epidermolysis bullosa dystrophica

Epidermolysis bullosa dystrophica or dystrophic EB (DEB) is an inherited disease affecting the skin and other organs.'Butterfly child' is the colloquial name for a child born with the disease, as their skin is seen to be as delicate and fragile as that of a butterfly.

The deficiency in anchoring fibrils impairs the adherence between the epidermis and the underlying dermis. The skin of DEB patients is thus highly susceptible to severe blistering. Collagen VII is also associated with the epithelium ...