Pemphigus vulgaris

Title

Categories:

Skin Diseases

Summary Summary

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

Cause Cause

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

Prognosis Prognosis

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

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Research Research

Research helps us better understand diseases and can lead to advances in diagnosis and treatment. This section provides resources to help you learn about medical research and ways to get involved.

Clinical Research Resources

ClinicalTrials.gov lists trials that are studying or have studied Pemphigus vulgaris. Click on the link to go to ClinicalTrials.gov to read descriptions of these studies.

Organizations Organizations

Nonprofit support and advocacy groups bring together patients, families, medical professionals, and researchers. These groups often raise awareness, provide support, and develop patient-centered information. Many are the driving force behind research for better treatments and possible cures. They can direct people to research, resources, and services. Many groups also have experts who serve as medical advisors. Visit their website or contact them to learn about the services they offer. Inclusion on this list is not an endorsement by GARD.

Organizations Supporting this Disease

International Pemphigus & Pemphigoid Foundation
1331 Garden Highway, Suite 100
Sacramento, CA 95833
Toll-free: 855-473-6744
Telephone: 916-922-1298
Fax: 916-922-1458
E-mail: info@pemphigus.org
Website: http://www.pemphigus.org

Social Networking Websites

RareConnect has an online community for patients and families with this condition so they can connect with others and share their experiences living with a rare disease. The project is a joint collaboration between EURORDIS (European Rare Disease Organisation) and NORD (National Organization for Rare Disorders).

Do you know of an organization? Send us your suggestions.

Living With Living With

Living with a genetic or rare disease can impact the daily lives of patients and families. These resources can help families navigate various aspects of living with a rare disease.

Genetics Resources

To find a medical professional who specializes in genetics, you can ask your doctor for a referral or you can search for one yourself. Online directories are provided by GeneTests, the American College of Medical Genetics, and the National Society of Genetic Counselors. If you need additional help, contact a GARD Information Specialist. You can also learn more about genetic consultations from Genetics Home Reference.

Learn More Learn More

These resources provide more information about this condition or associated symptoms. The in-depth resources contain medical and scientific language that may be hard to understand. You may want to review these resources with a medical professional.

Where to Start

MedlinePlus was designed by the National Library of Medicine to help you research your health questions, and it provides more information about this topic.
The National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS) support research into the causes, treatment, and prevention of arthritis and musculoskeletal and skin diseases, the training of basic and clinical scientists to carry out this research, and the dissemination of information on research progress in these diseases. Click on the link to view information on this topic.
The National Organization for Rare Disorders (NORD) has a report for patients and families about this condition. NORD is a patient advocacy organization for individuals with rare diseases and the organizations that serve them.

In-Depth Information

Medscape Reference provides information on this topic. You may need to register to view the medical textbook, but registration is free.
The Monarch Initiative brings together data about this condition from humans and other species to help physicians and biomedical researchers. Monarch’s tools are designed to make it easier to compare the signs and symptoms (phenotypes) of different diseases and discover common features. This initiative is a collaboration between several academic institutions across the world and is funded by the National Institutes of Health. Visit the website to explore the biology of this condition.
Online Mendelian Inheritance in Man (OMIM) is a catalog of human genes and genetic disorders. Each entry has a summary of related medical articles. It is meant for health care professionals and researchers. OMIM is maintained by Johns Hopkins University School of Medicine.
Orphanet is a European reference portal for information on rare diseases and orphan drugs. Access to this database is free of charge.
PubMed is a searchable database of medical literature and lists journal articles that discuss Pemphigus vulgaris. Click on the link to view a sample search on this topic.

GARD Answers GARD Answers

Questions sent to GARD may be posted here if the information could be helpful to others. We remove all identifying information when posting a question to protect your privacy. If you do not want your question posted, please let us know. Submit a new 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? See answer

Have a question? Contact a GARD Information Specialist.

References References

Lehrer, M. Pemphigus vulgaris. MedlinePlus. 2008; http://www.nlm.nih.gov/medlineplus/ency/article/000882.htm. Accessed 3/16/2010.
Pemphigus Vulgaris, Familial. Online Mendelian Inheritance in Man (OMIM). 2009; http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=169610. Accessed 3/16/2010.
Zeina B, Sakka N, Mansoor S. Pemphigus Vulgaris. eMedicine. 2010; http://emedicine.medscape.com/article/1064187-overview. Accessed 3/16/2010.
Vanchieri C, Baker C, Moshell A, Bystryn J, Diaz L, Stanley J. Pemphigus. National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS). 2007; http://www.niams.nih.gov/Health_Info/Pemphigus/default.asp. Accessed 3/16/2010.