Cutaneous malakoplakia: case report and review

Abstract

Malakoplakia is a rare acquired disease that can affect many systems but is more common in the urogenital tract. Cutaneous malakoplakia is even rarer. It is far more frequent in immunodeficient patients. We report a case of cutaneous malakoplakia in a kidney transplant patient who had recently stopped receiving immunosuppressive therapy to illustrate a review of the relevant recent literature.

Cutaneous malakoplakia masquerading as pyoderma gangrenosum

“…Although the pathogenesis of the disease is poorly understood, its occurrence is associated more commonly with the presence of an immunocompromised state affecting macrophage function rather than with age. Recent data report that it may occur even in healthy patients.…”

“…Prolonged therapy with systemic corticosteroids and the use of azathioprine and cyclophosphamide also contribute in this manner . The conditions leading to malakoplakia in most cases appear to reflect an acquired defect in cellular immunity , resulting in a defective lysosomal degradation of phagocytised bacteria .…”

“…The lesions may present as yellow‐to‐pink papules, but they can also appear as nodules, draining sinuses or ulcerations . Culture of the lesions can yield bacteria, most commonly coliform bacilli such as Escherichia coli (isolated in a 93%) , but also Klebsiella , Proteus , Pseudomonas , Mycobacterium avium , Mycobacterium tuberculosis , Shigella sp , Staphylococcus auereus , Enterococcus sp and Rhodococcus equi in HIV patients . Malakoplakia, according to Thorning and Vracko , is believed to result from the inadequate killing of bacteria by macrophages because of defective phagolysosomal activity .…”

Nonhealing surgical wound due to cutaneous malakoplakia

“…It can occur in many organ systems, most commonly the urinary (approximately 60%) and gastrointestinal tracts. Cutaneous malakoplakia is rare and clinically heterogeneous, and can present as papules, nodules, ulcers, intertriginous plaques and nonhealing surgical wounds …”

“…Around 90% of patients with malakoplakia have evidence of coliform bacteria (most commonly E. coli ) in their blood, urine or tissue . The failure of macrophages to phagocytose gram‐negative bacteria is thought to be central to the disease pathogenesis, and coexisting immunosuppression is common, particularly from organ transplantation, human immunodeficiency virus infection and diabetes mellitus . The histopathological hallmark is the pathognomic intracytoplasmic Michaelis–Gutmann bodies, which have a characteristic, concentrically layered, ‘targetoid’ appearance (Fig.…”