Hailey-Hailey disease

    Article Contributors: 
    Sean Klepper M.D.
    Stephen Lyle, M.D., ...

    Also known as: familial benign pemphigus

    Clinical Features:

    • Autosomal dominant disorder presenting in adolescence or adulthood
    • Vesicles form, then erode to form crusted plaques.
    • Usually limited to intertriginous zones, neck, axillae, and less often the antecubital, perianal and inframammary skin, but may sometimes become more widespread

    Histologic Features:

    • Extensive full-thickness acantholysis of the epidermis (described as resembling a "dilapidated brick wall")
    • Acanthosis
    • Sometimes focal dyskeratotic keratinocytes are seen, but are ususally sparse.
    • Basal layer "villi" are not a prominent feature, in contrast to Darier's disease.
    • Immunofluorescence is negative.

    External Links:

    Cases associated with this book:

  • Hailey- Hailey disease
    Author: Stephen Lyle, M.D., Ph.D.

    Conference: DermatopathologyConsultations.com Teaching Collection