A 26-year-old woman presented to our service with bilateral edema of the upper eyelids of 2 years duration.
In the temporal exploration, there were, in the nasal part of both eyelids, firmly-disturbed boundaries (appearance folding, folding, and epidermis), appearing in the area
They were neither mobile nor painful masses, which did not exceed the orbital rim.
The rest of the examination was normal.
To attempt to make a differential diagnosis between entities such as orbital pseudotumor, collagen disease, sarcoidosis, etc., a systemic examination was performed, resulting in all normal studies.
In the magnetic nuclear resonance (NMR) of the orbit performed, these masses were practically symmetrical and reached the eyeball elevator muscle of the upper eyelid without affection.
Corticosteroid treatment was established the same size (but AR was established with localised inflammatory process at recurrence level of methylprednisolone), with high doses (1 gr of Urbason®, HOECHST decrease).
Because this was not diagnosed, an incisional biopsy was performed, obtaining three pieces of pink-yellowish tissue.
Histological examination showed loose connective tissue infiltrated by histiocytic cells characteristic of xanthomatosis, which tended to form a granulomatous pattern.
Immunohistochemical staining for histiocytic cells (CD 68) was positive.
With all this the diagnosis of xgranulomatosis cerebri was reached.
More tests were performed to rule out multiorgan involvement: CT scan non-thoracic, cranial and orbital CT, X-ray, ultrasound scan non-muscle-pelvic osseous diphosphate with Tc 99.
All were normal.
It is therefore a case of xgranulomatosis, or pure orbital Erdheim-Chester disease without systemic manifestations.
Currently, after a new cycle of corticosteroids (methylprednisolone 80 mg/24 hours, Urbason®) and with a maintenance dose (methylprednisolone 4 mg/48 hours, Urba remains relatively stable.
