St. Luke's Hospital
Located in Chesterfield, MO
Main Number: 314-434-1500
Emergency Dept: 314-205-6990 Patient Billing: 888-924-9200
Find a Physician Payment Options Locations & Directions
Follow us on: facebook twitter Mobile Email Page Email Page Print Page Print Page Increase Font Size Decrease Font Size Font Size
Meet the Doctor
Spirit of Women
Community Health Needs Assessment
Home > Health Information

Multimedia Encyclopedia


    Pemphigus vulgaris

    Pemphigus vulgaris is an autoimmune disorder that involves blistering and sores (erosions) of the skin and mucus membranes.


    Pemphigus is an autoimmune disorder. The immune system produces antibodies against specific proteins in the skin and mucus membranes. These antibodies break the bonds between skin cells. This leads to the formation of a blister. The exact cause is unknown.

    Sometimes pemphigus is caused by certain medications, although this is rare. Medications that may cause this condition include:

    • A drug called penicillamine, which removes certain materials from the blood (chelating agent)
    • Blood pressure medications called ACE inhibitors

    Pemphigus is uncommon. It almost always occurs in middle-aged or older people.


    About 50% of people with this condition first develop painful blisters and sores in the mouth, followed by skin blisters. Skin sores may come and go.

    The skin sores may be described as:

    • Draining
    • Oozing
    • Crusting
    • Peeling or easily detached

    They may be located:

    • In the mouth
    • On the scalp, trunk, or other skin areas

    Exams and Tests

    The skin separates easily when the surface of unaffected skin is rubbed sideways with a cotton swab or finger. This is called a positive Nikolsky's sign.

    A skin biopsy is usually done to confirm the diagnosis.


    Severe cases of pemphigus may need wound management, similar to the treatment for severe burns. People with this condition may need to stay in a hospital and receive care in a burn unit or intensive care unit.

    Treatment is aimed at reducing symptoms, including pain. It also aims to prevent complications, especially infections.

    Treatment may involve:

    • Antibiotics and antifungal medications to control or prevent infections
    • Fluids and electrolytes given through a vein (IV)
    • IV feedings if there are severe mouth ulcers
    • Numbing (anesthetic) mouth lozenges to reduce mouth ulcer pain
    • Pain medications if local pain relief is not enough

    Body-wide (systemic) therapy is needed to control pemphigus and should be started as early as possible. Systemic treatment includes:

    • An anti-inflammatory drug called dapsone
    • Corticosteroids
    • Medications containing gold
    • Medications that suppress the immune system (such as azathioprine, methotrexate, cyclosporin, cyclophosphamide, mycophenolate mofetil, or rituximab)

    However, side effects from systemic therapy are a major complication.

    Some antibiotics are also effective, particularly minocycline and doxycycline. Intravenous immunoglobulin (IVIg) is occasionally used.

    Plasmapheresis is a process in which antibody-containing plasma is removed from the blood and replaced with intravenous fluids or donated plasma. Plasmapheresis may be used along with systemic medications to reduce the amount of antibodies in the blood.

    Ulcer and blister treatments include soothing or drying lotions, wet dressings, or similar measures.

    Outlook (Prognosis)

    Without treatment, this condition is usually life-threatening. Severe infection is the most frequent cause of death.

    With treatment, the disorder tends to be chronic. Side effects of treatment may be severe or disabling.

    Possible Complications

    • Secondary skin infections
    • Severe dehydration
    • Side effects of medications
    • Spread of infection through the bloodstream (sepsis)

    When to Contact a Medical Professional

    Your health care provider should examine any unexplained blisters.

    Call your health care provider if you have been treated for pemphigus vulgaris and you develop any of the following symptoms:

    • Chills
    • Fever
    • General ill feeling
    • Joint aches
    • Muscle aches
    • New blisters or ulcers


    Habif TP. Vesicular and bullous diseases. In: Habif TP, ed. Clinical Dermatology. 5th ed. Philadelphia, Pa: Mosby Elsevier;2009:chap 16.

    Baroni A, Lanza A, Cirillo N, Brunetti G, Ruocco E, Ruocco V. Vesicular and bullous disorders: pemphigus. Dermatol Clin. 2007;25(4):597-603.


    • Pemphigus, vulgaris on t...


    • Pemphigus, vulgaris - le...


      • Pemphigus, vulgaris on t...


      • Pemphigus, vulgaris - le...


      Review Date: 7/11/2012

      Reviewed By: Kevin Berman, MD, PhD, Atlanta Center for Dermatologic Disease, Atlanta, GA. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.

      The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition. A licensed medical professional should be consulted for diagnosis and treatment of any and all medical conditions. Links to other sites are provided for information only -- they do not constitute endorsements of those other sites. © 1997- A.D.A.M., Inc. Any duplication or distribution of the information contained herein is strictly prohibited.

      A.D.A.M. content is best viewed in IE9 or above, Fire Fox and chrome browser.

      Back  |  Top
      About Us
      Contact Us
      Locations & Directions
      Quality Reports
      Annual Reports
      Honors & Awards
      Community Health Needs

      Brain & Spine
      Sleep Medicine
      Urgent Care
      Women's Services
      All Services
      Patients & Visitors
      Locations & Directions
      Find a Physician
      Tour St. Luke's
      Patient & Visitor Information
      Contact Us
      Payment Options
      Financial Assistance
      Send a Card
      Mammogram Appointments
      Health Tools
      My Personal Health
      Spirit of Women
      Health Information & Tools
      Clinical Trials
      Employer Programs -
      Passport to Wellness

      Classes & Events
      Classes & Events
      Spirit of Women
      Donate & Volunteer
      Giving Opportunities
      Physicians & Employees
      For Physicians
      Remote Access
      Medical Residency Information
      Pharmacy Residency Information
      Physician CPOE Training
      St. Luke's Hospital - 232 South Woods Mill Road - Chesterfield, MO 63017 Main Number: 314-434-1500 Emergency Dept: 314-205-6990 Patient Billing: 888-924-9200
      Copyright © St. Luke's Hospital Website Terms and Conditions  |  Privacy Policy  |  Notice of Privacy Practices PDF  |  Patient Rights PDF Sitemap St. Luke's Mobile