Dominant mutations in keratin 5 (KRT5) or keratin 14 (KRT14) lead to epidermolysis bullosa simplex (EBS), which is characterized by intraepidermal blisters, tissue fragility, and collapse of the keratin network into cytoplasmic protein granules, especially upon mechanical stress and other types of stress (Kellner and Coulombe 2009). Here, KRT14 is linked to epidermolysis bullosa simplex.