Erdheim-Chester disease, a rare manifestation in the orbit

Introduction Erdheim-Chester disease (ECD) is a rare disease (less than 500 documented cases) belonging to the group of histiocytoses. The disease can affect several organ systems and the symptoms are correspondingly unspecific. Ocularly exophthalmos, visual disturbances, motility disorders, papilledema, xanthelasma and corneal manifestations have been described.