.

Renal Parenchymal Malakoplakia Presenting with Abscesses and Hepatic Extension Misdiagnosed as a Malignant Tumor: A Case Report / 대한내과학회지

Malakoplakia is a chronic inflammatory disorder that usually affects the urinary tract in immunocompromised patients and rarely extends to adjacent organs. Due to its mass-like presentation, malakoplakia is often clinically misdiagnosed as a neoplastic lesion. We describe the case of a 51-year-old female with renal malakoplakia and hepatic extension and large intraperitoneal abscesses that had been misdiagnosed as malignancy. She was diagnosed with myasthenia gravis 12 years prior and had been treated with oral corticosteroids and immunosuppressants. Radical nephrectomy concomitant with abscess drainage was performed. The final pathology was compatible with malakoplakia, and the patient was successfully treated with antibiotics. Although renal malakoplakia is a rare disease, it should be included in the differential diagnosis of patients with a renal mass who have a history of recurrent urinary tract infections or evidence of immunosuppression.

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.

Pulmonary malakoplakia: A report of two cases.

Malakoplakia of lung is an unusual condition that has been reported to occur in association with immunocompromised state, particularly in those with acquired immunodeficiency syndrome. We present two cases of pulmonary malakoplakia in immunocompetent individuals. The diagnosis was made on histopathological examination of surgically resected specimen.

Malakoplakia of the Kidney Extending to the Descending Colon in a Patient with Secondary Adrenal Insufficiency: A Case Report / 가정의학회지

Malakoplakia is an uncommon but distinctive type of chronic granulomatous inflammation that occurs most commonly in the genitourinary tract, especially the urinary bladder. Most patients have associated conditions characterized by some degree of immunosuppression, as seen in solid-organ transplants, autoimmune diseases requiring steroid use, chemotherapy, chronic systemic diseases, alcohol abuse and poorly controlled diabetes. We report an unusual case of the renal malakoplakia that involved the perirenal space, extending to the descending colon in a 65-year-old Korean woman with secondary adrenal insufficiency and diabetes mellitus.