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Inicio Archivos de la Sociedad Española de Oftalmología (English Edition) Necrobiotic xanthogranuloma. Differential diagnosis, treatment and systemic invo...
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Vol. 89. Issue 5.
Pages 186-189 (May 2014)
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1964
Vol. 89. Issue 5.
Pages 186-189 (May 2014)
Short communication
Necrobiotic xanthogranuloma. Differential diagnosis, treatment and systemic involvement. Case report
Xantogranuloma necrobiótico. Diagnóstico diferencial, tratamiento e implicaciones sistémicas. A propósito de un caso
Visits
1964
E. Sanz-Marcoa,
Corresponding author
empar19@hotmail.com

Corresponding author.
, E. Españaa,b, M.J. López-Pratsa, M. Chirivella-Casanovac, J. Aviñoa, M. Díaz-Llopisa,d,e
a Servicio de Oftalmología, Hospital Universitario La Fe, Valencia, Spain
b Facultad de Óptica, Universidad de Valencia, Valencia, Spain
c Servicio de Anatomía Patológica, Hospital Universitario La Fe, Valencia, Spain
d Departamento de Cirugía, Facultad de Medicina, Universidad de Valencia, Valencia, Spain
e Centro de Investigación de Enfermedades Raras (CIBERER), Valencia, Spain
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Abstract
Case report

A 48-year-old male was referred to our hospital for further evaluation of eyelid edema with bilateral yellowish ulcerated nodules. Suspecting a xanthogranulomatosis, imaging tests and biopsy were performed with diagnosis of necrobiotic xanthogranuloma. IgG monoclonal gammapathy was diagnosed in a systemic study. Systemic corticosteroids and cyclosporine were initiated unsuccessfully; therefore, intra-lesional injections of triamcinolone were started, which controlled the orbital disease.

Discussion

Necrobiotic xanthogranuloma is a rare condition that usually affects the eyelids and anterior orbit. Its diagnosis is important as it is associated with malignant lymphoproliferative processes. Intra-lesional corticosteroids were effective in our patient.

Keywords:
Xanthogranuloma
Necrobiotic xanthogranuloma
Xanthogranulomatosis
Corticosteroids
Monoclonal gammapathy
Resumen
Caso clínico

Varón de 48 años remitido por edema palpebral con nódulos ulcerados amarillentos en ambos párpados superiores. Las técnicas de imagen demostraron infiltración en ambas órbitas anteriores que fueron biopsiadas con el resultado de xantogranuloma necrobiótico. En el estudio sistémico se halló una gammapatía monoclonal IgG no conocida. Se inició tratamiento con corticoides y ciclosporina orales sin éxito, por lo que se pautaron inyecciones intralesionales de triamcinolona que controlaron la enfermedad orbitaria.

Discusión

El xantogranuloma necrobiótico es una rara entidad que suele afectar a párpados y órbita anterior. Su diagnóstico es importante porque se asocia a procesos linfoproliferativos malignos. Los corticoides intralesionales fueron efectivos en su manejo.

Palabras clave:
Xantogranuloma
Xantogranuloma necrobiótico
Xantogranulomatosis
Corticoides
Gammapatía monoclonal

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