Adult onset primary bilateral orbital xanthogranuloma: Clinical, diagnostic, and histopathologic correlations

Abstract

Juvenile or infantile xanthogranuloma is a known entity with dermatologic, ocular and systemic involvement. The orbital form is quite rare and is usually associated with other systemic manifestations. Isolated bilateral orbital involvement is known to occur in adults in association with generalized bone involvement and other organ systems as part of the Erdheim-Chester disease syndrome. Primary adult onset bilateral orbital xanthogranuloma not associated with other organ system involvement or dermatologic manifestations, is unusual and should be distinguished from other chronic orbital disorders, such as inflammatory pseudotumor, low grade lymphoma, and infectious orbitopathies. The authors present a case of primary adult onset bilateral orbital xanthogranuloma in a 38-year-old female with painless, progressive bilateral proptosis of 16 years' duration. To our knowledge this is the first case of its kind reported in the ophthalmic literature.