| |

158661 Suprabasal epidermolysis bullosa simplex

definition

Suprabasal epidermolysis bullosa simplex is a subtype of inherited epidermolysis bullosa simplex and comprises a group of clinically and genetically heterogeneous conditions, with phenotype ranging from mild to severe, principally characterized by infantile, localized or generalized, superficial skin erosions due to blistering within the middle or upper epidermal layers. Features of ectodermal dysplasia are frequently associated and depending on the specific disorder, variable extracutaneous involvement may be observed.