Ortiz Salvador J M, Subiabre Ferrer D, Pérez Ferriols A
Servicio de Dermatología, Hospital General Universitario de Valencia, Valencia, España.
Servicio de Dermatología, Hospital General Universitario de Valencia, Valencia, España.
Actas Dermosifiliogr. 2017 Jun;108(5):400-406. doi: 10.1016/j.ad.2016.12.005. Epub 2017 Mar 3.
Adult xanthogranulomatous disease of the orbit refers to a heterogeneous group of clinical syndromes with differing degrees of systemic involvement and distinct prognoses. The different syndromes all present clinically with progressively enlarging, yellowish lesions of the orbit. Histologically, the lesions are characterized by an inflammatory infiltrate of foam cells and Touton-type multinucleated giant cells. The xanthomatized histiocytes are CD68, S100, and CD1a. There are 4 clinical forms of xanthogranulomatous disease of the orbit: adult xanthogranulomatous disease of the orbit, adult onset asthma and periocular xanthogranuloma, necrobiotic xanthogranuloma, and Erdheim-Chester disease. The treatment of local lesions are treated with systemic corticosteroids and other immunosuppressors. Vemurafenib, tocilizumab, and sirolimus have shown promising results in systemic disease.
成人眼眶黄色瘤病是一组异质性临床综合征，全身受累程度不同，预后各异。所有不同综合征在临床上均表现为眼眶内进行性增大的黄色病变。组织学上，病变的特征是泡沫细胞和杜顿型多核巨细胞的炎性浸润。黄瘤化的组织细胞表达CD68、S100和CD1a。眼眶黄色瘤病有4种临床类型：成人眼眶黄色瘤病、成人起病的哮喘和眼周黄色瘤、坏死性黄色瘤以及厄尔德海姆-切斯特病。局部病变的治疗采用全身用糖皮质激素和其他免疫抑制剂。维莫非尼、托珠单抗和西罗莫司在系统性疾病治疗中已显示出有前景的结果。
