What is Malakoplakia?
Malakoplakia is a rare inflammatory condition usually found in individuals with weakened immune systems. Named by Michaelis and Gutmann in 1902 after discovering characteristic cells called the Michaelis-Gutmann bodies, the term ‘malakoplakia’ translates to ‘soft plaque’. While mostly affecting the urinary tract, malakoplakia can affect almost all organ systems including lungs, digestive system, hormonal system, musculoskeletal system, lymphatic system, skin, and the brain.
The exact cause of malakoplakia is not fully understood. However, a type of bacteria called Escherichia coli is commonly associated with this condition, although other bacteria have been found related to it as well.
Standard treatment for malakoplakia often includes surgery to remove the affected tissue and, or the use of oral antibiotics. This treatment can be administered in combination or antibiotics can be used alone.
What Causes Malakoplakia?
Malakoplakia is a disease that we don’t fully understand yet. However, scientists believe it might be due to the body’s immune cells, called macrophages, not being able to properly fight off bacteria. Our body needs certain chemicals, specifically Beta-glucuronidase and cyclic guanosine monophosphate (cGMP), to do this effectively. Some patients with malakoplakia have been found to have low levels of these chemicals, which might make it more difficult for their body to get rid of harmful bacteria.
Patients with malakoplakia often have unique structures inside their body cells, known as Michaelis-Gutman bodies. Scientists believe these are remnants of the affected cells’ unsuccessful attempts to get rid of the bacteria.
Malakoplakia often occurs in people with weakened immune systems. That can include people with HIV, those who have had organ transplants, or patients with certain conditions like cancer, diabetes, connective tissue diseases, hepatitis C, or sarcoidosis. Cases in children are rare but can happen, often in those with certain immune deficiencies.
Typically, malakoplakia is caused by a type of bacteria called gram-negative rods. The most common of these is Escherichia coli, but many other types of bacteria could be involved, including Klebsiella, Proteus, Corynebacterium, and others.
Risk Factors and Frequency for Malakoplakia
Malakoplakia is a condition that affects the genital and urinary tract more often in women than in men, with a ratio of about 4 to 1. It’s interesting to note that this gender difference ceases to be apparent when the condition is found outside of these areas. Conversely, the form of malakoplakia that affects the skin is more common in men, with a ratio of 2 to 1. The condition typically occurs most often in older people, specifically those over 50. However, it can affect a wide age range, from as young as 6 weeks old to people as old as 85 years.
Signs and Symptoms of Malakoplakia
Malakoplakia is a skin condition that often affects people with weakened immune systems. This weakening may be due to medications or certain medical conditions. The symptoms of malakoplakia can vary greatly. Some people may notice tender sores, small lumps, raised spots, flat patches on the skin, or growing masses. Although this condition often affects the perianal and genital areas, it can also occur in places like the chest, abdomen, head, neck, and limbs. It’s crucial for doctors to consider malakoplakia when examining patients with weakened immune systems and a history of recurrent infections.
- Sores, lumps, spots, patches, or growing masses on the skin
- Commonly affects perianal and genital areas
- Can also occur on the chest, abdomen, head, neck, and limbs
- Often seen in individuals with weakened immune systems
- Considered in patients with recurring infections
Testing for Malakoplakia
When looking into a condition called cutaneous malakoplakia, a doctor starts by understanding the patient’s history and doing a physical check-up. If there’s a mark on the skin that doesn’t look right, the doctor might take a small sample of it (a skin biopsy) to figure out exactly what it is. This biopsy helps the doctor confirm if it is indeed cutaneous malakoplakia, or if it could be an infectious process or another type of skin condition.
The skin biopsy will be examined for specific clues that suggest cutaneous malakoplakia – these clues are special types of cells known as Michaelis-Gutmann bodies and von Hansemann cells. In addition to the skin biopsy, the doctor might also culture the lesion. This involves growing the microorganisms (like bacteria or fungi) taken from your skin lesion in a lab, something that could help them figure out the best way to treat the condition, especially when it comes to deciding on the right antibiotics.
Treatment Options for Malakoplakia
Since malakoplakia is rare, there aren’t standardized treatment guidelines due to lack of large-scale research trials. Understood from the limited studies and individual experiences, three strategies are typically used for treating malakoplakia which includes antibiotic therapy, surgery, and lessening the use of medicines that dampen the immune response, if possible.
Antibiotics especially those that can boost the efficiency of the immune system’s white blood cells in killing bacteria, have shown success. These include fluoroquinolones, trimethoprim/sulfamethoxazole, and rifampin. It’s not certain how long these antibiotic therapies should last, but it’s suggested that long-term therapy is often effective. Additionally, combining bethanechol, a medicine that affects nerve impulses to certain muscles, and ascorbic acid (also known as Vitamin C) might be beneficial as they potentially enhance the bacteria-killing efficiency of white blood cells even further.
In terms of surgery, the decision to remove malakoplakia lesions depends on where they are in the body and what complications, if any, are present. For example, malakoplakia affecting the urinary tract, especially the kidneys, can be aggressive. If it affects the kidney tissue, removal of the kidney (nephrectomy) might be considered. If there’s any blockage in the urinary system (urethra, bladder, or ureters), surgical excision might be needed. In some scenarios, a combination of surgery and antibiotic therapy can provide the most effective treatment.
Malakoplakia is frequently seen in people with suppressed immune systems, such as individuals who have undergone organ transplants or have connective tissue diseases and are taking certain medications such as prednisone and azathioprine. Observations suggest that the use of these immune-suppressing drugs may lead to a decrease in the ability of white blood cells to kill bacteria, contributing to malakoplakia. Successful treatment of malakoplakia occurs when these medications are either discontinued or reduced in dose, where feasible.
A study reviewing 140 cases of malakoplakia provided a potential treatment approach: first, if possible, stop the use of prednisone and azathioprine in cases where malakoplakia has developed while these drugs were in use. Secondly, consider surgical resection and drainage along with the administration of a fluoroquinolone named ciprofloxacin. If surgery isn’t an option, prescribing ciprofloxacin could be considered. Lastly, whether adding bethanechol and ascorbic acid alongside these treatments offers any advantage remains unconfirmed.
What else can Malakoplakia be?
In diagnosing a medical condition, doctors often look at a variety of possibilities including infections, cancerous growths, and conditions that arise due to the body’s reaction to an abnormal process. Here are some of the conditions that they could consider:
- Tuberculosis
- Lepromatous leprosy
- Cryptococcus
- Actinomycosis
- Botryomycosis
- Leishmaniasis
- Condyloma lata
- Cutaneous Crohn’s disease
- Sarcoidosis
- Foreign body granuloma
- Hidradenitis suppurativa
- Granular cell tumors
- Xanthomas
- Lymphoma
What to expect with Malakoplakia
Cutaneous malakoplakia often shows up as a condition that tends to resolve on its own and seldom causes death. When the immune system is healthy, these lesions (or skin abnormalities) can disappear on their own within 4 to 6 months on average.
If a person undergoes surgical treatment with or without antibiotics, the reported cure rate is about 81%. However, the condition could vary and may come back or cause local disfigurement. Sometimes, it might involve internal organs as well, in spite of medical treatment and surgery.
Possible Complications When Diagnosed with Malakoplakia
The complications related to malakoplakia mostly depend on where it is located in the body and how severe it is. For instance, when malakoplakia affects the urinary system, it can lead to sudden damage to the kidneys, repeated urinary tract infections, kidney failure, and in very rare cases, can even be fatal.
Possible Complications:
- Sudden damage to the kidneys
- Repeated urinary tract infections
- Kidney failure
- Death (in rare cases)
Preventing Malakoplakia
Individuals who have a weakened immune system, due to medical treatment or secondary to another health condition, should be aware of potential side effects or complications. One such complication is malakoplakia, a rare inflammatory condition that can affect many areas of the body, including the skin. It’s crucial for these individuals to tell their healthcare provider if they notice any skin changes like persistent sores, growing spots, or skin ulcers.
