Sean Klepper M.D.
Stephen Lyle, M.D., ...
Also known as: familial benign pemphigus
- 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
- Extensive full-thickness acantholysis of the epidermis (described as resembling a "dilapidated brick wall")
- 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.