Knausz József, Lipták József, Andrásovszky Zsolt, Baranyay Ferenc
Kanizsai Dorottya Kórház Sebészeti Osztály Nagykanizsa.
Orv Hetil. 2010 Feb 7;151(6):220-3. doi: 10.1556/OH.2010.28785.
Malakoplakia is an acquired granulomatous disorder first described by Michaelis and Gutmann in 1902. The pathogenesis of malakoplakia is hardly known, but it thought to be secondary to an acquired bactericidal defect in macrophages occurring mostly in immunosuppressed patients.
63-year-old female patient had been treated with methylprednisolone for ten years, because of pulmonary sarcoidosis. For six month, recurrent abdominal abscess and vesico-cutaneous fistula developed. Histological examination proved malakoplakia, and Escherichia coli was detected in the abscess cavity.
Hematoxyline eosin staining, periodic acid-Schiff, Berlin-blue and Kossa reactions were performed.
Microscopically malakoplakia consists of mainly macrophages, known as von Hansemann cells with scattered targetoid intracytoplasmic inclusions known as Michaelis-Gutmann bodies. In our presented case, after urological-surgical intervention and antibiotic therapy, the patient became free from complaints and symptoms.
Malakoplakia has been described in numerous anatomic locations, mainly in the urogenital tract. Malakoplakia may be complicated with fistulas in different locations: vesico-coccygeal, rectoprostatic, anorectal fistulas have been were reported in the literature, while 6 cases of malakoplakia with Boeck's sarcoidosis are published.
In the presented case sarcoidosis and the 10-year immunosuppressive treatment with methylprednisolone might have been in the background of abdominal wall malakoplakia, complicated by vesico-cutaneous fistula. The patient was successfully treated with surgery and the followed antibiotic therapy.
软斑病是一种后天性肉芽肿性疾病，于1902年由米夏埃利斯和古特曼首次描述。软斑病的发病机制尚不清楚，但认为是继发于巨噬细胞后天性杀菌缺陷，主要发生在免疫抑制患者中。
一名63岁女性患者因肺结节病接受甲基强的松龙治疗10年。6个月来，反复出现腹部脓肿和膀胱皮肤瘘。组织学检查证实为软斑病，脓肿腔内检测到大肠杆菌。
进行苏木精伊红染色、过碘酸希夫反应、柏林蓝反应和科萨反应。
显微镜下，软斑病主要由巨噬细胞组成，即所谓的冯·汉泽曼细胞，伴有散在的靶样胞质内包涵体，即米夏埃利斯-古特曼小体。在我们报告的病例中，经过泌尿外科手术干预和抗生素治疗后，患者症状消失。
软斑病已在许多解剖部位被描述，主要在泌尿生殖道。软斑病可能在不同部位并发瘘管：文献报道过膀胱尾骨瘘、直肠前列腺瘘、肛门直肠瘘，同时还发表了6例合并结节病的软斑病病例。
在本病例中，结节病和10年的甲基强的松龙免疫抑制治疗可能是腹壁软斑病的背景，并发膀胱皮肤瘘。患者通过手术及后续抗生素治疗成功治愈。
