Clinical advances in adult orbital xanthogranulomatous disease

Jia-Ying Zhang, Jin Li
2017 Guoji Yanke Zazhi  
Adult orbital xanthogranulomatous disease is a group of rare orbital and ocular adnexal disorders, which is classified as class II non-Langerhans histiocytic proliferations. This disease can be classified into 4 subtypes based mainly on systemic involvement: adult-onset xanthogranuloma, necrobiotic xanthogranuloma, Erdheim-Chester disease and adult-onset asthma and periocular xanthogranuloma. Diagnosis depends on its characteristic clinical manifestations and pathologic features. Therapeutic
more » ... res. Therapeutic approaches are derived from anecdotal evidences, which include corticosteroid, immunosuppressive agent, surgical debulking and chemotherapy. The management of this disease varies with different subtypes, as well as associated systemic presentations. This review summarizes advances of etiopathogenesis, clinical features, diagnosis and treatment.
doi:10.3980/j.issn.1672-5123.2017.12.20 doaj:c10be21311294eef95d6b251ba3ebcdc fatcat:amxonqsduvg33by7cgsvuqdxmu