[Orbital inflammatory pseudotumor with optic neuritis in Behçet's disease].

Abstract

To report an atypical case of orbital inflammatory pseudotumor associated with optic neuritis revealing Behçet's disease.
A 37-year-old man was worked up for headache and progressive decreased bilateral visual acuity most profound in the left eye. Initial ophthalmologic exam was without abnormal findings. Orbital CT scan and MRI appeared consistent with orbital inflammatory pseudotumor involving the left lateral rectus muscle (orbital myositis). The patient was treated with oral corticosteroids, but clinical response was incomplete. Follow-up ophthalmologic exam revealed acute inflammatory right optic neuritis (visual acuity: 5/10, papillitis on fundus exam and papillary edema on fluorescein angiography) and persistence of left orbital myositis. The diagnosis of Behçet's disease with ocular complications was made on the basis of recurrent oral ulcers, scars from genital ulcerations and pseudofolliculitis. It was decided to treat the patient with a bolus of IV methylprednisolone for 3 consecutive days, followed by oral prednisolone and cochicine, leading to stabilization of the ocular manifestations.
To our knowledge, this is the first report of such an association leading to a diagnosis of Behçet's disease. The coexistence of these two manifestations as well as the contralateral nature of the involvement speaks to the pathogenic mechanism of these complications (systemic vasculitis).