Sean Klepper M.D.
Artur Zembowicz M.D....
- Group of inherited blistering disorders.
- A variety of subtypes exist, all of which arise in early childhood, except epidermolysis bullosa acquisita, which is of adult onset.
- Clinical features vary from one subtype to another, ranging from mild to severe and fatal.
- Noninflammatory subepidermal blister
- Sparse perivascular lymphocytic infiltrate
- Dermal scarring in old lesions of some subtypes
- Subtyping requires electron microscopy or immunomapping with a panel of antibodies against basement membrane zone proteins.
- Immunofluorescence is negative, except in epidermolysis bullosa acquisita, which shows linear IgG and complement at the dermal-epidermal junction, localizing to the floor of the blister on salt-split skin.