The clinical presentation and review of systems were most consistent with Vogt-Koyanagi-Harada (VKH) disease. The plan was to start 60mg of oral prednisone and prednisolone acetate 1% every two hours in both eyes; however, the patient was sent to have blood drawn right away to rule out infectious etiologies (syphilis and tuberculosis) prior to taking his first dose of prednisone, which came back negative. He was followed closely and demonstrated an adequate response to therapy with resolution of subretinal and intraretinal fluid; however, there was incomplete resolution of the choroidal thickening and infiltration. Vision in the right eye remained stable, and the left eye improved to 20/20.

Discussion

VKH disease is an immune-mediated condition against melanocyte-containing tissues (e.g., uvea, skin).¹ Ophthalmic involvement typically presents as a bilateral, granulomatous, non-necrotizing posterior or panuveitis, and extraocular involvement may include dysacusis, poliosis, alopecia and vitiligo.² Its incidence is variable as it tends to affect populations or races containing more pigment such as Asians, Hispanics, American Indians and Asian Indians.² In the United States, VKH accounts for only 1% to 4% of all uveitis referrals.² There may be a female predilection, and it often presents in the second to fifth decade of life.^1,2 

Classically, the disease progresses through four well-defined stages: (1) prodromal, (2) acute uveitic, (3) chronic-recurrent and (4) convalescent.^2,3 The prodromal phase manifests as a nonspecific viral illness that lasts a few days. Symptoms can include malaise, headache, dizziness, fever, neck stiffness and auditory disturbances (tinnitus and dysacusis).^2,3 The acute uveitic phase follows the prodromal phase with photophobia and blurred vision. Clinical findings of this stage include bilateral granulomatous posterior or panuveitis, choroidal thickening, multifocal exudative retinal detachments and optic nerve hyperemia and edema.^2,3

While recurrent exudative retinal detachments are uncommon, the chronic recurrent phase typically involves a smoldering panuveitis with acute episodic flares of granulomatous anterior uveitis that may be resistant to corticosteroid therapy.^{2, 3} Complications of recurrent and smoldering inflammation include cataract formation, uveitic glaucoma, posterior synechiae, chorioretinal atrophy and subretinal neovascularization.^2,3 The convalescent stage of the disease is predominantly characterized by depigmentation of the skin and choroid. This stage consists of poliosis, vitiligo, alopecia and most notably, the “sunset-glow” fundus, which describes a red-orange appearance of a pale optic nerve surrounded by peripapillary/juxtapapillary choroidal depigmentation.^2,3

The exudative process in VKH classically causes detachment of the neurosensory retina from the RPE, as well as bacillary layer detachments (BALADs), which is what was seen in our patient.^4,5 While BALADs were initially described as multilobulated serous retinal detachments due to the presence of subretinal fibrin and septae, advances in OCT technology have allowed better structural analysis.^4,5 The term BALAD describes intraphotoreceptor splitting where the photoreceptor inner segments are detached from the outer segments and underlying ELM.^4,5

Treatment

The mainstay of treatment for the acute uveitic phase is aggressive systemic and topical corticosteroid use.²  Initial treatment can be 60mg to 100mg per day of prednisone or a 200mg intravenous loading dose of methylprednisolone for three days with an oral taper; patients require a prolonged taper often for longer than six months.² The chronic-recurrent phase of the disease is less responsive to corticosteroid therapy and often requires the use of other biologic or cytotoxic immunosuppressive agents (e.g., azathioprine, cyclophosphamide, mycophenolate mofetil and tacrolimus).² Patients can often regain visual acuity of 20/40 or better barring any complications as previously mentioned.²