Adult xanthogranulomatous disease of the orbit and ocular adnexa: new immunohistochemical findings and clinical review

摘要

Background/aimsAdult xanthogranulomatous disease involving the ocular tissues is rare and poorly understood. Adult onset xanthogranuloma (AOX), adult onset asthma and periocular xanthogranuloma (AAPOX), necrobiotic xanthogranuloma (NBX), and Erdheim‐Chester disease (ECD) are the four syndromes within this disorder, which is diagnosed by characteristic histopathology. Experience with eight cases prompted a multi‐institutional effort to study the histopathology, immunohistochemistry, clinical findings, and systemic associations in this disorder.