Pemphigus vulgaris is an autoimmune condition that causes blistering of the skin and mucous membranes. This condition can be life-threatening. Pemphigus vulgaris has an increased incidence in Ashkenazi Jews and those of Mediterranean origin, but otherwise has an estimated incidence of 0.5-3.2 cases per 100,000 people. It affects everyone, but can affect teenage girls more. The average age of onset is 50-60, but it has appeared in children and the elderly.
Symptoms of pemphigus vulgaris
- Soft blister with clear fluid inside
- Affects the mouth
- Slow healing erosions in the mouth
- May affect the vulva, labia, vagina, cervix, oesophagus, conjunctiva, urethra, and anus
Causes of pemphigus vulgaris
The cause is unknown, however the circulating antibodies are directed at the cell surface of keratinocytes. There are several forms of pemphigus – vulgaris, foliaceus and paraneoplastic. Over 70 per cent of pemphigus cases are vulgaris. It can be triggered by some medication.
Treatment of pemphigus vulgaris
Typical treatment is steroids and immunosuppressants. The point of treatment is to reduce the inflammatory response and autoantibody production.