Epidermolysis Bullosa Simplex in Israel

Abstract
EPIDERMOLYSIS BULLOSA (EB) encompasses a heterogeneous group of hereditary disorders characterized by blistering of the skin upon exposure to mechanical stress. Although the most obvious signs of the disease are vesicles and bullae within the skin and mucous membranes, internal organs may also be involved.1 Traditionally, EB is classified in 3 major groups according to the level of dermoepidermal separation at the basement membrane (BM) zone. Epidermolysis bullosa simplex (EBS) results from separation of the skin above the BM as a result of basal keratinocyte cytolysis; junctional EB is caused by blister formation within the BM above the lamina densa, whereas in dystrophic EB, blisters develop below the BM.1,2