Retina/Uveitis Quiz 19

Rosa Y. Kim, MD | Massachusetts Eye and Ear Infirmary, Harvard Medical School

May 27, 1997
Figure 1
Figures 1-3. This is a patient who had 20/20 vision OU and was found to have these fundus and fluorescein findings.
Figure 2
Figure 3
Questions and Answers
1. What is the most likely diagnosis?
Answer: Sjogren's reticular dystrophy of RPE, characterized by an accumulation of dark pigment in a meshwork or fishnet-like pattern. This is an extremely rare condition.

2. What is the inheritance pattern of this condition?
Answer: Autosomal recessive

3. Does the pigmentary pattern in this condition change over time?
Answer: The network of pigment starts centrally, then extends toward the periphery in later stages of the disease, and finally disappears.

4. How is the vision affected?
Answer: Vision is usually unaffected in this condition

5. What would an ERG SHOW in this condition?
Answer: Both full field and foveal ERGs are normal.