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Unusual orbital involvement in Erdheim Chester disease: a radiological diagnosis
Arundeep Arora1, Sanjay Sharma, Neelam Pushker
1All India Institute of Medical Sciences, New Delhi, India.
Orbit (Amsterdam, Netherlands)
|May 29, 2012
Abstract:
Erdheim-Chester disease (ECD) is an exceedingly rare, disseminated non-Langerhan cell histiocytosis with multisystem involvement, having characteristic sclerotic skeletal lesions. We present an unusual case primarily manifesting as an extensive orbital disease, with low-grade systemic involvement. Owing to its rarity and therefore lack of general awareness it remains a difficult clinical and pathologic diagnosis. Immuno-histochemistry of the biopsy specimen is diagnostic.
