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Anais Brasileiros de Dermatologia

Print version ISSN 0365-0596

An. Bras. Dermatol. vol.88 no.3 Rio de Janeiro May/June 2013 

Case Report

Cutaneous malakoplakia: case report and review*

Malacoplaquia cutânea: relato de caso com revisão da literatura

João Paulo Junqueira Magalhães Afonso1 

Patricia Naomi Ando2 

Maria Helena Valle de Queiroz Padilha3 

Nilceo Schwery Michalany4 

Adriana Maria Porro5 

1MD – Dermatologist, Dermatology Department of Paulista Medical School – Federal University of São Paulo – (EPM-UNIFESP) — São Paulo (SP) Brazil.

2MD - Volunteer Dermatologist, Dermatology Department of Paulista Medical School – Federal University of São Paulo – (EPM-UNIFESP) — São Paulo (SP) Brazil.

3MD - Master Degree – Dermatologist, Dermatology Department of Paulista Medical School – Federal University of São Paulo – (EPM-UNIFESP) — São Paulo (SP) Brazil.

4MD – Professor – Pathologist, Pathology Department of Paulista Medical School – Federal University of São Paulo – (EPM-UNIFESP) — São Paulo (SP) Brazil.

5MD – PhD – Professor – Dermatologist, Dermatology Department of Paulista Medical School – Federal University of São Paulo – (EPM-UNIFESP) — São Paulo (SP) Brazil.


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.

Key words: Kidney Transplantation; Malacoplakia; Review; Skin


Malacoplaquia é uma doença adquirida rara que pode afetar diversos órgãos e sistemas, mas é mais comum no trato urogenital. O acometimento cutâneo é ainda menos frequente. Atinge principalmente imunodeficientes. Relatamos caso de malacoplaquia cutânea em um paciente transplantado renal que havia recentemente deixado de receber a terapia imunossupressora, a fim de ilustrar uma revisão da literatura recente relevante.

Palavras-Chave: Malacoplasia; Pele; Revisão; Transplante de Rim


Malakoplakia is a term derived from the Greek, meaning "soft plaque".1 , 2 The disease was first described in 1902 by Michaelis and Gutman.3 It describes a granulomatous process of infectious etiol ogy triggered by bacteria that occurs preferentially in subjects affected by primary or secondary immunodeficiency.4 , 5 The pathogenesis of malakoplakia remains poorly understood, and it is thought to represent an acquired bactericidal defect of macrophages associat ed with infection, immunosuppression, and/or immunosuppressive agentes.4 . 6 , 7

The most common site of occurrence is the urogenital tract, although the condition has also been found to affect the gastrointestinal and respiratory tracts, retroperitoneum, thyroid gland, lymph nodes, bones/joints, middle ear, eyes and brain.4 - 9 The condition has been considered rare, and cutaneous malako plakia is even rarer; the first case was reported by Leclerc and Bernier in 1972.10

We report a case of cutaneous malakoplakia in a kidney transplant recipient and proceed with a review of the topic.


A 51-year-old white man from Brazil, suffering from idiopathic chronic renal failure, presented with a immunosuppressive agentes.4 . 6 , 7 2-year history of asymptomatic cutaneous lesion on the left groin, noticed by his nephrologist during hospitalization due to sepsis caused by catheter infection.

The patient was frequently catheterized at this site since an unsuccessful kidney transplantation 2 years before.

The lesion was a yellow-erythematous-purple plaque measuring around 1 cm in diameter, on the left groin, near a femoral vein catheter (Figure 1).

FIGURE 1 Malakoplakia: Picture of the yellow-erythematous- purple plaque measuring little more than 1 cm in diameter on the left groin of the patient near a femoral vein cateter 

The lesion was sampled for histopathologic and culture studies. The culture results revealed the growth of Providentia spp and Candida albicans. Histopathologic analysis revealed a chronic inflammatory process characterized by sheets of closely packed macrophages containing PAS-positive inclusions (von Hansemann cells) and calcospherites known as Michaelis-Gutmann bodies, as demonstrated by Von Kossa stain, which shows the homogeneous bodies in black (Figures 2A, 2B e 2C). Prussian blue staining demonstrated the presence of hemosiderin inside macrophages, which may explain the purple color of the lesion (Figure 2D).

FIGURE 2 Malakoplakia: A-hematoxylin-eosin (400 X) stain showing the sheets of macrophages. B-von Hansemann cells in PAS stain (400 X)(black arrow). C-Michaelis- Gutmann bodies, shown in black after Von Kossa staining (400 X)(black arrow). D-Prussian blue demonstrates hemosiderin inside macrophages (400 X)  

The patient was treated with surgical excision in association with sulfametoxazol-trimetoprin antibiotic therapy. No evidence of recurrence was detected on 3-year follow-up, as shown in figure 3.

FIGURE 3 Malakoplakia: Left groin picture of the same patient after treatment and 3-year follow-up 


Malakoplakia is a rare granulomatous disease in which a defect of the killing capacity of macrophages after endocytosis is considered to be the central event. Disturbed phagosome-lysosome fusion was suggested, but it is still not clear how and why this disorder happens, and the hypothesis is not fully accepted.11 - 13 We reviewed published articles in indexed periodicals that appeared in a PubMed search performed using the term "cutaneous malakoplakia". Based on Kohl et al. 2008, we added cases of cutaneous malakoplakia published from January 2006 until January 2012, as demonstrated in chart 1.14

CHART 1 Reported cases of cutaneous Malakoplakia 

Reported Cases of Cutaneous Malakoplakia in the Literature*

Case No. Reference No. Age/Sex Location Gross Medical History
1 5 51 y/M Perianal, inguinal, scrotum Nodules and ulcerations Kidney Tx
2 5 67 y/M Right temple Nodule Kidney Tx
3 33 69 y/F Right axilla Ulceration and mass RA, breast carcinoma
4 34 40 y/F Inguinal, broad ligament Ulceration N/A
5 10 64 y/M Perianal Indurated mass RA
6 35 35 y/M Left eyelid Nodule Kidney Tx
7 1 64 y/M Perianal Ulceration Lymphoma
8 36 75 y/F Vulva Ulceration RA
9 37 50 y/F Abdominal wound Polypoid mass N/A
10 38 31 y/M Right axilla Mass HIV
11 9 32 y/M Abdomen Abdomen Abscess Kidney Tx
12 9 44 y/M Perianal and left lung Abscess Kidney Tx
13 9 42 y/M Right axilla Chronic abscess SLE
14 39 70 y/M Buttock Nodule Chronic hepatitis C
15 40 75 y/M Right hand and wrist Abscess N/A
16 41 41 y/M Peritoneal, supraclavicular Cystic mass and firm nodule DM
17 42 74 y/M Perianal Nonhealing lesion MPD
18 43 55 y/M Gluteal cleft Ulcers HIV
19 44 67 y/M Left neck Mass No significant PMH
20 45 81 y/F Frontal mass Irregular plaque DM
21 46 56 y/M Internal canthus of eye Nodule Sarcoidosis
22 32 44 y/F Buttock Nodule Kidney Tx
23 47 60 y/F Nasolabial sulcus Ulceration N/A
24 14 2 mo/M Colorectal and perianal Polypoid masses Immunodeficiency
25 2 68 y/M Left inguinal region Papules N/A
26 2 66 y/M Right axilla Nodule RA, DM
27 48 53 y/F Perineum Papules Kidney Tx
28 49 42 y/M Inguinal region Mass with ulceration Lymphoma
29 50 41 y/M Frontal scalp, right lung Abscess, pulmonary lesions HIV, Hepatitis B
30 51 64 y/F Left neck mass Mass with cavitation Thyroidectomy
31 52 60 y/M Gluteal fold Cutaneous fistula DM
32 53 62 y/M Chest Ulceration N/A
33 54 65 y/M Ureterocutaneous fistula Abdominal fistula N/A
34 19 51 y/M Perianal Nodule Heart Tx
35 55 69 y/M Left arm and flank Ulceration, mass Escherichia coli sepsis
36 56 55 y/F Abdominal wall Papules N/A
37 57 22 y/F Arm Fluctuating mass N/A
38 58 52 y/F Inferior abdomen Fistula with abscess Kidney Tx
39 59 30 y/M Perianal Abscesses Dermatomyositis
40 60 51 y/M Left thigh Mass with draining abscess HIV, DM
41 16 60 y/F Abdominal fold Pink-yellow plaques Healthy
42 17 23 y/M Perianal Pink nodules Healthy
43 18 14 Y/M Gluteal fold Papule Healthy
44 20 55 y/F Right Labia "Boil" (nodule + abscess) Heart Tx
45 27 58 y/M Perianal Erosive plaque Psoriasis
46 61 63 y/F Abdominal wall Fistula Pulmonary sarcoidosis
47 62 83 y/F Neck Goma (nodule + fistula) SLE, RA, Sjogren
48 63 45 y/F Perigenital Papules, nodules and sinuses HIV
49 64 24 y/M Abdominal wall Fistula Psoas abscess (Tuberculosis?)
50 65 87 y/M Buttock Scaly plaques and polypoid nodules No significant PMH
51 66 66 y/M Lower abdomen Abscesses, nodules and fistula Poor overall health
52 current article case 51 y/M Left groin Plaque Kidney Tx

While the majority of subjects are immunodeficient patients, including HIV-infected patients, patients with neoplasia, transplanted patients and others, more recently cases involving previously healthy patients have been reported.15 - 17 Almost all transplanted patient cases refer to kidney recipients, as the one in this article, but two were reported in heart transplant recipients.18 , 19 There are few reports in which prevalence among women is higher (2:1).20 , 21 The age peak occurs between the sixth and seventh decades, being even rarer in children.17 , 22

Approximately 90% of patients have coliform bacteria detected in urine, blood, or tissue, suggesting an infectious cause.4 The most commonly found bacterium is Escherichia coli, but Klebsiella, Proteus, Pseudomonas, Mycobacterium avium, Mycobacterium tuberculosis, Shigella, Staphylococcus aureus and Enterococcus spp were also found.23 , 24 Rhodococcus equi is the most commonly implicated microbe in HIV-infected patients.25 In 75% of cases, the disease affects the genitourinary tract, but other systems have been implicated, including the skin.26

No typical clinical presentation is described, skin presentation varies from papules, plaques, nodules, abscesses with or without fluctuation, and fistula to ulcers, cystic and polypoid masses.14 Therefore, the diagnosis is predominantly confirmed by anatomopathologic and culture studies. Vanbrabant et al.2004 recently described the possibility of using 18fluoro-deoxyglucose positron emission tomography for diagnosis and follow-up.27

Histopathologically, the pathognomonic finding of Michaelis-Gutmann bodies, which represent partially degraded bacterial organisms, can establish the diagnosis. Michaelis-Gutmann bodies are intracytoplasmic, round-ovoid, basophilic, concentric laminated inclusions in macrophages that are typically enlarged and display foamy cytoplasm and eccentric, hyperchromatic, round nuclei, denoted as Hansemann cells.

Differential diagnosis is possible with other infectious diseases or neoplastic and reactive/reparative processes. Infections to consider include tuberculosis, Whipple's disease, lepromatous leprosy, fungus (Cryptococcus), and parasites (leishmaniasis). Special stains for microorganisms and tissue culture are necessary. Reactive and neoplastic processes include Langerhans cell histiocytosis, fibrous histiocytoma, lymphoma, granular cell tumor, xanthoma, foreign-body granuloma, hemophagocytic syndromes, and sarcoidosis.14 Although generally presenting benign self-limited evolution, a fatal outcome is possible, but none was described in cutaneous malakoplakia.28 Pseudomalakoplakia was once described as a proliferation of histiocytes at a previous surgical site, but only as an abstract. No other publications on this theme are found.29

Our observation that the disease developed on a site of recognized trauma and contamination, in accordance with other related cases, highlights the importance of direct inoculation of bacteria in the pathophysiology, since the presence of immunosuppression is necessary, but not sufficient for its development.

There are no prospective comparative studies, probably due to the limited incidence, so approaches to management vary from surgical excision, with or without antibiotics, to the use of antibiotics alone.8 Van der Voort et al. 1996 compared treatments and concluded that surgical excision achieved the higher cure rate (90%), and that, when comparing antibiotics, quinolones seemed to be superior.7 The discontinuation of immunosuppressives and treatment of HIV could also be helpful.7 , 9 Sulfamethoxazole-trimethoprim is also cited as effective.30 It was selected by us due to considerations such as cost, access and drug interaction.

We illustrated this article reporting a case of cutaneous malakoplakia synchronous to previous immunosuppressive therapy, in a subject with no current immunosuppressive treatment. The option for surgical plus antibiotic treatment resulted in cure with no recurrence to date. We also reviewed the literature and counted the reported cases of cutaneous malakoplakia described on chart 1 (including this one).31 - 66


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* Syudy carried out at the Dermatology Department of Paulista Medical School (Departamento de Dermatologia da Escola Paulista de Medicina - UNIFESP) - São Paulo (SP) Brazil.

Received: April 02, 2012; Accepted: June 27, 2012

MAILING ADDRESS: João Paulo Junqueira Magalhães Afonso, Rua Borges Lagoa 508 - Vila Clementino 04038-001 - São Paulo - SP, Brazil. E-mail:

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Conflict of interest: None

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