Green Michael B, Daly Mary K, Laver Nora M V, Lefebvre Daniel R
Veterans Affairs Boston Healthcare System, Department of Ophthalmology, 150 South Huntington Avenue, Boston, MA, 02130, USA.
Boston University School of Medicine, Department of Ophthalmology, 72 East Concord Street, Boston, MA, 02118, USA.
Am J Ophthalmol Case Rep. 2021 Mar 21;22:101043. doi: 10.1016/j.ajoc.2021.101043. eCollection 2021 Jun.
To present a case of adult onset asthma with periocular xanthogranuloma (AAPOX), and discuss existing literature on adult orbital xanthogranulomatous diseases (AOXGDs) and their treatment.
A 63 year old male presented with progressive bilateral eyelid swelling with overlying yellow plaques associated with asthma. CT scan showed periorbital swelling with enlargement of the superior and lateral rectus muscles bilaterally. Biopsy demonstrated orbital xanthogranulomatous disease with increased IgG4 plasma cells. The patient was treated with intralesional triamcinolone, oral prednisone, and cyclophosphamide without significant improvement. Surgical debulking was eventually performed which improved his external symptoms until he was lost to follow up 15 months later.
AOXGDs are a group of rare infiltrative diseases of the eyelids and orbit that can be associated with significant systemic morbidities. While they all have similar underlying histopathologic features, appreciating the clinical difference between these diseases is important in understanding patient prognosis and ensuring appropriate clinical monitoring. There is also growing research demonstrating that AAPOX, along with other AOXGDs, may represent part of a continuum of IgG4 related disease, similar to what is seen in this case. There is currently no reliably effective treatment for AOXGDs, and additional research into the management of these diseases is necessary.
报告一例成人发作性哮喘合并眶周黄色瘤（AAPOX）的病例，并讨论关于成人眼眶黄色瘤性疾病（AOXGDs）及其治疗的现有文献。
一名63岁男性出现双侧眼睑进行性肿胀，伴有与哮喘相关的黄色斑块。CT扫描显示眶周肿胀，双侧上直肌和外直肌增粗。活检显示眼眶黄色瘤性疾病，IgG4浆细胞增多。患者接受了病灶内注射曲安奈德、口服泼尼松和环磷酰胺治疗，但无明显改善。最终进行了手术减容，改善了其外部症状，直到15个月后失访。
AOXGDs是一组罕见的眼睑和眼眶浸润性疾病，可伴有严重的全身疾病。虽然它们都有相似的潜在组织病理学特征，但认识这些疾病之间的临床差异对于理解患者预后和确保适当的临床监测很重要。也有越来越多的研究表明，AAPOX以及其他AOXGDs可能是IgG4相关疾病连续谱的一部分，本病例即如此。目前对于AOXGDs尚无可靠有效的治疗方法，有必要对这些疾病的管理进行更多研究。
