Cite this page: Pernick N. Retinitis pigmentosa. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/eyeretinaretinitispigmentosa.html. Accessed September 29th, 2023.
Definition / general
- Various inherited retinal disorders that are not actually inflammatory
- Common (incidence of 1/3600) with variable inheritance or as part of Refsum disease
- Usually causes loss of rods and cones to apoptosis and focal proliferation of retinal pigment epithelium
- Loss of rods causes early night blindness and restricted visual fields (bilateral loss of peripheral vision)
- Loss of cones affects central visual acuity
- Retinal atrophy causes constriction of retinal vessels, optic nerve head atrophy and accumulation of retinal pigment around blood vessels
Microscopic (histologic) description
- Migration of macrophages and retinal pigment epithelial cells with melanin into retina, particularly around vessels
- Also atrophy of photoreceptors in retina and choriocapillaris