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A case of Erdheim-Chester disease with bilateral orbital involvement
1Zhongshan Ophthalmic Center, Sun Yat-sen University of Medical Sciences, Guangzhou, 510060 China.
Purpose:
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.