Hereditary Epidermolysis Bullosa

Epidermolysis Bullosa Simplex in Five Consecutive Generations

“…Histological studies here suggest epidermolysis bullosa simplex to be a disease of the epidermis, especially the basal cell, as was postulated by Lowe (1967). The mechanism of cellular necrosis in this disease might be an inherent weakness of cell structure or be the release by trauma of a necrotizing agent (Lowe, 1967). Perhaps as mentioned by Lever (1964), this elastic tissue is affected in the other variants apart from the simplex type.…”

Epidermolysis Bullosa Heriditaria

“…On the basis of an analysis of a series comprising 1181 cases, Touraine i2 described three different types of EBH: two dominant types were termed epidermolysis bullosa simplex and epidermolysis bullosa hyperplastica, respectively, the third, recessive type being termed epidermolysis bullosa polydysplastica. With support in some histobiochemical studies, Lowe 21 classified the disease into three groups: Epidermolysis bullosa simplex, epidermolysis bullosa dystrophica, dominant form (EBDD), and epidermolysis bullosa dystrophica, recessive form (EBDR). This is in accordance with the classification of Touraine.…”

The Basal Lamina in Skin Disease

“…Many studies have been reported on the different types of epidermolysis bullosa (E.B.). [41][42][43][44] The findings are discussed generally in terms of basal laminar location in relation to the dermal-epidermal separation, rather than precise laminar morphology. This disease can be divided into four basic ultrastructural types: (1) epidermolysis bullosa letalis (Herlitz)-separation in the plane between the basal cell plasma membrane and the basement membrane; (2) epidermolysis bullosa dystrophic, dom inant-defect through the basement membrane; (3) epidermolysis bu I losa dystrophic, recessi ve-cl eavage below the basement membrane with defect in upper dermis; (4) epidermolysis bullosa simplexcleavage through the basal cell cytoplasm (Figs.…”