Malakoplakia (from Greek ''Malako'' "soft" + ''Plako'' "plaque") is a rare inflammatory condition which makes its presence known as a

papule A papule is a small, well-defined bump in the skin. It may have a rounded, pointed or flat top, and may have a dip. It can appear with a stalk, be thread-like or look warty. It can be soft or firm and its surface may be rough or smooth. Some h ...

plaque Plaque may refer to: Commemorations or awards * Commemorative plaque, a plate or tablet fixed to a wall to mark an event, person, etc. * Memorial Plaque (medallion), issued to next-of-kin of dead British military personnel after World War I * Pla ...

ulceration An ulcer is a discontinuity or break in a bodily membrane that impedes normal function of the affected organ. According to Robbins's pathology, "ulcer is the breach of the continuity of skin, epithelium or mucous membrane caused by sloughing o ...

that usually affects the

genitourinary tract The genitourinary system, or urogenital system, are the organs of the reproductive system and the urinary system. These are grouped together because of their proximity to each other, their common embryological origin and the use of common pathw ...

. However, it may also be associated with other bodily organs. It was initially described in the early 20th century as soft yellowish plaques found on the

mucosa A mucous membrane or mucosa is a membrane that lines various cavities in the body of an organism and covers the surface of internal organs. It consists of one or more layers of epithelial cells overlying a layer of loose connective tissue. It is ...

of the

urinary bladder The urinary bladder, or simply bladder, is a hollow organ in humans and other vertebrates that stores urine from the kidneys before disposal by urination. In humans the bladder is a distensible organ that sits on the pelvic floor. Urine enters ...

. Microscopically it is characterized by the presence of foamy

histiocyte A histiocyte is a vertebrate cell that is part of the mononuclear phagocyte system (also known as the reticuloendothelial system or lymphoreticular system). The mononuclear phagocytic system is part of the organism's immune system. The histiocyt ...

s (called von Hansemann cells) with

basophil Basophils are a type of white blood cell. Basophils are the least common type of granulocyte, representing about 0.5% to 1% of circulating white blood cells. However, they are the largest type of granulocyte. They are responsible for inflammator ...

ic inclusions called

Michaelis–Gutmann bodies Michaelis–Gutmann bodies, (M-G bodies) are concentrically layered basophilic inclusions found in Hansemann cells in the urinary tract. They are 2 to 10 μm in diameter, and are thought to represent remnants of phagosomes mineralized by iron and ...

. It usually involves

gram-negative Gram-negative bacteria are bacteria that do not retain the crystal violet stain used in the Gram staining method of bacterial differentiation. They are characterized by their cell envelopes, which are composed of a thin peptidoglycan cell wall ...

bacteria.

Causes

Malakoplakia is thought to result from the insufficient killing of

bacteria Bacteria (; singular: bacterium) are ubiquitous, mostly free-living organisms often consisting of one biological cell. They constitute a large domain of prokaryotic microorganisms. Typically a few micrometres in length, bacteria were among ...

macrophage Macrophages (abbreviated as M φ, MΦ or MP) ( el, large eaters, from Greek ''μακρός'' (') = large, ''φαγεῖν'' (') = to eat) are a type of white blood cell of the immune system that engulfs and digests pathogens, such as cancer cel ...

s. Therefore, the partially digested bacteria accumulate in macrophages and leads to a deposition of

iron Iron () is a chemical element with symbol Fe (from la, ferrum) and atomic number 26. It is a metal that belongs to the first transition series and group 8 of the periodic table. It is, by mass, the most common element on Earth, right in f ...

and

calcium Calcium is a chemical element with the symbol Ca and atomic number 20. As an alkaline earth metal, calcium is a reactive metal that forms a dark oxide-nitride layer when exposed to air. Its physical and chemical properties are most similar to ...

. The impairment of

bactericidal A bactericide or bacteriocide, sometimes abbreviated Bcidal, is a substance which kills bacteria. Bactericides are disinfectants, antiseptics, or antibiotics. However, material surfaces can also have bactericidal properties based solely on their ...

activity manifests itself as the formation of an

ulcer An ulcer is a discontinuity or break in a bodily membrane that impedes normal function of the affected organ. According to Robbins's pathology, "ulcer is the breach of the continuity of skin, epithelium or mucous membrane caused by sloughing o ...

, plaque or papule. Malakoplakia is associated with patients with a history of

immunosuppression Immunosuppression is a reduction of the activation or efficacy of the immune system. Some portions of the immune system itself have immunosuppressive effects on other parts of the immune system, and immunosuppression may occur as an adverse react ...

due to

lymphoma Lymphoma is a group of blood and lymph tumors that develop from lymphocytes (a type of white blood cell). In current usage the name usually refers to just the cancerous versions rather than all such tumours. Signs and symptoms may include enlar ...

diabetes mellitus Diabetes, also known as diabetes mellitus, is a group of metabolic disorders characterized by a high blood sugar level ( hyperglycemia) over a prolonged period of time. Symptoms often include frequent urination, increased thirst and increased ap ...

kidney transplant Kidney transplant or renal transplant is the organ transplant of a kidney into a patient with end-stage kidney disease (ESRD). Kidney transplant is typically classified as deceased-donor (formerly known as cadaveric) or living-donor transplantati ...

ation, or because of long-term

therapy A therapy or medical treatment (often abbreviated tx, Tx, or Tx) is the attempted remediation of a health problem, usually following a medical diagnosis. As a rule, each therapy has indications and contraindications. There are many different ...

with systemic

corticosteroid Corticosteroids are a class of steroid hormones that are produced in the adrenal cortex of vertebrates, as well as the synthetic analogues of these hormones. Two main classes of corticosteroids, glucocorticoids and mineralocorticoids, are involv ...

Diagnosis

As malakoplakia is a condition associated with chronic

cystitis A urinary tract infection (UTI) is an infection that affects part of the urinary tract. When it affects the lower urinary tract it is known as a bladder infection (cystitis) and when it affects the upper urinary tract it is known as a kidney ...

, it usually presents itself as a cystitis condition. The following investigations may help in making the diagnosis of malakoplakia: *

Urinalysis Urinalysis, a portmanteau of the words ''urine'' and ''analysis'', is a panel of medical tests that includes physical (macroscopic) examination of the urine, chemical evaluation using urine test strips, and microscopic examination. Macroscopic e ...

which indicates presence of bacteria and white blood cells. *

Urine culture Bacteriuria is the presence of bacteria in urine. Bacteriuria accompanied by symptoms is a urinary tract infection while that without is known as asymptomatic bacteriuria. Diagnosis is by urinalysis or urine culture. ''Escherichia coli'' is the ...

* Cystoscopic evaluation confirms presence of ulcer or papule. * Biopsy to rule out other causes. * The presence of large multinucleated malakoplakia giant cells with concretions called as Michaelis Gutman bodies on microscopy.

Treatment

Treatments for malakoplakia include catheterizing the affected person with full aseptic precautions. Additionally, irrigating the bladder with distilled water three times daily with 3 way Foley's catheter and urinary

antiseptic An antiseptic (from Greek ἀντί ''anti'', "against" and σηπτικός ''sēptikos'', "putrefactive") is an antimicrobial substance or compound that is applied to living tissue/skin to reduce the possibility of infection, sepsis, or putre ...

s like

pyridium Phenazopyridine is a medication which, when excreted by the kidneys into the urine, has a local analgesic effect on the urinary tract. It is often used to help with the pain, irritation, or urgency caused by urinary tract infections, surgery, or ...

may be used to help relieve urinary symptoms. Antibiotics such as

trimethoprim-sulfamethoxazole Trimethoprim/sulfamethoxazole, sold under the brand name Bactrim among others, is a fixed-dose combination antibiotic medication used to treat a variety of bacterial infections. It consists of one part trimethoprim to five parts sulfamethoxazo ...

ciprofloxacin Ciprofloxacin is a fluoroquinolone antibiotic used to treat a number of bacterial infections. This includes bone and joint infections, intra abdominal infections, certain types of infectious diarrhea, respiratory tract infections, skin infec ...

may be used.

History

Leonor Michaelis Leonor Michaelis (16 January 1875 – 8 October 1949) was a German biochemist, physical chemist, and physician, known for his work with Maud Menten on enzyme kinetics in 1913, as well as for work on enzyme inhibition, pH and quinones. E ...

and Carl Gutmann first described malakoplakia in 1902 after analyzing a patient's tumor at the request of

David Paul von Hansemann David Paul von Hansemann (5 September 1858 – 1920) was a German pathologist born in Eupen, east Belgium. He is remembered for his work in the field of oncology, in particular, his concept pertaining to anaplasia of cancer cells. He studied ...

References

External links