Bifocal malakoplakia in a patient living with HIV: case report.

BACKGROUND: Malakoplakia is a rare chronic granulomatous disease characterized by the presence of Michaelis-Gutmann bodies (MGBs) within histiocytic aggregates. It predominantly affects immunocompromised individuals, including those living with Human Immunodeficiency Virus (HIV). CASE PRESENTATION: We present a unique case of bifocal malakoplakia in a 49-year-old man, previously with Coronavirus disease 2019 (COVID-19) and HIV positive, presented with respiratory symptoms, weight loss, and lymphadenopathy. He had various infections including Non-Tuberculous Mycobacteria (NTM), Cytomegalovirus (CMV), and Candida, with evolving lung and gastrointestinal issues. Despite treatment attempts, he deteriorated due to respiratory distress, multi-organ failure, and coagulopathy, leading to his unfortunate demise. CONCLUSION: This report presents a distinctive and complex case of malakoplakia in an HIV-positive patient, a rare inflammatory disorder originally described by Michaelis and Gutmann in 1902. The hallmark Michaelis-Gutmann organisms were observed, confirming the diagnosis. While typically affecting the urinary tract, this case demonstrates the exceptional ability of malakoplakia to manifest in various organ systems, including pulmonary, gastrointestinal, and more. Although Escherichia coli is a prevalent associated pathogen, the exact cause remains elusive. Treatment, often involving surgical excision and antibiotic therapy, underscores the challenging nature of managing this condition in immunocompromised individuals.

A very complicated UTI: malakoplakia following E. coli urinary tract infection.

Malakoplakia is a rare inflammatory disorder believed to result from a defect in macrophage phagocytic function triggering a granulomatous reaction. It can present with genitourinary, gastrointestinal, or cutaneous manifestations in immunocompromised or, less commonly, immunocompetent hosts. We describe a case of renal malakoplakia in a young, otherwise healthy patient presenting with nephromegaly and sepsis following an E. coli urinary tract infection. We discuss diagnosis and management, including antibiotic selection and the decision to pursue nephrectomy. This case highlights the potential for kidney recovery with prolonged antibiotic therapy in conjunction with adjunct immunomodulatory therapies and source control.

Successful treatment of malakoplakia of the liver and skin in a pediatric liver transplant patient.

BACKGROUND: Malakoplakia occurs uncommonly at any age, but pediatric reports are exceptionally limited. Malakoplakia appears primarily in the urinary tract, although involvement of essentially all organs has been reported, cutaneous malakoplakia is very uncommon and liver involvement is the rarest. METHOD: We report the first pediatric case of concurrent hepatic and cutaneous malakoplakia in a pediatric liver transplant recipient. We also provide a literature review for cutaneous malakoplakia cases in children. RESULT: A 16-year-old male received a deceased-donor liver transplant for autoimmune hepatitis, present with the persistence of the liver mass of unknown etiology and cutaneous plaque-like lesions around the surgical scar. Core biopsies taken from the skin and abdominal wall lesions demonstrated histiocytes containing Michaelis-Gutmann bodies (MGB) revealing the diagnosis. The patient successfully was treated with antibiotics alone for 9 months without surgical intervention or a decrease in immunosuppressive therapy. CONCLUSION: This case demonstrates the need to include malakoplakia in the differential diagnosis of mass-forming lesions after solid transplantation and increase awareness of this very rare entity in pediatrics.

Cutaneous malakoplakia arising on the ankle of a patient with pyoderma gangrenosum.

Malakoplakia is a rare chronic inflammatory condition that most commonly involves the urogenital tract. Cutaneous malakoplakia is extremely rare and many patients diagnosed with skin involvement are immunosuppressed. While the clinical presentation of cutaneous malakoplakia is variable, the histopathologic features are quite distinct and include sheets of closely packed dermal histiocytes with foamy-appearing cytoplasm and Michaelis-Gutmann bodies that are positive with certain immunohistochemical stains. While the exact pathogenesis of malakoplakia is unknown, it has been associated with certain bacterial infections. Treatment generally involves a combination of surgery and antimicrobial agents and/or modulation of immunosuppressant therapy if appropriate. Herein, the authors report a unique case of cutaneous malakoplakia arising in a patient on chronic immunosuppressive therapy for the management of pyoderma gangrenosum.

A Surgical Challenge Generated by Colonic Malakoplakia in Disguise as a Locally Advanced Colonic Malignancy-A Case Report.

Colonic malakoplakia is an uncommon granulomatous development of cells resulting from the impaired capacity of the mononuclear cells to eliminate the phagocytosed bacteria, and in rare cases it can also affect the gastrointestinal tract. We report the case of a 78-year-old female patient that was admitted to hospital by The Emergency Department with the diagnosis of bowel obstruction, confirmed by the clinical and paraclinical investigations. We decided to surgically manage the case for suspicious symptomatic colonic neoplasm. The histological examination of the surgical specimens revealed colonic malakoplakia, characterized by the presence of the aggregated granular histiocytes and Michaelis-Gutmann bodies. Through this paper, we want to raise awareness for Malakoplakia, which remains an extremely rare disease that may affect multiple organs, and because it does not present specific symptoms or clinical manifestations, the final diagnosis remains the histopathological study. The clinical conduct should be decided after taking into consideration all the aspects of this pathology along with the benefits and risks for the patient.

Prostatic malakoplakia: clinicopathological assessment of a multi-institutional series of 49 patients.

Prostatic malakoplakia (MP) is rare, with only case reports and small series (< five patients) available in the literature. In this study we analysed an international multi-institutional series of 49 patients with prostatic MP to more clearly define its clinicopathological features. The median age was 67 years and the median serum prostate-specific antigen (PSA) was 7.5 ng/ml. MP was clinically manifest in most cases (28 of 45 patients with data available, 62%). Of 43 patients with detailed clinical history available, 21 (49%) had concurrent or metachronous malignancies (including prostate cancer). Diabetes or insulin resistance was present in 11 patients (26%). Additionally, three patients had a history of solid organ transplantation and one had HIV. Of note, six of 34 patients (18%) without concurrent prostate cancer had an abnormal digital rectal examination and/or lesions on magnetic resonance imaging (MRI) with prostate imaging reporting and data system (PIRADS) scores 4-5. The initial diagnosis was made on core biopsies (25 of 49, 51%), transurethal resection specimens (12 of 49, 24%), radical prostatectomies (10 of 49, 20%), Holmium-laser enucleation (one of 49, 2%) and cystoprostatectomy (one of 49, 2%). Tissue involvement was more commonly diffuse or multifocal (40 of 49, 82%). Von Kossa and periodic acid-Schiff stains were positive in 35 of 38 (92%) and 26 of 27 lesions (96%), respectively. Of note, two cases were received in consultation by the authors with a preliminary diagnosis of mesenchymal tumour/tumour of the specialised prostatic stroma. The present study suggests that prostatic MP is often associated with clinical findings that may mimic those of prostate cancer in a subset of patients. Moreover, MP may be found incidentally in patients with concurrent prostate cancer.

Colonic malakoplakia in a dual stem cell and cardiac transplant recipient: A case report and literature review.

We describe the first published case of malakoplakia in a dual stem cell and cardiac transplant recipient. In the 2 months following cardiac transplantation, our patient developed persistent diarrhea and recurrent E coli bacteremia. Biopsies obtained from areas of colonic thickening revealed malakoplakia. Despite improvement in symptoms with prolonged antimicrobial therapy and reduction of his immunosuppression, he eventually died from sepsis. Our case highlights not only the importance of the timely diagnosis of this rare disorder, but also the difficulty in determining optimal treatment duration, particularly where excision of involved areas is not possible, as data on this disease are lacking. Here we describe our case and review the available literature published on malakoplakia in the cardiac transplant population.

Malakoplakia in the Urinary Bladder of 4 Puppies.

Malakoplakia in humans most often affects the urinary bladder and is characterized by inflammation with von Hansemann-type macrophages, with or without Michaelis-Gutmann bodies, and is frequently associated with Escherichia coli infection. We describe the microscopic features of malakoplakia in the urinary bladder of 4 puppies. In all cases, the lamina propria of the urinary bladder was markedly expanded by sheets of large, round to polygonal macrophages with intracytoplasmic, periodic acid-Schiff-positive granules and granular inclusions, and rare Prussian blue-positive inclusions. Macrophages were positive for CD18 and Iba1. In 2 cases, Michaelis-Gutmann bodies were detected with hematoxylin and eosin stain and were best demonstrated with von Kossa stain. E. coli infection was confirmed in 2 cases with bacterial culture or polymerase chain reaction (PCR) and sequencing of the bacterial 16S ribosomal RNA gene. Transmission electron microscopy of one case demonstrated macrophages with abundant lysosomes, phagolysosomes, and rod-shaped bacteria. Microscopic features were similar to human cases of malakoplakia. In dogs, the light microscopic characteristics of malakoplakia closely resemble granular cell tumors and histiocytic ulcerative colitis.

[Penile malakoplakia associated with lichen sclerosus]. / Malakoplakie du pénis associée à un lichen scléreux.

INTRODUCTION: Malakoplakia is a granulomatosis of infectious origin in reaction to a chronic bacterial infection (most commonly urinary Escherichiacoli) related to an acquired phagocytosis impairment. PATIENTS AND METHODS: We report a case of penile malakoplakia in a 69-year-old man with lichen sclerosis and stenosis of the urinary meatus leading to recurrent urinary tract infections. The clinical aspect was suggestive of squamous cell carcinoma of the glans developing on lichen sclerosus, but histological examination revealed penile malakoplakia. DISCUSSION: Malakoplakia of the genital mucosa is rare, with only one case of involvement of the glans being reported in the literature. The association with lichen sclerosus is probably not fortuitous but could in fact be due to chronic urinary tract infection favored by stenosis of the urethral meatus and possible local immunodepression following prolonged application of clobetasol. CONCLUSION: We report a case of penile malakoplakia associated with chronic E. coli urinary tract infection, due originally to associated genital lichen sclerosus.

Malakoplakia of the urinary system.

Malakoplakia is regarded as a chronic granulomatous inflammatory disease with a good prognosis. It usually affects the urinary system, especially the urinary bladder. Bacterial infections, including E. coli are thought to be the main factor in pathogenesis. It frequently coexists with chronic diseases and immunosuppression state. Histopathological examination of affected tissue samples is thought to be the best diagnostic method. The basic microscopic feature is mixed inflammatory infiltration containing foamy histiocytes known as von Hansemann cells, frequently with basophilic inclusions known as Michaelis-Gutmann bodies. Symptoms and the clinical course of malakoplakia depend on location and the extent of the lesions. The lesion is treated successfully with antibiotic therapy and surgical excision.

Malakoplakia after kidney transplantation: Case report and literature review.

Malakoplakia is a granulomatous disease associated with an infectious etiology, usually involving the urinary tract. It reveals itself as a recurrent urinary tract infection (r-UTI), and in some cases, it is associated with impairment of renal function. Immunosuppression is one of its main associated factors, and it has been increasingly described in patients with solid organ transplantation (SOT), mainly kidney transplantation. Macroscopically, it can form masses and sometimes it may be confused with neoplasia, which is why histological findings are fundamental for the diagnosis. Here, we present a case of bladder malakoplakia, manifested by r-UTI from Escherichia coli in a patient with renal transplantation, refractory to long-term antibiotic treatment and reduction in immunosuppression, which resolved after surgical management. We also summarize the clinical characteristics of malakoplakia and compare them with previous reports in the literature on SOT.

Granulomatous & histiocytic dermatitides.

Granulomas of the skin may be classified in several ways. They are either infectious or non-infectious in character, and they contain areas of necrobiosis or necrosis, or not. Responsible infectious agents may be mycobacterial, fungal, treponemal, or parasitic organisms, and each case of granulomatous dermatitis should be assessed histochemically for those microbes. In the non-infectious group, examples of necrobiotic or necrotizing granulomas include granuloma annulare; necrobiosis lipoidica; rheumatoid nodule; and lupus miliaris disseminates faciei. Non-necrobiotic/necrotizing and non-infectious lesions are exemplified by sarcoidosis; foreign-body reactions; Melkersson-Rosenthal syndrome; Blau syndrome; elastolytic granuloma; lichenoid and granulomatous dermatitis; interstitial granulomatous dermatitis; cutaneous involvement by Crohn disease; granulomatous rosacea; and granulomatous pigmented purpura. Histiocytic dermatitides that do not feature granuloma formation are peculiar reactions to infection, such as cutaneous malakoplakia; leishmaniasis; histoplasmosis; lepromatous leprosy; rhinoscleroma; lymphogranuloma venereum; and granuloma inguinale.

Cutaneous malakoplakia masquerading as pyoderma gangrenosum.

Cutaneous malakoplakia is a rare infection-related granulomatous disease frequently associated with immunocompromised states. Foamy macrophages containing basophilic granules, called the Michaelis-Gutman bodies, are pathognomonic. We report a case of cutaneous malakoplakia in a 77-year-old male with pyoderma gangrenosum and a 2-year history of a non-healing malleolar ulcer treated successfully with cotrimoxazole.

Malakoplakia associated with prostatic adenocarcinoma: Report of 4 cases and literature review.

Malakoplakia is an inflammatory process that has been rarely reported in the prostate. Malakoplakia in association with prostatic carcinoma is exceedingly rare with only 4 previously reported cases. We describe the clinical features and the associated pathology in 4 patients who demonstrated malakoplakia of the prostate in association with prostatic adenocarcinoma. Prostatic malakoplakia presenting in association with prostatic adenocarcinoma was identified in 4 patients through a search from the records of 3 institutional databases with large in-house and consult uropathology practices. In 2 of the patients the diagnostic needle biopsy contained only prostatic carcinoma; malakoplakia in association with prostatic carcinoma was documented on prostatectomy, performed 15 and 8weeks after the biopsy, respectively. Both patients experienced urinary infections during the interval between the biopsy and the prostatectomy. The third and fourth patient had a long-standing history of "prostatitis", and acute urinary tract infection with urinary retention, respectively. The needle biopsy in both patients showed concomitant malakoplakia and prostatic carcinoma. One of them also had malakoplakia on the initial biopsy containing only atypical glands and on the subsequent one demonstrating carcinoma. One patient was treated conservatively and one with prostatectomy. Although coexistent prostatic carcinoma and malakoplakia are exceedingly rare, malakoplakia can likely occur as an exceptionally rare complication of a prostate needle biopsy, particularly in individuals with long-term or acute urinary tract infections at the time of the biopsy.

Testicular malakoplakia: a rare sonographic mimic of malignancy.

Testicular malakoplakia is a rare, benign condition that most often occurs in an immunocompromised patient with chronic infection. Its occurrence is acute, with pain and testicular enlargement. Sonography is commonly performed to evaluate the patient for epididymitis or torsion. The appearance of testicular malakoplakia on sonography examination has only rarely been described, making its diagnosis challenging. Because its appearance overlaps with testicular neoplasm and infection, it should be considered when making a differential diagnosis of a diffuse testicular abnormality in the appropriate clinical setting.

Successful treatment of renal allograft and bladder malakoplakia with minimization of immunosuppression and prolonged antibiotic therapy.

Malakoplakia is an unusual granulomatous inflammatory disorder associated with diminished bactericidal action of leucocytes that occurs in immunosuppressed hosts. Cases of renal allograft malakoplakia are generally associated with a poor graft and patient survival. We present the case of a 56-year-old female with allograft and bladder malakoplakia occurring two years after renal transplantation complicated by an early antibody mediated rejection. Following a number of symptomatic urinary tract infections caused by resistant Gram-negative bacilli, a diagnosis of malakoplakia was made by biopsy of a new mass lesion of the renal allograft. Cystoscopy also revealed malakoplakia of the bladder wall. Immunosuppressant regimen was modified. Mycophenolate mofetil was ceased, prednisolone reduced to 5 mg/day and tacrolimus concentrations were carefully monitored to maintain trough serum concentrations of 2-4 µg/L. Concurrently, she received a prolonged course of intravenous antibiotics followed by 13 months of dual oral antibiotic therapy with fosfomycin and faropenem. This joint approach resulted in almost complete resolution of allograft malakoplakia lesions and sustained regression of bladder lesions on cystoscopy with histological resolution in bladder lesions. Her renal function has remained stable throughout the illness. If treated with sustained antimicrobial therapy and reduction of immunosuppression, cases of allograft malakoplakia may not necessarily be associated with poor graft survival.

Malakoplakia with digestive tract involvement in a pig.

Malakoplakia is a rare, granulomatous, inflammatory disease that mimics malignant tumors and can affect any organ. Herein is described a case of malakoplakia in a 10-month-old slaughter pig. Diffuse, pleomorphic, round cell infiltrates, mainly histiocytes, with a tumor-like growth pattern at gross examination, infiltrated the stomach, pancreas, omentum, and mesenteric lymph nodes. The histiocytes and multinucleated giant cells had concentric, target-like inclusions known as Michaelis-Gutmann bodies. Microorganisms were not detected by the periodic acid-Schiff reaction, Ziehl-Neelsen, Gram, and Warthin-Starry staining or by electron microscopic and bacteriologic investigations. Porcine circovirus type 2 and porcine reproductive and respiratory syndrome viruses were not detected by immunohistochemistry in the sections examined.

Endoscopic mucosal resection of a rectal malakoplakia in a healthy adult.

Malakoplakia in the gastrointestinal tract is rare in healthy young people without underlying disease. Sufficient tissue is required for accurate diagnosis. We describe a malakoplakia that developed in a healthy young woman and was treated by endoscopic mucosal resection (EMR). A 40-year-old woman with a history of taking oral contraceptives until one year earlier was referred to our hospital with anal bleeding and constipation. A colonoscopy carried out at our another hospital 18 months earlier disclosed no abnormal findings. Colonoscopy at presentation revealed a yellowish-white tumor, 5 mm in diameter, in the rectum. The lesion was slightly protruded and had a smooth flat surface, without erosion or ulceration. EMR was carried out for a definitive diagnosis. Histopathological examination showed that the tumor contained granular histiocytes, positive for CD68 and negative forcytokeratin (AE1/AE3). Several histiocytes contained intracytoplasmic round bodies (Michaelis-Gutmann bodies), which reacted positively with periodic acid-Schiff and calcium (Von Kossa) stains. Intracytoplasmic Escherichia coli (von Hansemann bodies) were identified by Giemsa staining. Based on these results, the tumor in the rectum was diagnosed as a malakoplakia. Following EMR, the patient did not receive further treatment for malakoplakia because she had no symptoms associated with malakoplakia. She has been well for more than 9 months, with no symptoms of disease. Awareness of colorectal malakoplakia is important in patients taking steroids, including oral contraceptives.