What is Dermatology - Are there any hereditary basement membrane zone (BMZ) and dermis disorders that cause blistering and skin damage?
Yes. A complex series of hereditary illnesses causes the skin to become friable, resulting in bullous lesions and scarring. Junctional epidermolysis bullosas (JEBs) and dystrophic epidermolysis bullosas (DEBs) are two subgroups within this group (DEBs). These disorders, like the EBS diseases, impair the epidermal layers because critical structural parts of the BMZ and dermis are lacking, causing the skin to split and blister readily. In JEBs, the separation takes place within the BMZ's lamina lucida (LL). This group has lower levels or anomalies in LL components like laminins I and V, 19-DEJ-1 protein, XII collagen, plectin, XVII collagen (bullous pemphigoid antigen), and alpha 4 beta 6 integrin. Separation occurs below the BMZ in the dermal layer in the DEB group, with a decreased quantity or absence of type VII collagen. The clinical picture is usually more severe the deeper the split occurs in the skin, with greater scarring and loss of function. DEB sufferers often have significant deforming scars and a shorter life expectancy.
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