A case of Erdheim-Chester disease with bilateral orbital involvement.
OBJECTIVE
To describe a case of Erdheim-Chester disease with bilateral orbital involvement.


METHODS
A 43-year-old female with bilateral proptosis was presented. Its clinical features, image findings, pathological character and therapeutic effect were evaluated.


RESULTS
CT demonstrated bilateral, diffuse orbital mass. Histopathologic assessment revealed a diffuse xanthogranulomatous process with clusters of lipidladen histocytes. Numerous Touton giant cells were scattered throughout the lesion. Renal and heart failure happened during a 6-year follow-up period. Long bones roentgenogram demonstrated diffuse symmetrical sclerosis with extensive, lytic lesions. Systemic administration of corticosteroids, chemotherapy, immunoglobulin and traditional Chinese medicine showed good therapeutic result.


CONCLUSIONS
An administration of systemic corticosteroids, chemotherapy, immunoglobin and traditional Chinese medicine can control Erdheim-Chester disease. Further exploration of its pathogenesis and collection of useful clinical data are required.
Wu
Z
Z
Zhongshan Ophthalmic Center, Sun Yat-sen University of Medical Sciences, Guangzhou, 510060 China.
Yan
J
J
Hong
W
W
Yuan
Y
Y
Dai
L
L
D000328:Adult; D003520:Cyclophosphamide / Q000627:therapeutic use; D003907:Dexamethasone / Q000627:therapeutic use; D003951:Diagnostic Errors; D004365:Drugs, Chinese Herbal / Q000627:therapeutic use; D031249:Erdheim-Chester Disease / Q000000981:diagnostic imaging / Q000188:drug therapy / Q000473:pathology; D005260:Female; D006801:Humans; D009915:Orbit / Q000000981:diagnostic imaging / Q000473:pathology; D009916:Orbital Diseases / Q000000981:diagnostic imaging / Q000188:drug therapy / Q000473:pathology; D008517:Phytotherapy; D014057:Tomography, X-Ray Computed; D014750:Vincristine / Q000627:therapeutic use
D004365:Drugs, Chinese Herbal; D014750:Vincristine; D003907:Dexamethasone; D003520:Cyclophosphamide