In this type, blistering occurs in the lamina densa, which is a component of the basement membrane zone between the lamina lucida and the underlying dermis of the skin. DEB accounts for about 25 percent of cases. DEB is characterised by the scarring of the healed wounds, resulting in contraction of the joints, fusion of the fingers and toes, contraction of the mouth membranes, and narrowing of the oesophagus.
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It may be inherited in a recessive or dominant manner.
DEB inherited in a dominant manner is often the less severe form. But severity can increase with age due to scarring, contraction and damage to skin tissue.
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The recessive form of DEB is more severe, and patients are at a high risk of developing squamous cell carcinoma (an aggressive type of skin cancer).
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