- AKA PPK with scleroatrophy; sclerotylosis
- AD
- Mutation in SMARCAD1
- At birth, atrophic skin on dorsal hands/feet
- Diffuse symmetric PPK (mild, palms>soles)
- Pseudosclerodermatous appearance, scleroatrophy
- Prayer sing in fingers
- Nail anomalies
- Increased risk SCC in atrophic areas
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