National Center for Advancing and Translational Sciences Genetic and Rare Diseases Information Center, a program of the National Center for Advancing and Translational Sciences

Pemphigus


Pemphigus is a group of rare autoimmune diseases that cause blistering of the skin and mucous membranes (mouth, nose, throat, eyes, and genitals).[1] This condition can occur at any age, but often strikes people in middle or older age. Studies have shown that some populations may be at greater risk for certain types of pemphigus. For instance, people of Jewish descent and those from India, Southeast Europe, and the Middle East are at greater risk for pemphigus vulargis, while pemphigus foliaceus is more common in North America, Turkey, and South America.[2] Pemphigus is a chronic disease which is best controlled by early diagnosis and treatment.[3] Treatment includes steroids to reduce inflammation, drugs that suppress the immune system response and antibiotics to treat associated infections.[1][4]

There are four main types of pemphigus:[2]

  • Pemphigus vulgaris
  • Pemphigus foliaceus
  • IgA pemphigus
  • Paraneoplastic pemphigus
    • Last updated: 8/12/2016
    Treatment of pemphigus may consist of three phases: control, consolidation, and maintenance. The control phase involves intense therapy until no new lesions develop. In the consolidation phase, therapy continues until existing lesions have cleared. In the maintenance phase, medications are gradually reduced to the lowest possible dose that prevents the development of new lesions.[5] Different medications may be used alone or in combination depending on the severity in each person.[6] Medications that may be used to treat pemphigus include:[6][5][1][7]
    • Corticosteroids - topical corticosteroids may be enough for people with mild pemphigus, but many people need systemic (oral) corticosteroids.
    • Immunosuppressants - these prevent the immune system from mistakenly attacking healthy tissue. Examples include azathioprine, methotrexate, mycophenolate, cyclophosphamide, and cyclosporine.
    • Rituximab - this is an injection that may be used if other medications are causing serious side effects or are ineffective. It is a biologic therapy that targets the cells responsible for making pemphigus antibodies.
    • IVIG (intravenous immune globulin)- this is an intravenous treatment that may be given over the course of several days.
    • Plasmapheresis (also called plasma exchange) - this is a therapy that removes the responsible antibodies from the blood.
    Last updated: 11/30/2017

    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 related to Pemphigus. Click on the link to go to ClinicalTrials.gov to read descriptions of these studies.

      Please note: Studies listed on the ClinicalTrials.gov website are listed for informational purposes only; being listed does not reflect an endorsement by GARD or the NIH. We strongly recommend that you talk with a trusted healthcare provider before choosing to participate in any clinical study.

    Patient Registry

    • A registry supports research by collecting of information about patients that share something in common, such as being diagnosed with Pemphigus. The type of data collected can vary from registry to registry and is based on the goals and purpose of that registry. Some registries collect contact information while others collect more detailed medical information. Learn more about registries.

      Registries for Pemphigus:
      International Pemphigus & Pemphigoid Foundation Natural History Registry
       

    Support and advocacy groups can help you connect with other patients and families, and they can provide valuable services. Many develop patient-centered information and are the driving force behind research for better treatments and possible cures. They can direct you to research, resources, and services. Many organizations also have experts who serve as medical advisors or provide lists of doctors/clinics. Visit the group’s 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

    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).

    Organizations Providing General Support

    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 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.
    • MayoClinic.com provides information about pemphigus. Click on the above link to access this information.

    In-Depth Information

    • 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.
    • PubMed is a searchable database of medical literature and lists journal articles that discuss Pemphigus. Click on the link to view a sample search on this topic.

    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

    • Are there medications besides prednisone which can successfully treat pemphigus? What about "alternative" medical treatments? See answer


    1. Questions and Answers about Pemphigus. National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS). June 2015; http://www.niams.nih.gov/Health_Info/Pemphigus/default.asp.
    2. All About Pemphigus (Patient Edition)Pemphigus. International Pemphigus & Pemphigoid Foundation. 2016; http://www.pemphigus.org/living-with-pemphigus-pemphigoid/all-about-pemphigus-patient-edition/.
    3. Pemphigus. MayoClinic.com. 2016; http://www.mayoclinic.org/diseases-conditions/pemphigus/home/ovc-20157520?DSECTION=all&p=1.
    4. Pemphigus. MedlinePlus. July 13, 2016; https://www.nlm.nih.gov/medlineplus/pemphigus.html.
    5. Treatments. Pemphigus & Pemphigoid Foundation. 2016; http://www.pemphigus.org/research/clinically-speaking/treatments/.
    6. Pemphigus. Mayo Clinic. August 4, 2017; https://www.mayoclinic.org/diseases-conditions/pemphigus/diagnosis-treatment/drc-20350409.
    7. Tsuruta D, Ishii N, Hashimoto T. Diagnosis and Treatment of Pemphigus. Immunotherapy. 2012; 4(7):735-745. https://www.medscape.com/viewarticle/769843_1.