Lethal acantholytic EB Simplex

     Lethal acantholytic EB simplex is an extremely rare form of autosomal recessive EB simplex that is caused by mutations in the desmoplakin gene. There has only been tow people who have been described with having this type of EBS. They both hve died soon after birth.
      Milia and scarring were not observes, although significant nail dystrophy was seen, hair loss or poor growth and the teeth at birth where resported. Blistering of the mouth and intestine/gastrointestinal tract/gastrointestinal tract and trachea/respiratory tract was noted. Eyes involvement was not reported.