Clinical features | Scoring | Assigned score | |
Major criteria | Patterned plaques |
| |
Joint contractures | |||
"Cobblestoning" | |||
Marked induration/peau d'orange | |||
Minor criteria | Puckering/linear banding |
| |
Superficial plaque/patch | |||
Dermal papules | |||
Scleral plaques (age <45 years) | |||
Total clinical score: | |||
Histologic features | Scoring | Assigned score | |
Increased dermal cellularity | 1 | ||
CD341 cells with tram-tracking | 1 | ||
Thick and thin collagen bundles | 1 | ||
Preserved elastic fibers | –1 | ||
Septal involvement | 1 | ||
Osseous metaplasia | 3 | ||
Total histologic score: |
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