Malakoplakia Clinical Presentation

Updated: Apr 16, 2019
  • Author: Amira M Elbendary, MBBCh, MSc; Chief Editor: William D James, MD  more...
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Presentation

History

Patients typically present with a history of immunosuppression due to renal transplantation, [15] diabetes mellitus, or lymphoma or a history of receiving long-term therapy with systemic corticosteroids. [16, 17]

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 infection [18] and AIDS, [19] which may be because of the preservation of monocytic antimicrobial function in these patients.

Malakoplakia can mimic colonic, [20, 21, 22, 23, 24] gastric, [25] pancreatic, [26] oropharyngeal, [27, 28] or genital tract carcinoma. [29, 30] Involvement of the bone [31] and the lungs has also caused diagnostic difficulty. Malakoplakia can present with renal failure [32, 33] or a pyelonephritislike picture. [34]

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Physical Examination

Malakoplakia lesions may present as erythematous papules, subcutaneous nodules, draining abscesses, ulcers, nonhealing surgical wounds, indurated plaque, or draining fistulas. [35, 36, 37, 38]

Malakoplakia was also reported masquerading as pyoderma gangrenosum, presenting as a nonhealing malleolar ulcer for 2 years. [39] The most common site affected in the reported cases was perianal (19%). Other affected sites included the abdomen, face, neck, and vulva. [40] In one study, perianal and inguinal skin, vulva, and scrotum were affected in 41% of patients with cutaneous malakoplakia, followed by abdominal wall, thorax, and head and neck at 20% and by extremities and axilla at 10%. [41]  

The most common presentation in reported cases of cutaneous malakoplakia is nodules (23%), with about one quarter of these lesions complicated by abscess or ulcer formation.

Rarely, malakoplakia involves more than one anatomical site in the same patient. [42]

Duration varies from weeks to months and, rarely, years.

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Complications

Complications include local disfigurement and organ dysfunction with visceral involvement.

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