Epidermolytic ichthyosis (EI), pachyonychia congenita (PC) and epidermolysis bullosa (EB) are rare genodermatoses caused by function-impairing mutations in different keratins (EI: K1 or K10; PC: K6, K16 or K17; EB: K5 or K14) [182]. The gene discussed is KRT5; the disease is epidermolytic ichthyosis.