Seastrom Stacey, Bookout Angela, Hogan Daniel J
Nova Southeastern University College of Osteopathic Medicine, Largo Medical Center, 201 14th St, Largo, FL 33770, USA.
Cutis. 2014 Dec;94(6):293-6.
Necrobiotic xanthogranuloma (NXG) is an indolent non-Langerhans cell histiocytosis characterized by yellow xanthomatous plaques that tend to ulcerate. Necrobiotic xanthogranulomas have a predilection for the bilateral periorbital region and often present with consequential ophthalmic findings. Histopathology usually reveals a distinctive pattern of histiocytic xanthogranuloma with hyaline necrobiosis. Necrobiotic xanthogranuloma has been documented to have a close association with paraproteinemia. We report the case of a 76-year-old man with periorbital NXG without development of a monoclonal gammopathy. Clinically, the patient presented with dry eyes and substantial periorbital edema with multiple yellow indurated plaques. He developed the condition 30 years prior to presentation at which time it was initially diagnosed as xanthelasma. He underwent surgical excision of the lesions 10 years prior to the current presentation and biopsy results revealed a diagnosis of NXG. The periorbital lesions recurred several years prior to presentation, prompting annual computed tomography scans to rule out ocular invasion. Periorbital edema and plaques improved during a 6-month regimen of acitretin but returned to baseline just months after discontinuation.
坏死性黄色肉芽肿（NXG）是一种惰性非朗格汉斯细胞组织细胞增生症，其特征为黄色瘤样斑块，易于溃疡。坏死性黄色肉芽肿好发于双侧眶周区域，常伴有相应的眼部表现。组织病理学检查通常显示具有透明样坏死的组织细胞性黄色肉芽肿的独特模式。坏死性黄色肉芽肿已被证明与副蛋白血症密切相关。我们报告一例76岁男性眶周NXG患者，未发生单克隆丙种球蛋白病。临床上，患者表现为干眼症和严重的眶周水肿，伴有多个黄色硬结斑块。他在就诊前30年就出现了这种情况，当时最初被诊断为睑黄瘤。在本次就诊前10年，他接受了病变的手术切除，活检结果显示诊断为NXG。在就诊前几年，眶周病变复发，促使每年进行计算机断层扫描以排除眼部侵犯。在阿维A治疗6个月期间，眶周水肿和斑块有所改善，但停药后仅数月就恢复到基线水平。
