Clinical Features:

• 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.

Histologic Features:

• 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.

Cases associated with this book:

Epidermolysis bullosa
Author: Artur Zembowicz M.D. Ph.D.

Conference: DermatopathologyConsultations.com Teaching Collection