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  • Grand Rounds

    A child presented to the Bascom Palmer Eye Institute emergency department (BPEI ED) with 2-month history of progressive bilateral redness and a 2-week history of photophobia and blurry vision in both eyes. His medical history is relevant for a diagnosis of atopic dermatitis made at age 2 years and treated with dupilumab injections every other week for the last 7 months. At the BPEI ED, his uncorrected distance visual acuity was 20/25 in both eyes. The slit lamp examination showed a papillary reaction in the inferior tarsal conjunctiva (right 2+, left 2+), ciliary flush (right 1+, left 2+), anterior chamber and anterior vitreous cells (right trace, left 2+), and posterior vitreous cells (right trace, left 1+). Dilated fundus exam showed multifocal moderate size lesions in different stages of evolution scattered in the posterior pole and mid-periphery in both eyes. Spectral-domain optical coherence tomography (SD-OCT) through the lesions showed choroidal inflammatory lesions with infiltration under the retinal pigment epithelium that blocked transmission. The macula was normal in both eyes. While fluorescein angiography showed early blockage and late staining of the lesions, indocyanine green angiography showed focal early and late hypofluorescent lesions in both eyes. An extensive infectious and autoimmune workup performed in collaboration with the pediatrics department was relevant for elevated C-reactive protein (79.5 mg/L, normal value <8.0 mg/L), erythrocyte sedimentation rate (77 mm/h, normal value <10 mm/h), and complement 4 (48 mg/dL, normal value <45 mg/dL). Nine days after the first visit, the dilated fundus exam showed improvement of some lesions but worsening of others. Thus, treatment with oral prednisone (30 mg/day) was prescribed. His next dose of dupilumab was deferred. After deferring his last dose of dupilumab for 10 days and treatment with oral prednisone, most of the placoid lesions in fundus exam have flattened with serpiginous borders. However, there are persistent vitreous cells and placoid sub-RPE infiltration in OCT. At this point, the diagnosis of relentless placoid chorioretinopathy (RPC)-associated with dupilumab was established. After three weeks of treatment with oral prednisone, the patient developed two new retinal hemorrhages and increased large vessel sheathing in the right eye. Immunosuppressive therapy (IMT) with adalimumab, mycophenolate mofetil, and methotrexate was started. After 5.5 months under treatment with IMT, the patient is stable with no new lesions. His visual acuity is 20/20 in both eyes.

    Presentation Date: 02/13/2025
    Issue Date: 08/29/2025