Objective: Behcet disease (BD) is a chronic, relapsing inflammatory disorder of unknown etiology characterized by obstructive vasculitis. Ocular surface involvement is a less frequent anterior segment finding. We report a patient with BD whose ocular presentation was severe surface involvement.

Methods: A 26-year-old Chinese woman, who was diagnosed as having BD by a dermatologist because of oral and genital ulceration and erythema nodosum in the upper extremities 1 month ago, presented with dry eye syndrome, corneal ulceration, and conjunctival lesions. Both clinical and confocal microscope examination revealed bilateral severe inflammatory ocular surface disease. However, she did not have the common clinical ocular manifestations such as iridocyclitis, vitritis, retinal perivasculitis, or retinitis for BD. In addition to oral methylprednisolone and thalidomide, topical 1% prednisolone acetate ophthalmic suspension, tacrolimus ophthalmic suspension, and artificial tears were used. We observed for the first time histopathological changes of the eyelid in BD by confocal microscopy.

Conclusions: Ocular surface lesions should be noted as an uncommon but possible manifestation of BD. Such severe ocular surface lesions without uveitis require a thorough medical history, dermatological examination, and serological testing to arrive at a diagnosis of BD. Accordingly, routine examination of the ocular surface is recommended in patients with BD, and BD should be included in a list of differential diagnoses for patients with ocular surface lesions.