A substantial contribution to the pathogenesis of severe inflammatory skin diseases including RDEB has been ascribed to sustained mechanical and/or oxidative stress-induced release of IL-1β by epidermal keratinocytes [184], which not only maintains skin inflammation but can spill over in the systemic circulation of RDEB patients to affect remote organs and promote life-threatening complications such as amyloidosis and kidney and heart involvement [184,185,186]. The gene discussed is IL1B; the disease is recessive dystrophic epidermolysis bullosa.