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Genetic and Rare Diseases Information Center (GARD)

Pemphigus vulgaris

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Your Question

I was diagnosed with pemphigus vulgaris in 2004 at the age of 54. Is this disease something that I have had all of my life? Was it just in remission until I got older? 

Our Answer

We have identified the following information that we hope you find helpful. If you still have questions, please contact us.

What is pemphigus vulgaris?

Pemphigus vulgaris is an autoimmune disorder that involves blistering of the skin and mucous membranes. It occurs almost exclusively in middle-aged or older people. Many cases begin with blisters in the mouth, followed by skin blisters that may come and go. In most cases, the exact cause of pemphigus vulgaris is unknown.[1] It has rarely been observed in multiple members of the same family.[2] Treatment is aimed at reducing symptoms and preventing complications. Severe cases are treated similarly to severe burns.[1]

Last updated: 3/16/2010

What causes pemphigus vulgaris?

Pemphigus vulgaris is an autoimmune disorder. The immune system produces antibodies against specific proteins in the skin and mucous membranes. These antibodies create a reaction that cause skin cells to separate.[1]

Although it is rare, some cases of pemphigus vulgaris are caused by certain medications. Medications that may cause this condition include:[1]

  • Blood pressure medications called ACE inhibitors
  • Chelating agents such as penicillamine, which remove certain materials from the blood

While in many cases the exact cause of pemphigus vulgaris remains unknown, several potentially relevant factors have been identified.[2][3]

  • Genetic factors: Predisposition to pemphigus is linked to genetic factors. Certain major histocompatibility complex (MHC) class II molecules, in particular alleles of human leukocyte antigen (HLA) DR4, appear to confer susceptibility to pemphigus vulgaris.
  • Age: Peak age of onset is from 50-60 years. Infants with neonatal pemphigus typically recover after protection from their mother's antibodies have cleared their systems. The disease may, nonetheless, develop in children or in older persons, as well.
  • Disease association: Pemphigus commonly occurs in individuals who also have other autoimmune diseases, particularly myasthenia gravis and thymoma.

Pemphigus is not contagious. It does not spread from person to person. Though there can be a genetic predisposition to develop pemphigus, there is no indication the disease is hereditary.[4]

Last updated: 3/16/2010

Have I always had pemphigus vulgaris? Was it in remission until now?

We are not able to comment on whether you have always had pemphigus vulgaris or if it has been in remission. What we do know is that it occurs almost exclusively in middle-aged or older people.[1] In addition, research studies suggest a genetic predisposition to the disease.[4]

Normally, our immune system produces antibodies that attack viruses and harmful bacteria to keep us healthy. In people with pemphigus, however, the immune system mistakenly attacks the cells in the epidermis, or top layer of the skin, and the mucous membranes. The immune system produces antibodies against proteins in the skin known as desmogleins. These proteins form the glue that keeps skin cells attached to keep the skin intact. When desmogleins are attacked, skin cells separate from each other and fluid can collect between the layers of skin, forming blisters that do not heal. In some cases, these blisters can cover a large area of skin.[4]

It is unclear what triggers the disease, although it appears that some people have a genetic susceptibility. Environmental agents may trigger the development of pemphigus in people who are likely to be affected by the disease because of their genes. In rare cases, it may be triggered by certain medications. In those cases, the disease usually goes away when the medication is stopped.[4]

We recommend that you speak with your doctor regarding questions you may have regarding your case.

Last updated: 3/16/2010

What is the prognosis for individuals with pemphigus vulgaris?

Without treatment, this condition is usually deadly. Generalized infection is the most frequent cause of death. With treatment, the disorder tends to be chronic in most cases. Side effects of treatment may be severe or disabling.[1] The International Pemphigus Foundation provides patient support services to help people with the disease cope with its effects.[4]
Last updated: 3/16/2010

See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.