The Management of Hereditary Epidermolysis Bullosa: Experience of a Moroccan Sub-Population

Hereditary epidermolysis bullosa (HEB) is rare genodermatose characterized by epithelial fragility leading to the formation of bubbles, cutaneous and mucosal erosions [1]. They would affect about 1/50000 to 1/20000 newborns [2]. The cause of this genodermatosis is a deficiency of one of the proteins involved in anchoring the epidermis to the dermis. The deficient protein determines the level of bubble formation and, therefore, the form and severity of the EB subtype. This is a basis for classification. The resulting form is the result of an autoimmune reaction against collagen VII, the protein anchoring fibrils in the papillary dermis, causing detachment of the epidermis. The clinical severity is variable, ranging from minor functional discomfort to the death of the child, to the culprits responsible for severe disabilities, to the infectious, nutritional, cicatricial and functional complications they cause. The aim of this work is to describe the particularities of HEB in our population epidemiologically, clinically, as well as the search for different complications in order to establish a good therapeutic management.