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Vol. 108. Issue 5.
Pages 400-406 (June 2017)
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Vol. 108. Issue 5.
Pages 400-406 (June 2017)
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DOI: 10.1016/j.adengl.2017.03.030
Adult Xanthogranulomatous Disease of the Orbit: Clinical Presentations, Evaluation, and Management
Enfermedad xantogranulomatosa orbitaria del adulto. Formas clínicas, evaluación y manejo
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J.M. Ortiz Salvador
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Josema.ortiz.salvador@gmail.com

Corresponding author.
, D. Subiabre Ferrer, A. Pérez Ferriols
Servicio de Dermatología, Hospital General Universitario de Valencia, Valencia, Spain
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Abstract

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.

Keywords:
Adult xanthogranulomatous disease of the orbit
Histiocytosis
Granulomatous inflammatory diseases
Foam cells
Touton-type multinucleated giant cells
Resumen

La enfermedad xantogranulomatosa orbitaria del adulto comprende un grupo heterogéneo de síndromes clínicos con diferentes grados de afectación sistémica y pronóstico variable.

Todas las formas se manifiestan clínicamente como lesiones amarillentas infiltradas orbitarias de crecimiento progresivo. Histológicamente se caracteriza por un infiltrado inflamatorio compuesto fundamentalmente por histiocitos espumosos y células gigantes multinucleadas tipo Touton. Estos histiocitos xantomatizados son CD68+, S100-y CD1a-.

Existen 4 formas clínicas de enfermedad xantogranulomatosa orbitaria del adulto: el xantogranuloma orbitario del adulto, el asma del adulto asociado a xantogranulomas orbitarios, el xantogranuloma necrobiótico y la enfermedad de Erdheim-Chester.

El tratamiento de las lesiones locales se basa fundamentalmente en corticosteroides sistémicos y otros inmunosupresores. En los casos con enfermedad sistémica vemurafenib, tocilizumab y sirolimus ofrecen resultados prometedores.

Palabras clave:
Enfermedad xantogranulomatosa orbitaria del adulto
Histiocitosis
Enfermedades inflamatorias granulomatosas
Histiocitos espumosos
Células gigantes multinucleadas tipo Touton

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