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Simulate retinitis pigmentosa (RP) — the most common inherited retinal dystrophy and the leading genetic cause of blindness in the working-age population worldwide, with a prevalence of approximately 1 in 3,000–5,000 individuals. RP is a clinically and genetically heterogeneous group of progressive rod–cone dystrophies characterised by primary rod photoreceptor degeneration beginning in the mid-peripheral retina and advancing centripetally toward the macula, followed by secondary cone photoreceptor loss. The classic clinical triad consists of: (1) bone-spicule intraretinal pigment deposits (melanin liberated from degenerating RPE cells that migrates into the inner retina, aligning along retinal blood vessels in a characteristic spiculate pattern); (2) waxy-pale optic disc (gliotic pallor from retrograde ganglion cell axonal degeneration); and (3) arteriolar attenuation (narrowing of retinal arterioles from chronic low-grade vascular remodelling secondary to outer retinal atrophy). The hallmark functional sequence is: nyctalopia (night blindness) as the earliest symptom in the 1st–2nd decade → progressive mid-peripheral ring scotoma expanding both centripetally and centrifugally → tunnel vision (constricted central island) → eventual macular cone involvement with central visual acuity loss in the 4th–6th decade. Over 100 causative genes have been identified; the three major inheritance patterns are autosomal dominant (adRP, ~30–40%; rhodopsin RHO p.Pro23His is the single commonest adRP mutation worldwide), autosomal recessive (arRP, ~50–60%; USH2A usherin mutations are the commonest arRP cause, also causing Usher syndrome type 2), and X-linked (xlRP, ~5–15%; RPGR ORF15 mutations account for >70% of xlRP). The first FDA-approved gene therapy for any inherited retinal disease — voretigene neparvovec-rzyl (Luxturna, Spark Therapeutics, 2017) — targets RPE65-associated RP/LCA via subretinal AAV2-RPE65 delivery. Model three major RP genetic subtypes with ΔE colour shift, CIE xy chromaticity, and image simulation.

Retinitis pigmentosa colour science simulation by Auric Artisan.

Base color
RP genetic subtype & settings
Rod–cone degeneration severity 50%
Image simulation
Upload JPG/PNG (max 1200 × 1200). See how a scene appears through the progressive tunnel vision of adRP (RHO — slow mid-peripheral field loss with preserved macular cone island until late stages), arRP (USH2A — moderate rod-cone degeneration ± sensorineural hearing impairment in Usher syndrome type 2), or xlRP (RPGR — severe early-onset rod-cone degeneration with rapid field constriction).
Research notes
RP as the archetypal rod-cone dystrophy: Retinitis pigmentosa is the clinical prototype for all inherited photoreceptor degenerations. The rod-before-cone degeneration sequence explains the characteristic symptom progression: nyctalopia first (rod loss), then peripheral field loss (mid-peripheral rod-dominant retina), then colour vision and central acuity changes last (macular cone involvement). Full-field ERG is the gold standard for quantifying RP severity — the scotopic (rod-driven) a- and b-wave amplitudes decline years before symptoms, while photopic (cone-driven) responses are initially preserved. The International Society for Clinical Electrophysiology of Vision (ISCEV) protocol defines RP staging by ERG amplitude reduction.
Swatches
Normal
HEX: — • RGB: — • xy: —
RP affected
HEX: — • RGB: — • xy: —
ΔE (CIE76)
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ΔE (CIEDE2000)
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Deep preview
Normal
RP (deep)
Chromaticity (CIE xy)
Rod-cone degeneration chromaticity shift
D65 white point: 0.313, 0.329
Image simulation

Retinitis pigmentosa simulations model the rod-first-then-cone photoreceptor degeneration sequence using genetic-subtype-specific severity functions in the sRGB colour space. adRP (RHO): models slow progressive rod scotopic loss — cone-mediated colour vision is preserved until late stages, with predominantly luminance reduction and desaturation reflecting the loss of rod-cone coupling signals that normally amplify cone pathway gain in mesopic conditions. arRP (USH2A): models moderate rod-cone degeneration with earlier cone involvement than adRP — greater chroma loss and luminance reduction at equivalent severity. xlRP (RPGR): models severe early-onset rod-cone loss with rapid desaturation and significant luminance impact — consistent with the clinical observation that xlRP patients lose central acuity ~5–10 years earlier than adRP patients. ΔE2000 per Sharma et al. (2005). Not for clinical diagnosis — RP requires full-field ERG, OCT, visual field testing, and genetic testing.

Multi-condition comparison
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Compare adRP (RHO — slow rod degeneration, preserved macular cone island), arRP (USH2A — moderate rod-cone with ± hearing loss), and xlRP (RPGR — severe early-onset rod-cone degeneration) colour impact side by side.