A multisystem disorder characterized by granulomatous panuveitis with exudative retinal detachments, frequently associated with neurologic and cutaneous manifestations.
A wide spectrum of disease with few typical cases.
More common in patients with a genetic predisposition, including Asian, Middle Eastern, Hispanic, and Native American populations.
Several human leukocyte antigen (HLA) associations include: HLA-DR4, HLA-DR53, and HLA-DQ4.
Criteria include bilateral ocular involvement without previous ophthalmologic disease.
Early manifestations include diffuse choroiditis that may include retinal detachment or focal areas of subretinal fluid.
Late manifestations of disease include evidence of previous early manifestations, ocular depigmentation and chorioretinal scars, retinal pigment epithelium clumping and migration, or anterior uveitis.
Patients may have evidence of neurologic and auditory findings as well as dermatologic signs.
The neurologic and auditory manifestations include meningismus, tinnitus, and cerebrospinal fluid pleocytosis.
Dermatologic signs include alopecia, and vitiligo.