LOF mutations in the DSG1 gene promote loss of membrane expression of DSG1, thus leading to skin dermatitis, multiple severe allergies, and metabolic wasting (SAM) syndrome [22,148], whose clinical features comprise striate palmoplantar keratoderma, as well as FTT and atopic manifestations similar to NS or PSS1. Here, DSG1 is linked to striate palmoplantar keratoderma.