Epidermolysis bullosa is the generic name given to a group of genetic diseases in which the skin and mucosal lining tissues (epithelia) undergo, either spontaneously or as a result of minimal trauma, dislodging and blistering. Severity varies widely: there are mild forms that allow near-normal life and very severe forms, which can be lethal in neonatal or prenatal life. The first symptoms usually occur in the first few months of life. Based on the depth of the lesions, bullous epidermolysis is divided into three classes: simple, junctional, and dystrophic. In the simple forms, the epidermis (the most superficial layer of skin) is involved, while the mucous membranes are rarely affected; boils heal without scarring, and the main complication is infection of the lesions. In the junctional forms, the lesions are deeper (between the epidermis and the dermis), and the boils are extensive and often involve the mucous membranes; lethal epidermolysis bullosa (Herlitz type) belongs to this group.
Read more.
