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:

• Dermnet NZ
• Wikipedia

Cases associated with this book:

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

Conference: DermatopathologyConsultations.com Teaching Collection