butterfly-shaped pigmentary maculopathy

Abnormal pigmentation of yellow, white, or black color accumulates in the central area of the macula.  The deposition of pigment appears in the retinal pigment epithelium (RPE) level in a pattern more or less resembling the wings of a butterfly.  The peripheral retina has diffuse pigment mottling.  Drusen-like structures may be seen at the peripheral borders of the macular pigmentation.  Visual fields are normal usually but there may be some decrease in central sensitivity.

However, this is a generalized retinal disorder as revealed by the abnormal mass response (decreased light/dark ratio) of the electrooculogram (EOG).   Patients may not have visual symptoms until their late 20s or early 30s even though the pigmentation may be evident in the second decade.  Color vision, dark adaptation and the ERG are normal.  Younger patients may have normal vision.