The following case emphasizes the importance of including IgG4-related disease (RD) in the differential diagnosis of intraocular inflammation and multiple cranial nerve palsies. A 33-year-old man, with a history of idiopathic bilateral panuveitis, presented with a new right pupillary-sparing partial third nerve palsy, which spontaneously resolved in 2?weeks, but was followed 1?month later, by a right sixth nerve palsy, which also resolved within a few weeks. Motility disturbance was accompanied by a decrease in the central acuity in the right eye. Magnetic resonance imaging/angiography (MRI/MRA) demonstrated a densely enhancing osteodestructive skull base process extending through the cavernous sinus and into the right superior orbital fissure. Biopsy of the lesion was consistent with IgG4-related disease (RD). This is the first reported case of IgG4-RD associated panuveitis without scleral involvement, expanding the list of clinical manifestations of the IgG4-RD.
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