Case report: A patient was studied because of upper lid bilateral edema and xanthelasmae-like lesions after three years of evolution. During the ophthalmologic examination orange-yellowish lesions and two symmetrical tumours were observed on the temporal part of both upper lids. Corticoid-therapy was undertaken which reduced the size of the tumours, however the size increased again after the discontinuation of treatment. A biopsy was performed and lid xanthogranulomatosis was diagnosed. Other systemic examinations were normal.
Discussion: Erdheim-Chester disease is a xanthogranulomatosis that can affect ocular and periorbital structures. Combination of xanthelasmae-like lesions and bilateral orbital masses should make us consider this process and try to locate any associated systemic conditions.