Overview: What are pemphigoid and pemphigus?
The information in this section has been gathered from existing peer-reviewed and other literature and has been reviewed by expert dermatologists on the CSPA Medical Advisory Board.
Pemphigoid and pemphigus are autoimmune diseases that cause severe blistering of mucosal surfaces and the skin. In an autoimmune disease, the immune system, which attacks foreign invaders, wrongly attacks a healthy part of the body. With pemphigoid and pemphigus, the skin and mucous membranes are the target. The causes of these conditions are unknown.
Pemphigoid has two major forms:
Bullous pemphigoid causes itching that may be painful as well as blisters that appear mostly on the abdomen, back, arms and legs. If left untreated, it can be severe and rarely life-threatening.
Cicatricial pemphigoid is also known as mucosal membrane pemphigoid, as the lesions usually occur on mucous membrane surfaces. The gums are commonly affected—and epithelium of the eye (cornea and conjunctiva) is affected in up to one third of cases. Cicatricial pemphigoid can result in blindness if it involves the eyes, respiratory compromise if it involves the deeper parts of the throat, and infections if it leaves areas of open skin.
The type of pemphigoid one has depends on which antibodies are produced and in which layer of the skin the blisters occur.
Pemphigus has several types, including pemphigus vulgaris (the most common), pemphigus foliaceus and paraneoplastic pemphigus (the rarest). Blisters of varying sizes break out on the skin, the lining of the mouth, the thin covering of the penis, the vagina, and other mucous membranes. It is life-threatening if not treated.
When faced with pemphigus, the body produces auto-antibodies that attack desmoglein, the “glue” of the epidermis that keeps the skin intact. When this happens, the skin cells become separated from each other. This causes burn-like lesions or blisters that do not heal. In some cases, these blisters can cover a large area of the body. Pemphigus can be fatal if left untreated as the blisters spread and become infected. Early diagnosis is important, and aggressive treatment strategies are often needed.
Definitive statistics on pemphigoid and pemphigus are not available. However, research has determined the following:
- Pemphigoid affects men and women equally.
- Though the onset usually occurs in middle-aged and older adults, pemphigus vulgaris and pemphigus foliaceus can also occur in young adults and children.
- The number of pemphigus cases diagnosed each year is estimated to range from five per 100,000 to one per million, depending upon the type of pemphigus and the ethnicity of the affected population.
- Certain groups of people (Eastern European Jews, people of Mediterranean, Northern India and Persian descent) have a higher incidence of the disease.