Acute Multifocal Placoid Pigment Epitheliopathy

Acute Multifocal Placoid Pigment Epitheliopathy (AMPPE) is a condition of rapid loss of central vision, which usually resolves in 2 to 3 weeks without treatment. The disease is a multifocal inflammatory disorder that generally appears as irregular creamy yellow-white or gray shaped opacities, located around flat lesions in the choroid and retinal pigment epithelium. Symptoms appear as painless vision loss over several days often preceding a viral illness. The patients will often complain of headaches, photophobia, pain, and red eyes, stiff neck, vomiting, fever, and malaise. Of note is that occasionally hearing loss occurs. Vision loss recovers in 80 % of patients leaving discrete pigment epithelial scars.

The fluorescein angiogram is quite characteristic and shows lesions with early blockage and late diffuse staining with spontaneous resolution over several months. The spots will leave partially depigmented retinal epithelium areas behind. The vision should return if the fovea is uninvolved. The cause is thought to be a virus or infection of the choroid and pigment epithelium. No treatment would be necessary unless cerebral vasculitis and/or severe foveal disease is present. Treatment for foveal involvement would be systemic steroids.