Multisystem Radiologic Manifestations of Erdheim-Chester Disease
نویسندگان
چکیده
منابع مشابه
Multisystem Radiologic Manifestations of Erdheim-Chester Disease
Erdheim-Chester Disease is a rare form of multiorgan non-Langerhans' cell histiocytosis that affects individuals between the ages of 50 and 70 with an equal distribution among males and females. It is associated with significant morbidity and mortality that is mostly due to infiltration of critical organs. Some of the sites that Erdheim-Chester Disease affects include the skeletal system, centr...
متن کاملErdheim-Chester Disease: Case Report with Aggressive Multisystem Manifestations and Review of the Literature
Erdheim-Chester disease (ECD) is an extremely rare and aggressive form of non-Langerhans cell histiocytosis. ECD usually presents with bone pain in adults aged 40-60. Its etiology is unknown but it is thought to be either a reactive or neoplastic disorder. Recently, mutation of the proto-oncogene BRAF (BRAFV600E) has been found in more than 50% of cases. The multisystemic form of ECD is associa...
متن کاملNeurological manifestations in Erdheim-Chester disease: Two case reports.
Erdheim-Chester disease (ECD) is a rare form of non-Langerhans cell histiocytosis characterised by multiorgan xanthogranulomatous infiltration of histiocytes (CD68+/CD1−). Two highly suggestive characteristics of this disease are sclerosis of the long bones and perirenal fat infiltration. The central nervous system (CNS) involvement occurs in 30% to 50% of cases and is the main predictor of a p...
متن کاملErdheim-Chester Disease
Erdheim-Chester disease (ECD) is a rare, xanthogranulomatous, non-Langerhans cell histiocytosis with frequent systemic involvement. Although the diagnosis is based on characteristic histological and radiological findings, its identification can be challenging because of its heterogeneous presentation. Osteosclerosis of long bones, often associated with bone pain, is the most common initial mani...
متن کاملErdheim-chester disease.
Erdheim-Chester disease (ECD) is a rare, non-Langerhans cell histiocytosis of unknown etiology, characterized by multi-organ involvement. ECD is usually diagnosed on the basis of characteristic radiologic and histopathological findings. Lesions may be skeletal and/or extraskeletal in location, and may include the skin, lung, heart, and central nervous system. We describe here a 68-year-old man ...
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ژورنال
عنوان ژورنال: Case Reports in Radiology
سال: 2016
ISSN: 2090-6862,2090-6870
DOI: 10.1155/2016/2670495