eMedicine Specialties > Dermatology > Bacterial Infections

Malakoplakia

Author: Raphael J Kiel, MD, Associate Professor of Medicine, Wayne State University School of Medicine; Consulting Staff, Infectious Diseases Division, William Beaumont Hospital
Contributor Information and Disclosures

Updated: Oct 21, 2008

Introduction

Background

Malakoplakia is an inflammatory condition presenting as a plaque or a nodule that usually affects the genitourinary tract but may rarely involve the skin. Malakoplakia was first described in the early 1900s as yellow soft plaques that were seen on the mucosa of the urinary bladder. Microscopically, malakoplakia is characterized by the presence of foamy histiocytes with distinctive basophilic inclusions, which are known as Michaelis-Gutmann bodies.

Cutaneous malakoplakia is rare but presents in patients who are immunocompromised and have defects in macrophage function. Lesions are yellow-to-pink papules, but they can present as nodules or ulcerations and are often diagnosed only after biopsy. Cultures of the lesions can yield bacteria, most commonly Escherichia coli.

A related eMedicine article is Bladder, Cystitis.

Pathophysiology

Malakoplakia is believed to result from the inadequate killing of bacteria by macrophages or monocytes that exhibit defective phagolysosomal activity. Partially digested bacteria accumulate in monocytes or macrophages and lead to the deposition of calcium and iron on residual bacterial glycolipid. The presence of the resulting basophilic inclusion structure, the Michaelis-Gutmann body, is considered pathognomonic for malakoplakia.

Studies have suggested that a decreased intracellular cyclic guanosine monophosphate (cGMP) level may interfere with adequate microtubular function and lysosomal activity, leading to an incomplete elimination of bacteria from macrophages and monocytes.

Frequency

United States

The total number of patients with malakoplakia is fewer than 500. Most patients have genitourinary tract disease, although involvement of the gastrointestinal tract and other visceral organs has been described. Cutaneous malakoplakia is rare, with fewer than 50 cases reported in the literature. Because this disease is often diagnosed after biopsy of nondescript lesions, the true incidence is most likely higher.

International

International frequency of malakoplakia is the same as in the United States.

Mortality/Morbidity

Malakoplakia most often occurs in patients who are immunocompromised. Often, malakoplakia is resistant to treatment, with a mean duration of the skin lesions of 3-6 months.

  • Mortality in patients with malakoplakia is most often due to an underlying condition.
  • Significant morbidity relates to the chronicity of the condition, which can resist local and systemic therapy. Draining sinuses, persistence of disfiguring skin lesions, and involvement of visceral organs constitute significant morbidity in patients with malakoplakia.

Race

Cutaneous malakoplakia is more commonly reported in whites (39%) than in African Americans (19%) or Asians (12%), although the patient's ethnic group is often not specified in case reports.

Sex

Cutaneous malakoplakia occurs with a male-to-female ratio of 2.3:1.

Age

Cutaneous malakoplakia presents in patients with a broad age range. The median age at the time of presentation is 53 years. Occurrence of the disease is associated more with the presence of an immunocompromised state affecting monocyte and macrophage function than with age.

Clinical

History

  • Patients typically present with a history of immunosuppression due to renal transplantation,1 diabetes mellitus, or lymphoma or a history of receiving long-term therapy with systemic corticosteroids.
  • Close to one quarter of patients present with internal organ involvement, most commonly in the retroperitoneal area, the kidney, the bladder, or the colon. Symptoms reflect the organ system involved, but patients can often present with draining sinuses originating from deeper organ involvement.
  • Malakoplakia rarely presents in patients with HIV infection2 and AIDS,3 which may be because of the preservation of monocytic antimicrobial function in these patients.
  • Malakoplakia can mimic colonic,4,5,6,7,8 gastric,9 pancreatic,10 oropharyngeal,11 or genital tract carcinoma. Involvement of the bone12 and the lungs has also caused diagnostic difficulty.

Physical

  • Lesions present as a papule, a plaque, or an ulceration that is yellow to pink or skin colored. Lesions may have associated drainage.
  • Common locations include the perianal or inguinal areas, the buttocks, and the abdominal wall.
  • Draining abscesses/sinuses have been reported to occur near the urethra, the thigh, the vulva, and the perianal area. One patient with malakoplakia who presented with an axillary abscess has been described.
  • Lesions may be clinically misdiagnosed as an abscess, a malignancy,13 a lymphoma, or a benign skin tag.
  • The area may appear fluctuant, or it may present as a solitary tender nodule or a group of papules.
  • Often, lesions are chronic but are not debilitating to the patient.

Causes

Defective macrophage killing of bacteria, most commonly E coli, leads to malakoplakia. Defective macrophage killing results in an accumulation of bacterial degradation products and a granulomatous reaction, which clinically manifests as the formation of a papule, a plaque, or an ulceration. Although E coli is the most common gram-negative bacteria isolated, other enteric bacteria may be found on culture. Staphylococcus aureus, Pseudomonas aeruginosa, and Rhodococcus equi may also be cultured.14

Risk factors for the development of malakoplakia include the following:

  • Prolonged therapy with systemic corticosteroids
  • Organ transplantation1
  • Diabetes mellitus
  • Lymphoma
  • Rheumatoid arthritis

More on Malakoplakia

Overview: Malakoplakia
Differential Diagnoses & Workup: Malakoplakia
Treatment & Medication: Malakoplakia
Follow-up: Malakoplakia
Multimedia: Malakoplakia
References
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References

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Further Reading

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Keywords

malakoplakia, malacoplakia, soft plaque, genitourinary tract plaque, genitourinary tract nodule, GU tract nodule, GU tract plaque, cutaneous malakoplakia, cutaneous malacoplakia

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Contributor Information and Disclosures

Author

Raphael J Kiel, MD, Associate Professor of Medicine, Wayne State University School of Medicine; Consulting Staff, Infectious Diseases Division, William Beaumont Hospital
Raphael J Kiel, MD is a member of the following medical societies: American College of Physicians-American Society of Internal Medicine and American Geriatrics Society
Disclosure: Nothing to disclose.

Medical Editor

Peter Fritsch, MD, Chair, Department of Dermatology and Venereology, University of Innsbruck, Austria
Peter Fritsch, MD is a member of the following medical societies: American Dermatological Association, International Society of Pediatric Dermatology, and Society for Investigative Dermatology
Disclosure: Nothing to disclose.

Pharmacy Editor

David F Butler, MD, Professor of Dermatology, Texas A&M University College of Medicine; Chair, Department of Dermatology, Director, Dermatology Residency Training Program, Scott and White Clinic
David F Butler, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, American Society for Dermatologic Surgery, American Society for MOHS Surgery, Association of Military Dermatologists, and Phi Beta Kappa
Disclosure: 3M Pharmaceutical Grant/research funds Other; Graceway Pharmaceuticals Grant/research funds Other

Managing Editor

Jeffrey J Miller, MD, Associate Professor, Department of Dermatology, Penn State University, Milton S Hershey Medical Center
Disclosure: Nothing to disclose.

CME Editor

Glen H Crawford, MD, Assistant Clinical Professor, Department of Dermatology, University of Pennsylvania School of Medicine; Chief, Division of Dermatology, The Pennsylvania Hospital
Glen H Crawford, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, Phi Beta Kappa, and Society of USAF Flight Surgeons
Disclosure: Nothing to disclose.

Chief Editor

Dirk M Elston, MD, Director, Department of Dermatology, Geisinger Medical Center
Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology
Disclosure: Nothing to disclose.

 
 
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