Organotypic and humanized animal models of genodermatoses

Skin pathology comprises a large number of rare inherited diseases, known as genodermatoses, with a wide range of phenotypes and severities. Although transgenic and gene knockout mouse models for several of these disorders exist, current efforts to find specific disease treatments and define precise pathogenesis mechanisms require better surrogate models with human cells. Current advanced methods to faithfully replicate a pathological skin condition rely on bioengineering techniques and regeneration of cultured human skin in immunodeficient mice. In this chapter, we provide numerous examples of how skin three-dimensional organotypic cultures and in vivo systems, based on bioengineering, facilitate the study and management of genodermatoses.