Malakoplakia, a rare granulomatous disorder, was reported for the first time in a patient with systemic sclerosis in a recent case report.
The report, “Bladder Malakoplakia in Systemic Sclerosis Patient: A Case Report and Review Literature,” was published in the Journal of Endourology Case Reports.
Systemic sclerosis is a rare connective tissue disease that manifests in the skin and blood vessels, affecting all systems of the body. Patients exhibit a thickening of the skin and may develop internal organ involvement, such as pulmonary fibrosis, or scarring of the lung tissue; pulmonary hypertension, which is increased blood pressure within the arteries of the lungs; or kidney failure.
Malakoplakia is a rare inflammatory disease that predominantly affects the genitourinary tract system, which includes the reproductive organs and urinary system. It is a granulomatous disease, meaning a condition characterized by masses of immune cells called granulomas that form at sites of infection or inflammation.
Although the mechanisms that lead to the development of malakoplakia are uncertain, it is thought to develop due to chronic infection — particularly in patients whose immune systems are compromised.
In this case report, physicians in Thailand present the first-ever case of malakoplakia in a systemic sclerosis patient.
A 66-year-old woman went to the hospital with avascular necrosis, the death of bone tissue due to an interruption of the blood supply, in the left hip, and panniculitis, inflammation of the fatty layer under the skin. The patient was later diagnosed with diffuse systemic sclerosis and pulmonary fibrosis.
She was given methotrexate, an immunosuppressant, and prednisolone, a steroid. The next year, another immunosuppressant agent, cyclophosphamide, was added to her treatment regimen. At this point, the patient’s systemic sclerosis was under control, and there was no evidence of further organ involvement.
Two years later, however, she was complaining of hematuria, or blood in urine, and dysuria, which is pain or difficulty urinating. Clinical examination showed no other abnormalities except skin thickening due to systemic sclerosis.
Since there were no abnormalities observed in her kidney, ureter, and bladder system using ultrasonography, researchers conducted a cystoscopy — a procedure where the doctor examines the inside of the bladder and the urethra, the tube that carries urine out of the body. This test showed a white-yellowish plaque in the bladder.
Another test also showed the presence of macrophages (immune cells) and mixed inflammatory cells, indicative of a granulomatous disorder, such as malakoplakia.
Researchers also identified the presence of granules with deposits of iron and calcium, known as Michaelis–Gutman bodies, in a group of cells called the von Hansemann cells. This finding confirmed a diagnosis of malakoplakia.
Cyclophosphamide treatment was permanently halted, and the patient was treated with oral antibiotics. After resolution of the hematuria and dysuria, the patient continued a preventive dose of the antibiotic trimethoprim-sulfamethoxazole, as well as prednisolone and methotrexate.
The team performed a literature search among published scientific reports and concluded: “To the best of our knowledge, this is the first case report of malakoplakia in a systemic sclerosis patient.”
They emphasized that “physicians treating scleroderma patients with lower urinary tract symptoms or hematuria should be vigilant if the condition does not resolve after treatment. Although it is uncommon, it is important to maintain a suspicion for malakoplakia, as this is the key to diagnosis and treatment.”