中国麻风皮肤病杂志 ›› 2024, Vol. 40 ›› Issue (11): 802-804.doi: 10.12144/zgmfskin202411802

• 病例报告 • 上一篇    下一篇

伴IgG-κ型副球蛋白血症的渐进性坏死性黄色肉芽肿一例

赵鹏远,邵开心,王玉坤,于晓静   

  1. 山东大学齐鲁医院皮肤科,山东济南,250012
  • 出版日期:2024-11-15 发布日期:2024-10-15

Necrobiotic xanthogranuloma with immunoglobulin G kappa paraproteinemia: a case report

ZHAO Pengyuan, SHAO Kaixin, WANG Yukun, YU Xiaojing   

  1. Department of Dermatology, Qilu Hospital of Shandong University, Jinan 250012, China
  • Online:2024-11-15 Published:2024-10-15

摘要: 渐进性坏死性黄色肉芽肿临床易误诊为类脂质渐进性坏死、睑黄瘤、环状肉芽肿等。本例患者眼睑及眶周、颈后及腰部皮肤散布黄红色斑块,组织病理符合渐进性坏死性黄色肉芽肿,血清免疫固定电泳示单克隆IgG,Kappa轻链。目前尚无标准治疗,国内外报道的治疗方法包括系统使用糖皮质激素、大剂量注射免疫球蛋白、甲氨蝶呤、沙利度胺、利妥昔单抗、干扰素α以及TNF-α抑制剂和抗疟药物等,本例患者应用甲泼尼龙、沙利度胺、白芍总苷治疗7个月,皮损明显改善,目前仍在继续随访中。

关键词: 渐进性坏死性黄色肉芽肿, 副球蛋白血症, 治疗

Abstract: Necrobiotic xanthogranuloma is easy to be misdiagnosed as progressive lipoid necrosis, xanthelasma palpebrarum, and annular granuloma. In this case, yellow-red plaques were scattered on the eyelid, periorbital skin, neck and waist, and histopathology was consistent with necrobiotic xanthogranuloma. Serum immunofixation electrophoresis showed protein of monoclonal IgG and Kappa light chain. There is no standard treatment and the reported treatment methods included systemic use of glucocorticoids, high-dose injection of immunoglobulin, methotrexate, thalidomide, rituximab, interferon-α, TNF-α inhibitors and antimalarial drugs. This patient was treated with methylprednisolone, thalidomide, and total glucosides of paeony, the skin lesions were significantly improved after 7 months, and the patient is under follow-up.

Key words: necrobiotic xanthogranuloma, paraproteinemia, treatment