Epidermolysis bullosa

    Article Contributors: 
    Sean Klepper M.D.
    Artur Zembowicz M.D....

    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.
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    Cases associated with this book:

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

    Conference: DermatopathologyConsultations.com Teaching Collection