Pemphigus vulgaris

What causes pemphigus vulgaris?

Pemphigus vulgaris is an autoimmune disorder. In the case of pemphigus vulgaris, the immune system mistakenly produces antibodies against specific proteins in the skin and mucous membranes, known as desmogleins. These proteins form the glue that keeps skin cells attached and 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.

Although it is rare, some cases of pemphigus vulgaris are caused by certain medications. Among others, medications that may cause this condition include:

Emotional stress, thermal burns, ultraviolet rays, and infections have also been reported as triggers for pemphigus vulgaris.

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

  • 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 increase 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 at any age.
  • Disease Association: Pemphigus occurs more commonly in people 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.

Last updated on 05-01-20

What is the prognosis for individuals with pemphigus vulgaris?

Recent advances in the treatment of pemphigus vulgaris have significantly improved the long term outlook for people with the disease. For most people, the disease can be controlled with prednisone (corticosteroids) in combination with other medications or therapies. However, both the use of steroids and the other medications or therapies can cause side effects that may sometimes be serious, including increasing the risk of developing a fatal infection. Pemphigus vulgaris and its treatments can be debilitating and may cause lost time at work, weight loss, loss of sleep, and emotional distress. Connecting with others facing the same concerns and challenges can help a person cope with the changes the disease causes in their life.

The International Pemphigus Foundation provides patient support services to help people with the disease cope with its effects.

Last updated on 05-01-20

How might pemphigus vulgaris be treated?

The goal of treatment of pemphigus vulgaris is to promote and maintain remission, as well as avoid complications, such as infections. Medications and therapies are used to decrease blister formation and promote healing of blisters and reduce scarring. Once in remission, it is important to determine the minimal dose of medication necessary to control the disease process, since both long term use of steroids and other immunosuppressive drugs can increase the risk of other medical problems. Treatment is individualized, and takes into account the person's other health conditions.

The most common first line therapy for pemphigus vulgaris is:

  • Steroids (corticosteroids), such as prednisone. Steroids can help a person go into remission and stay in remission, however this must be balanced with the medical problems that can be caused by the long term use of steroids.

Other immunosuppessives and immunotherapies may be used in addition to steroid therapy to reduce the risk of relapse or to reduce the dosage strength of the steroid being used. Again the choice of the additional drug or therapy (adjuvant therapy) must be balanced against the possible negative side effects.

Most common choices to be added to steroid treatment (first line adjuvant therapies) include:

Other choices may include:

Additional medications may be considered if a person does not achieve remission with a combination of steroids and one of the medications or therapies listed above.

Antibiotics, antivirals, and antifungals may be used to prevent or fight an infection. Wound care may include baths and wound dressings to help heal blisters and sores. Severe cases of pemphigus vulgaris may require hospital stays to receive proper wound care, as well as intravenous fluids and electrolytes if mouth ulcers are severe. Treatment may also involve pain medication or pain management therapies.

Last updated on 05-01-20

Name: International Pemphigus & Pemphigoid Foundation 1331 Garden Highway, Suite 100
Sacramento, CA, 95833, United States
Phone: 916-922-1298 Toll Free: 855-473-6744 Fax : 916-922-1458 Email: info@pemphigus.org Url: http://www.pemphigus.org

Note, these links are external searches against the National Laboratory of Medicine's drug database. You may need to adjust the search if there are no results found.

Drug Name Generic Name
Rituxan(R); Mabthera® rituximab

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