- Demonstrates revertant mosaicism - loss of heterozigozity, revertant cell corrects the defect - leads to islands of sparing that increase over time
- Mutation in KRT1 or KRT10 (like epidermolytic ichthyosis)
- Affected skin: loss of epidermal differentiation above the basal layer with nucleolar vacuolization, loss of granular layer and acanthosis
- Revertant skin: normal
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