Department of Dermatology, Asahikawa Medical University, Asahikawa, Japan.
Clin Exp Dermatol. 2011 Aug;36(6):628-31. doi: 10.1111/j.1365-2230.2011.04041.x.
Adult orbital xanthogranulomatous disease (AOXGD) is a rare granulomatous disorder, which has four subtypes: adult-onset xanthogranuloma (AOX), adult-onset asthma with periocular xanthogranuloma, necrobiotic xanthogranuloma and Erdheim-Chester disease. We report a 42-year-old woman who presented with yellowish nonulcerative nodules on her eyelids. On histopathological examination of a nodule, mild degeneration of collagen fibres was seen, with surrounding infiltration of numerous foam cells and Touton giant cells in the deep dermis. Lymphoid follicles were seen in the reticular dermis. There was no apparent necrobiosis of collagen fibres. There were no clinical symptoms of asthma and no laboratory signs of paraproteinaemia during a follow-up of more than 5 years. We diagnosed this case as AOX, but further long-term follow-up would be required for the differentiation from the other AOXGDs. Dermatologists should be aware of these rare granulomatous disease conditions with ocular/orbital location, because they may cause ophthalmological complications.
成人眼眶黄色肉芽肿病（AOXGD）是一种罕见的肉芽肿性疾病，有 4 种亚型：成人起病的黄色瘤（AOX）、伴有眶周黄色瘤的成人起病哮喘、坏死性黄色肉芽肿和 Erdheim-Chester 病。我们报告了 1 例 42 岁女性，其眼睑出现黄色非溃疡性结节。结节的组织病理学检查显示胶原纤维轻度变性，真皮深层可见大量泡沫细胞和 Touton 巨细胞浸润，网状真皮内可见淋巴滤泡。胶原纤维无明显坏死。在超过 5 年的随访中，无哮喘的临床症状，也无副蛋白血症的实验室迹象。我们诊断该病例为 AOX，但需要进行长期随访以与其他 AOXGD 相鉴别。皮肤科医生应该注意这些罕见的具有眼部/眼眶位置的肉芽肿性疾病，因为它们可能引起眼部并发症。
