Unusual orbital involvement in Erdheim Chester disease: a radiological diagnosis.
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.
All India Institute of Medical Sciences, New Delhi, India.
Arundeep
A
Arora
Sanjay
S
Sharma
Neelam
N
Pushker
Seema
S
Kashyap
Sameer
S
Bakhshi
D000368:Aged; D001706:Biopsy; D003937:Diagnosis, Differential; D031249:Erdheim-Chester Disease / Q000175:diagnosis* / Q000473:pathology / Q000601:surgery; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D009916:Orbital Diseases / Q000175:diagnosis* / Q000473:pathology / Q000601:surgery; D014057:Tomography, X-Ray Computed