- AD
- Defect in GJB2 (connexin 26) gene
- Diffuse honey-comb PPK appears in infancy
- Constriction bands in childhood → pseudoainhum
- Star-fish keratoses on dorsal hands, feet, elbows, knees
- Classic - with deafness
- Loricrin mutation variant - ichthyosis
- Treatment
- Retinoids may prevent pseudoainhum