What is Pemphigus Vulgaris?
Pemphigus vulgaris (PV) is a rare condition that causes blisters to develop on the skin and on the lining of the mouth.
PV is the most common form of pemphigus, which is a group of rare skin blistering diseases.1 PV is an autoimmune condition in which your body’s immune system, its defense against infection, attacks the cells deep in the skin and mucous membranes (the protective linings of the mouth, throat, nose and genitals).2
The immune system normally produces antibodies to fight infection. In PV, the immune system produces antibodies to proteins in your body that help to maintain the structure of the skin and mucous membranes, by gluing them together. The production of antibodies against these proteins, which are called desmogleins, causes the blisters to form on the skin and on other parts of the body, commonly in the mouth. The type of antibodies produced in PV determines the parts of the body that are affected. Antibodies against desmoglein 1, desmoglein 3 or both proteins may be present.1 It is the presence of these antibodies in the blood that is important in achieving a diagnosis of PV.2
Healthy skin
The skin cells are held together by the desmoglein proteins which act like a glue. This also occurs in the mucous membranes, such as on the inside of the mouth
Skin with PV
The antibodies in people with PV attack the desmoglein proteins that glue the cells together. Skin cells separate from each other causing blisters and lesions to form
The cause of PV is unknown but it is thought to be slightly more common in women. Onset of PV is typically between 50 and 60 years of age although it can affect people of all ages.3 PV is extremely rare, affecting approximately 0.5–50 people per million.1 Due to its rare nature, PV may be mistaken for other skin blistering diseases and it can be challenging to achieve a diagnosis of PV.
The blisters that form in PV are fragile and easily burst creating lesions on the skin, in the mouth or on other parts of the body.1 These lesions can be painful, slow to heal and increase the risk of developing an infection due to the raw exposed skin.2
PV is a chronic condition and patients may experience ‘flares’ or ‘relapses’ during its course.2 There is currently no cure for PV but it can be effectively managed with treatment to stop the development of new blisters and to help affected areas to heal minimizing the impact of the condition on your daily life.2
The International Pemphigus and Pemphigoid Foundation (IPPF) is a nonprofit organization seeking to improve the quality of life for all people affected by pemphigus and pemphigoid through early diagnosis and support. Visit the IPPF website to learn more about PV (link: http://www.pemphigus.org/living-with-pemphigus-pemphigoid/all-about-pemphigus-patient-edition/) and to find out how you can contact patient support groups in your country (link: http://www.pemphigus.org/peer-support/local-support/)
References
- Kasperkiewicz M, et al. Nat Rev Dis Primers. 2017; 3:17026.
- Hertl M, et al. J Eur Acad Dermatol Venereol. 2015; 29:405-414.
- Alpsoy E, et al. Arch Dermatol Res. 2015; 307:291-298.
