Alshatti Yaqoub, Alenezi Meshaan
Gastroenterology Department, Adan Hospital Kuwait, Hadiya, Kuwait.
Eur J Case Rep Intern Med. 2024 Dec 16;11(12):005067. doi: 10.12890/2024_005067. eCollection 2024.
Malakoplakia is a rare granulomatous condition that occurs due to defective lysosomal digestion during phagocytosis and can mimic inflammatory bowel disease (IBD) or malignancies, particularly in immunosuppressed patients. We report the case of a 62-year-old male with IgG4-related orbitopathy, who developed persistent diarrhoea and colonic lesions 6 weeks after receiving rituximab therapy for nephrotic syndrome secondary to membranoproliferative glomerulonephritis. Colonoscopy revealed pancolitis with mucosal granularity, loss of vascular pattern, and small nodules, raising initial suspicion for IBD. However, histological analysis of colonic biopsies confirmed malakoplakia with the presence of Michaelis-Gutmann bodies, pathognomonic for this condition. The patient was treated with ciprofloxacin for 2 weeks, and steroids were discontinued, leading to complete symptom resolution and significant histological improvement. During follow-up, the number and size of white lesions decreased, and no Michaelis-Gutmann bodies were detected. This case underscores the importance of maintaining a broad differential diagnosis for gastrointestinal lesions in immunosuppressed patients, as misdiagnosis can result in inappropriate escalation of immunosuppressive therapy. Recognizing the characteristic histopathology of malakoplakia and linking it with clinical findings are critical for timely diagnosis and effective management. This report adds to the limited literature on rituximab-associated malakoplakia, highlighting the unique challenges in its diagnosis and treatment.
Malakoplakia, though rare, should be considered in immunosuppressed patients with atypical gastrointestinal lesions to prevent misdiagnosis as inflammatory bowel disease.Histopathological evidence, such as Michaelis-Gutmann bodies, is essential for diagnosing malakoplakia.Prompt discontinuation of immunosuppressants and targeted antibiotic therapy can lead to clinical and histological resolution.
软斑病是一种罕见的肉芽肿性疾病，由于吞噬作用过程中溶酶体消化缺陷而发生，可模仿炎症性肠病（IBD）或恶性肿瘤，尤其是在免疫抑制患者中。我们报告了一例62岁男性，患有IgG4相关眼眶病，在接受利妥昔单抗治疗继发于膜增生性肾小球肾炎的肾病综合征6周后出现持续性腹泻和结肠病变。结肠镜检查显示全结肠炎，伴有黏膜颗粒状、血管纹理消失和小结节，最初怀疑为IBD。然而，结肠活检的组织学分析证实为软斑病，存在米氏小体，这是该病的特征性表现。患者接受环丙沙星治疗2周，停用类固醇，症状完全缓解，组织学有显著改善。随访期间，白色病变的数量和大小减少，未检测到米氏小体。该病例强调了免疫抑制患者胃肠道病变进行广泛鉴别诊断的重要性，因为误诊可能导致免疫抑制治疗的不适当升级。认识软斑病的特征性组织病理学并将其与临床发现联系起来对于及时诊断和有效管理至关重要。本报告增加了关于利妥昔单抗相关软斑病的有限文献，突出了其诊断和治疗中的独特挑战。
软斑病虽罕见，但在有非典型胃肠道病变的免疫抑制患者中应予以考虑，以防止误诊为炎症性肠病。组织病理学证据，如米氏小体，对于诊断软斑病至关重要。及时停用免疫抑制剂和针对性抗生素治疗可导致临床和组织学缓解。
