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Enfermedad xantogranulomatosa orbitaria del adulto. Formas clínicas, evaluación y manejo. / Enfermedad xantogranulomatosa orbitaria del adulto. Formas clínicas, evaluación y manejo. / Adult Xanthogranulomatous Disease of the Orbit: Clinical Presentations, Evaluation, and Management.

Ortiz Salvador, J M; Subiabre Ferrer, D; Pérez Ferriols, A.
Actas Dermosifiliogr; 108(5): 400-406, 2017 Jun.
Artigo em Inglês, Espanhol | MEDLINE | Mar 2017 | ID: mdl-28262109
Resumo: 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.