Adult Xanthogranulomatous Disease of the Orbit: Clinical Presentations, Evaluation, and Management

• Published in: Actas dermo-sifiliográficas (English ed.) 2017-06, Vol.108 (5), p.400-406
• Main Authors: Ortiz Salvador, J M, Subiabre Ferrer, D, Pérez Ferriols, A
• Format: Article
• Language: eng ; spa
• Subjects: Adrenal Cortex Hormones - therapeutic use; Adult; Antibodies, Monoclonal - therapeutic use; Combined Modality Therapy; Diagnosis, Differential; Disease Management; Erdheim-Chester Disease - diagnosis; Erdheim-Chester Disease - pathology; Erdheim-Chester Disease - therapy; Histiocytosis - diagnosis; Histiocytosis - pathology; Histiocytosis - therapy; Humans; Immunosuppressive Agents - therapeutic use; Necrobiotic Xanthogranuloma - diagnosis; Necrobiotic Xanthogranuloma - pathology; Necrobiotic Xanthogranuloma - therapy; Orbital Diseases - diagnosis; Orbital Diseases - pathology; Orbital Diseases - therapy; Radiotherapy, Adjuvant
• ISSN: 1578-2190
• DOI: 10.1016/j.ad.2016.12.005

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.