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Erdheim-Chester Disease: The Importance of Information Integration
Conclusion: This case highlights the need for developing a high index of suspicion for presentations of retroperitoneal fibrosis thatcould represent IgG4-related disease but fail to respond to steroids. When unusual multisystem involvement occurs, one should consider a diagnosis of a rare histiocytosis. Vemurafenib appears to be an effective treatment for even advanced cases of both ECD and Langerhans histiocytosis bearing the BRAF V600E mutation.Case Rep Oncol 2017;10:613 –619
Source: Case Reports in Oncology - July 11, 2017 Category: Cancer & Oncology Source Type: research

Association between Ischemic Stroke and Erdheim –Chester Disease: A Case Report and Review of Literature
We report the case of a 68-year-old woma n who presented with an acute ischemic stroke associated with a multisystemic disorder including insipidus diabetes, infiltration of the aorta and the carotid arteries, perirenal infiltration, aortitis, and lytic bone lesions.
Source: Journal of Stroke and Cerebrovascular Diseases - June 13, 2017 Category: Neurology Authors: Guillaume Fargeot, Sabrina Stefanizzi, Sebastien Depuydt, Frederic Klapczynski, Alain Ameri Tags: Case Studies Source Type: research

Long-term outcome of basilar stenosis in Erdheim–Chester disease: A case report
Conclusion: Stroke is a rare treatable and potentially reversible complication of ECD. The pathophysiological processes explaining stroke in this disease are uncertain, but periarterial stenosis of cerebral arteries may be a mechanism.
Source: Medicine - September 1, 2016 Category: Internal Medicine Tags: Research Article: Clinical Case Report Source Type: research