Table 1 Girardi score—definition and classification of clinical and histological findings [56]

Clinical findings major criteria

Patterned plaques

Joint contractures

“Cobblestoning”

Marked induration / Peaud’orange

Clinical findings minor criteria

Puckering / linear banding

Superficial plaque / patch

Dermal papules

Scleral plaques (<45 years)

Histological findings

Increased dermal cellularity (Score +1)

CD34+ cells with tram tracking (Score + 1)

Thick & thin collagen bundles (Score + 1)

Preserved elastic fibres (Score -1, if absent)

Septal involvement (Score +1)

Osseous metaplasia (Score +3)