Table 3.
Phenodex-FlorMore scoring system for the evaluation of PXE clinical manifestations.
| Organ System Findings | |
|---|---|
| Skin | |
| S0 | No sign |
| S1 | Papules/bumps |
| S2 | Plaques of coalesced papules |
| S3 | Lax and redundant skin |
| Eyes (score of RE + score of LE) | |
| E0 | No sign |
| E1 | Peau d’orange and/or Coquille d’oeuf |
| E2 | Angioid streaks and/or comet lesion with or without comet tail |
| E3 | Bleeding and/or scarring and/or pattern dystrophy-like changes |
| E4 | Extensive atrophic area involving the entire posterior pole |
| Gastrointestinal | |
| G0 | No sign |
| G1 | Gastrointestinal bleeding as related to PXE |
| Vascular | |
| V0 | No sign |
| V1 | Weak or absent pulse or peripheral artery disease revealed by vascular imaging |
| V2 | Intermittent claudication |
| V3 | Vascular surgery or stroke/transient ischaemic attack (TIA) |
| Cardiac | |
| C0 | No sign |
| C1 | Chest pain/angina/abnormal EKG or abnormal stress test with no symptom or mitral insufficiency |
| C2 | Heart attack |
| Renal | |
| R0 | No sign |
| R1 | Nephrolithiasis |
| In bold, the features are added. RE= right eye; LE= left eye; EKG= electrocardiogram. | |