Summary
Epidermolysis bullosa are a group of skin fragility during which blisters and erosions occur either spontaneously or after mild physical trauma. There are several forms of congenital and hereditary epidermolysis bullosa as well as acquired forms clinically very similar to the former but with adult onset. About 20 different congenital and inherited forms have been observed and classified into 3 groups according to the layer in which cleavage occurs in the dermoepidermal junction: intraepidermal forms (mild, non-cicatricial), junctional forms(cleavage within the pars lucida) and dermatolytic or dystrophic forms (cleavage under the lamina basalis). Intraepidermal forms: Dowling-Meara's herpetiform epidermolysis bullosa is an autosaomal recessive inherited form characterized by groups of blisters forming circinate spots with central heal similar to herpetiform dermatitis; lesions are very painful and predominate in plica; molecular disorders affect keratin V and XIV; prenatal diagnosis is feasible. Weber-Cockayne epidermolysis bullosa simplex is transmitted as an autosomal dominant trait; it is marked by juvenile or adolescent onset and only affects the palms and plants of hands and feet during the hot season; molecular anomalies involve keratin V and XIV. Ogna's epidermolysis bullosa simplex, transmitted as an autosomal dominant trait, is a very rare form with hemorrhagic blisters and occurrence of skin bruising. Koebner's epidermolysis bullosa simplex, transmitted as an autosomal dominant or recessive trait, is extremely rare: the blister form in the basal keratinocytes; molecular disorders affect keratin V and XIV. *Author: C. Blanchet-Bardon, M.D. (March 2002) *
