Skin Necrosis in Children: Genodermatosis
摘要
The term genodermatosis comprises a large group of dermatoses caused by genetic defects, including monogenic and mosaic disorders. From the physiopathogenic point of view, various compartments of the skin can be disturbed, such as intraepidermal or epidermal-dermal adhesion, cornification, DNA repair, the vascular system, etc. The onset of clinical symptoms is most commonly at birth or during early childhood, and the clinical course is progressive, without spontaneous healing or remission.