RESUMEN
Erdheim-Chester disease is an infiltrative form of histiocytosis characterized by replacement of normal tissues by lipid-laden histiocytes. The disease typically infiltrates the medullary portion of the diaphysis and metaphysis of long bones, producing a characteristic radiological pattern dominated by bone sclerosis. It usually affects adults of 40 years of age with a clinical spectrum ranging from an asymptomatic focal bone lesion to multisystemic disease. This case report documents unique imaging and pathologic findings of Erdheim-Chester disease using close postmortem pathologic-imaging correlation.
Asunto(s)
Huesos/diagnóstico por imagen , Huesos/patología , Enfermedad de Erdheim-Chester/diagnóstico , Imagen por Resonancia Magnética , Enfermedad de Erdheim-Chester/diagnóstico por imagen , Enfermedad de Erdheim-Chester/patología , Humanos , Masculino , Persona de Mediana Edad , RadiografíaRESUMEN
Erdheim-Chester disease (ECD) is a rare form of histiocytosis of unknown origin characterized by tissue infiltration by lipid-laden histiocytes. Typically, the diaphyseal and metaphyseal portions of the tubular bones are affected, leading to a characteristic radiographic pattern of bone sclerosis. Orbital involvement is not infrequent and is manifested by exophthalmos and periorbital xanthomatous lesions, with associated visual problems. This case report documents imaging and pathologic findings in a patient with ECD with extensive orbital involvement.