Noh Geunwoong, Han Chi Wha
Allergy and Clinical Immunology Center, Cheju Halla General Hospital, Jeju City, Jeju, South Korea.
Department of Internal Medicine, Jeju National University Hospital, Jeju City, Jeju, South Korea.
Am J Case Rep. 2021 Jan 4;22:e929519. doi: 10.12659/AJCR.929519.
BACKGROUND Pfeifer-Weber-Christian disease (PWCD), also referred to as idiopathic nodular panniculitis, is a rare idiopathic disease characterized by lobular panniculitis of adipose tissue with systemic symptoms and multiple organ involvement and is usually treated with corticosteroids and cyclosporine A. We report a case of PWCD that was unresponsive to standard treatment but responded to intravenous immune globulin (IVIG) therapy. CASE REPORT A 35-year-old Korean woman presented with fever, malaise, myalgia, and painful nodules in the left breast. Histology of the breast nodules showed lobular panniculitis consistent with PWCD. She did not respond to corticosteroid and cyclosporine A. She was effectively treated with intravenous immune globulin (IVIG). IVIG therapy began with 60 g (1 g/kg) 4 times per week, 2 times every other week. Subsequently, the IVIG dose was reduced for maintenance therapy to 25 g (400 mg/kg) twice every other week and monthly. The patient showed immediate and dramatic improvement. General signs and symptoms, such as fever, malaise, and myalgia, were absent, and the masses had nearly subsided, with several very small hard nodules remaining for 3 months until the time of this report. CONCLUSIONS IVIG was an effective immunomodulatory therapeutic for PWCD in this case. This report shows that PWCD is a rare condition that is difficult to diagnose, but the histopathology of nodular panniculitis supports the diagnosis. In cases that do not respond to standard immunosuppressive therapy, including corticosteroids and cyclosporine A, IVIG therapy may lead to a favorable response with rapid symptomatic relief.
费弗尔-韦伯-克里斯蒂安病（PWCD），也称为特发性结节性脂膜炎，是一种罕见的特发性疾病，其特征为脂肪组织的小叶性脂膜炎伴全身症状和多器官受累，通常采用皮质类固醇和环孢素A进行治疗。我们报告一例对标准治疗无反应但对静脉注射免疫球蛋白（IVIG）治疗有反应的PWCD病例。病例报告：一名35岁的韩国女性出现发热、不适、肌痛以及左乳疼痛性结节。乳房结节的组织学检查显示与PWCD一致的小叶性脂膜炎。她对皮质类固醇和环孢素A治疗无反应。她接受静脉注射免疫球蛋白（IVIG）治疗后效果良好。IVIG治疗开始时为每周4次，每次60 g（1 g/kg），每两周2次。随后，IVIG剂量减至每两周2次，每次25 g（400 mg/kg）用于维持治疗，并每月一次。患者立即出现显著改善。发热、不适和肌痛等一般体征和症状消失，肿块几乎消退，仅残留几个非常小的硬结节，持续3个月直至本报告撰写之时。结论：在该病例中，IVIG是治疗PWCD的一种有效的免疫调节疗法。本报告表明，PWCD是一种难以诊断的罕见疾病，但结节性脂膜炎的组织病理学支持诊断。在对包括皮质类固醇和环孢素A在内的标准免疫抑制治疗无反应的病例中，IVIG治疗可能会带来良好反应并迅速缓解症状。
