SMARCAD1 and palmoplantar keratoderma-sclerodactyly syndrome: Recently discovered autosomal dominant Huriez syndrome (also known as sclerotylosis) is caused by mutations in the skin specific isoform of SMARCAD1 (SWI/SNF-related matrix-associated actin-dependent regulator of chromatin, subfamily A containing DEAD/H box 1), which is involved in DNA DSBR and chromatin remodeling (19, 64, 65).