BACK TOTOP Browse A-ZSearchBrowse A-ZABCDEFGHIJKLMNOPQRSTUVWXYZ0-9 E-mail FormEmail ResultsName:Email address:Recipients Name:Recipients address:Message: Print-FriendlyBookmarksbookmarks-menuPemphigus vulgarisPemphigus vulgaris (PV) is an autoimmune disorder of the skin. It involves blistering and sores (erosions) of the skin and mucous membranes.Autoimmune disorderAn autoimmune disorder occurs when the body's immune system attacks and destroys healthy body tissue by mistake. There are more than 80 types of aut...ImageRead Article Now Book Mark Article Mucous membranesMucosa is moist tissue that lines certain parts of the inside of your body. It is in your: NoseMouthLungsUrinary and digestive tracts Glands in this...ImageRead Article Now Book Mark Article Causes The immune system produces antibodies against specific proteins in the skin and mucous membranes. These antibodies break the bonds between skin cells. This leads to the formation of a blister. The exact cause is unknown.AntibodiesAn antibody is a protein produced by the body's immune system when it detects harmful substances, called antigens. Examples of antigens include micr...ImageRead Article Now Book Mark Article In rare cases, pemphigus is caused by some medicines, including:A medicine called penicillamine, which removes certain materials from the blood (chelating agent) Blood pressure medicines called ACE inhibitors Nonsteroidal anti-inflammatory drugs (NSAIDs)Pemphigus is uncommon. It most often occurs in middle-aged or older people. Symptoms About 50% of people with this condition first develop painful blisters and sores in the mouth. This is followed by skin blisters. Skin sores may come and go.The skin sores may be described as:Draining Oozing Crusting Peeling or easily detachedThey may be located:In the mouth and down the throat 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 sign.Nikolsky signNikolsky sign is a skin finding in which the top layers of the skin slip away from the lower layers when rubbed.ImageRead Article Now Book Mark Article A skin biopsy and blood tests are often done to confirm the diagnosis.Skin biopsyA skin lesion biopsy is when a small amount of skin is removed so it can be examined. The skin is tested to look for skin conditions or diseases. A...ImageRead Article Now Book Mark Article Treatment Severe cases of pemphigus may need wound management, similar to the treatment for severe burns. People with PV may need to stay in a hospital and receive care in a burn unit or intensive care unit.BurnsBurns commonly occur by direct or indirect contact with heat, electric current, radiation, or chemical agents. Burns can lead to cell death, which c...ImageRead Article Now Book Mark Article Treatment is aimed at reducing symptoms, including pain. It also aims to prevent complications, especially infections.Treatment may involve:Antibiotics and antifungal medicines to control or prevent infections Fluids and electrolytes given through a vein (IV) if there are severe mouth ulcers ElectrolytesElectrolytes are minerals in your blood and other body fluids that carry an electric charge. Electrolytes affect how your body functions in many ways...Read Article Now Book Mark Article IV feedings if there are severe mouth ulcers Numbing (anesthetic) mouth lozenges to reduce mouth ulcer pain Pain medicines 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 medicine called dapsone Corticosteroids Medicines containing gold Medicines that suppress the immune system (such as azathioprine, methotrexate, cyclosporine, cyclophosphamide, mycophenolate mofetil, or rituximab) Antibiotics may be used to treat or prevent infection. Intravenous immunoglobulin (IVIg) is occasionally used.Plasmapheresis may be used along with systemic medicines to reduce the amount of antibodies in the blood. Plasmapheresis is a process in which antibody-containing plasma is removed from the blood and replaced with intravenous fluids or donated plasma.Ulcer and blister treatments include soothing or drying lotions, wet dressings, or similar measures. Outlook (Prognosis) Without treatment, this condition can be 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.ChronicChronic refers to something that continues over an extended period of time. A chronic condition is usually long-lasting and does not easily or quick...ImageRead Article Now Book Mark Article Possible Complications Complications of PV include:Secondary skin infections Severe dehydration Side effects of medicines Spread of infection through the bloodstream (sepsis)SepsisSepsis is an illness in which the body has a severe, inflammatory response to bacteria or other germs.ImageRead Article Now Book Mark Article When to Contact a Medical Professional Your health care provider should examine any unexplained blisters.Call your provider if you have been treated for PV and you develop any of the following symptoms:Chills Fever General ill feeling Joint aches Muscle aches New blisters or ulcersOpen ReferencesReferencesAmagai M. Pemphigus. In: Bolognia JL, Schaffer JV, Cerroni L, eds. Dermatology. 4th ed. Philadelphia, PA: Elsevier; 2018:chap 29.Dinulos JGH. Vesicular and bullous diseases. In: Dinulos JGH, ed. Habif's Clinical Dermatology. 7th ed. Philadelphia, PA: Elsevier; 2021:chap 16.James WD, Elston DM, Treat JR, Rosenbach MA, Neuhaus IM. Chronic blistering dermatoses. In: James WD, Elston DM, Treat JR, Rosenbach MA, Neuhaus IM, eds. Andrew's Diseases of the Skin. 13th ed. Philadelphia, PA: Elsevier; 2020:chap 21.Patterson JW. The vesiculobullous reaction pattern. In: Patterson JW, ed. Weedon's Skin Pathology. 5th ed. Philadelphia, PA: Elsevier; 2021:chap 7. AllVideoImagesTogPemphigus vulgaris on the back - illustration Pemphigus vulgaris is an autoimmine disorder, where the body's immune system attacks some of the proteins in the skin. Pemphigus usually occurs in middle-aged or older people. This picture shows a close-up of the blistering on the back. Most of the blisters have broken (denuded), which is common since these blisters are fragile.Pemphigus vulgaris on the backillustrationPemphigus vulgaris - lesions in the mouth - illustration Pemphigus vulgaris is an autoimmine disorder, where the body's immune system attacks some of the proteins in the skin. Pemphigus usually occurs in middle-aged or older people. This picture shows a close-up of lesions in the mouth.Pemphigus vulgaris - lesions in the mouthillustrationPemphigus vulgaris on the back - illustration Pemphigus vulgaris is an autoimmine disorder, where the body's immune system attacks some of the proteins in the skin. Pemphigus usually occurs in middle-aged or older people. This picture shows a close-up of the blistering on the back. Most of the blisters have broken (denuded), which is common since these blisters are fragile.Pemphigus vulgaris on the backillustrationPemphigus vulgaris - lesions in the mouth - illustration Pemphigus vulgaris is an autoimmine disorder, where the body's immune system attacks some of the proteins in the skin. Pemphigus usually occurs in middle-aged or older people. This picture shows a close-up of lesions in the mouth.Pemphigus vulgaris - lesions in the mouthillustrationRelated Information Mucosa(Special Topic)Vesicles(Symptoms)Autoimmune disorders(Condition)Antibody(Special Topic)Bullae(Symptoms)Asymptomatic(Special Topic)Candida infection of the skin(Condition)Sepsis(Condition)Electrolytes(Special Topic) Review Date: 3/22/2020 Reviewed By: Elika Hoss, MD, Senior Associate Consultant, Mayo Clinic, Scottsdale, AZ. Also reviewed by David Zieve, MD, MHA, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team. 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