Erdheim-chester Disease
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چکیده
DEFINITION The histopathologic characteristics of ECD overlap xanthogranuloma and distinctions between the two are made on clinical and radiologic findings. Lipid-laden histocytes with foamy or eosinophilic cytoplasm infiltrate bones and various organs and generate a fibroblastic response that leads to critical organ failure. The histocytes are CD68+, Factor XIIIa+ CD1a-, S100-, and lack Birbeck granules. Touton-like giant cells are commonly found.
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 کاملRare Presentation of a Rare Disease (Erdheim-Chester disease): A Case Report.
ABSTRACT Erdheim-Chester disease (ECD) was first reported by J. Erdheim and W. Chester, in 1930. There are less than 250 reported cases till date. We report a case of ECD in a 16- year-old Malay male, who initially presented with elusive anemic symptoms with more specific symptoms of bony pain, cardiorespiratory and hepatic involvement evolving as the disease progressed. KEY WORDS Erdheim-Che...
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Erdheim-Chester disease is a rare form of non-Langerhans cell histiocytosis characterized by the migration and infiltration of lipid-laden CD68, CD1a and S100 histiocytes to various target organs, which leads to the disruption of physiological tissue architecture and reactive fibrosis, and thus impairs organ function.We describe the first case of a patient with Erdheim-Chester disease with mult...
متن کاملGastrointestinal Erdheim-Chester disease.
We report a rare case of Erdheim-Chester Disease, a non-Langerhans cell histiocytosis. A 60-year old female presented with a seven-month history of vague abdominal symptoms. A large retroperitoneal mass was detected on computed tomography (CT), but multiple CT-guided biopsy samples were inconclusive. Laparoscopy revealed a mass in the distal ileum, which was resected. Histology and immuno-histo...
متن کاملErdheim-Chester disease, moving away from the orphan diseases: A case report
With approximately 750 cases reported, Erdheim-Chester disease is an exceedingly rare histiocyte cell disorder. Affected sites typically include long bones, large vessels and central nervous system. However, cutaneous and pulmonary involvement can also occur. The diagnosis is ascertained by identification of foamy histiocytes positive for CD68, CD163, and factor XIIIa on immunoperoxidase staini...
متن کاملErdheim-Chester disease: a rare cause of interstitial lung disease.
Erdheim-Chester disease is a non-Langerhans cell histiocytosis that is progressive and may lead on to multi-organ involvement. Pulmonary involvement is rare, its presentation is nonspecific, and it carries an adverse outcome. Several radiological features, when considered together, may point to the diagnosis. This condition should be considered in the differential diagnosis of interstitial lung...
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