A 22-year-old female with a history of recurrent oral ulcers and vulvovaginal burning presented to the emergency department with painless bilateral uveitis of 8 months duration which acutely worsened in the past 2 weeks. Of note, the patient was seen outpatient by ophthalmology 5 days prior to presentation whereby a dilated eye exam demonstrated intraretinal hemorrhage in the right eye, dot-blot hemorrhage in the left eye, and bilateral vitritis with concern for cytomegalovirus retinitis (CMV) retinitis. Recommendations included 1% prednisolone eye drops twice a day, 1% cyclopentolate eye drops twice a day, and valganciclovir twice a day. However, the patient was only compliant with the cyclopentolate drops due to financial hardship. On presentation, she also endorsed floaters, blurry vision, and flashes of light. She denied any acute oral or genital symptoms; however, she reported a history of recurrent "canker sores" and burning in the vulvovaginal area that began approximately 5 years ago. She denied a history of genital ulcers. Physical exam was remarkable for photophobia and visual acuity of 20/50 in the right eye and 20/80 in the left eye with correction. Intraocular pressure in each eye was 16 mmHg by tonometry and pupils were equal, round, and reactive to light bilaterally. Extraocular movements were intact bilaterally. A slit lamp eye examination showed a flat macula and similar hemorrhages as seen in the ophthalmology office 5 days prior to presentation. Laboratory evaluation revealed an elevated erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP). Extensive infectious disease testing for CMV, Herpes simplex virus (HSV), HIV, Varicella-zoster virus (VZV), syphilis, toxoplasmosis, tuberculosis, Hepatitis B and C was undergone, all of which resulted negative. Likewise, rheumatologic workup including testing for antinuclear antibody (ANA), anti-neutrophil cytoplasmic antibody (ANCA), angiotensin-converting enzyme (ACE), and human leukocyte antigen B27 (HLA-B27) was negative.
With concern for infectious retinitis, the patient was started on a course of IV acyclovir 10 mg/kg every 8 h. Her hospital course was complicated by acute kidney injury (AKI) secondary to acyclovir-induced nephrotoxicity; the medication was immediately discontinued, and she was given IV hydration to resolve the AKI. After discussion with ophthalmology about the patient's clinical course, presentation, and laboratory data, there was concern for BD as she met clinical diagnostic criteria. She was started on IV methylprednisolone 250 mg every 6 h for 3 days. The patient experienced significant improvement of photophobia and complete resolution of blurry vision and flashes of light.
At this point with the patient's negative infectious workup, it was felt a more detailed rheumatologic workup was needed. The patient was instructed to follow-up with outpatient rheumatology for skin pathergy testing to provide additional support to the diagnosis of BD and for long-term management. In that time, she was discharged home with a 2-week oral prednisone taper (50 mg daily for 7 days and 25 mg daily for the next 7 days) and valacyclovir to provide coverage for possible underlying retinitis.
Unfortunately due to financial hardship, this patient was unable to follow-up with the outpatient rheumatologist. Less than 3 months later she presented to the hospital with similar ocular symptoms and was readmitted for painless panuveitis. Of note, the patient reported she developed a painful ulcer on her labia a month prior that resolved spontaneously. She was given high-dose intravenous steroids for 3 days. Pathergy testing performed by rheumatology during this hospitalization was negative. Her vision returned to baseline and the patient was discharged with a scheduled outpatient rheumatology follow-up to determine long-term therapy.