Pemphigus vulgaris is an autoimmune skin disorder characterized by blistering of the skin and mucous membrane.

Pemphigus involves blistering of the outer (epidermal) layer of the skin and mucous membranes. It is an autoimmune disorder in which the immune system produces antibodies against specific proteins in the skin and mucous membrane. These antibodies produce a reaction that leads to a separation of epidermal cells. The exact cause of the development of antibodies against the body's own tissues is unknown.

Sometimes pemphigus appears in reaction to medications, although this is rare. Pemphigus may be a side effect of certain blood pressure medications (ACE inhibitors) or chelating agents (medications, such as penicillamine, that remove certain materials from the blood).

Pemphigus is uncommon. It occurs almost exclusively in middle-aged or older people of all races and ethnic groups. About one-half of the cases of pemphigus vulgaris begin with blisters in the mouth, followed by skin blisters. The blisters (bullae) are relatively asymptomatic, but the lesions become widespread and complications develop rapidly and may be debilitating or fatal.

• Skin lesions which may be:
  • Recurrent or relapsing
  • Blistering, loose
  • Mouth or skin ulcers
  • Draining, oozing, crusting
  • Located on the mucous membrane of the mouth
  • Located on the scalp, trunk, or other skin areas
  • Spreading to other skin areas
  • Peeling superficially or detaching easily

• Nikolsky's sign is positive -- when the surface of unaffected skin is rubbed sideways with a cotton swab or finger, the skin separates easily.
• A skin lesion biopsy shows separation of epithelial cells from each other.
• An examination of the biopsy tissue with immunofluorescence confirms pemphigus.