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This report describes the case of a patient who had symmetrical exophthalmos, periorbital xanthelasmas and reduced vision. Next to Wegener’s granulomatosis t he differential diagnosis of Erdheim-Chester disease is discussed. It concerns a rare systemic histiocytosis of unknown etiology. Above all, the skeleton syste m with symmetrical long bone osteosclerosis is affected. Manifestations in the a rea of the orbit have seldom been reported with bilateral retrobulbar infiltrati ons, exophthalmos, diplopia, compression of the optic nerve and periorbital xant helasmas.
This report describes the case of a patient who had symmetrical exophthalmos, periorbital xanthelasmas and reduced vision. Next to Wegener’s granulomatosis t he differential diagnosis of Erdheim-Chester disease is discussed. It concerns a rare systemic histiocytosis of unknown etiology. Above all, the skeleton syste m with symmetrical long bone osteosclerosis is affected. Manifestations in the a rea of the orbit have seldom been reported with bilateral retrobulbar infiltrati ons, exophthalmos, diplopia, compression of the optic nerve and periorbital xant helasmas.