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Pemphigus vulgaris
Pemphigus vulgaris (PV) is an autoimmune disorder of the skin. It involves blistering and sores (erosions) of the skin and mucous membranes .
Autoimmune disorder
An 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...
Mucous membranes
Mucosa is moist tissue that lines certain parts of the inside of your body. It is in your: NoseMouthLungsUrinary and digestive tracts Glands in this...
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.
Antibodies
An antibody is a protein produced by the body's immune system when it detects harmful substances, called antigens. Examples of antigens include micr...
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
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 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 sign .
Nikolsky sign
Nikolsky sign is a skin finding in which the top layers of the skin slip away from the lower layers when slightly rubbed.
A skin biopsy is often done to confirm the diagnosis.
Skin biopsy
A 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...
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.
Burns
Burns commonly occur by direct or indirect contact with heat, electric current, radiation, or chemical agents. Burns can lead to cell death....
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
Electrolytes
Electrolytes 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 drug 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.
Chronic
Chronic refers to something that continues over an extended period of time. A chronic condition is usually long-lasting and does not easily or quick...
Possible Complications
Complications of PV include:
- Secondary skin infections
- Severe dehydration
- Side effects of medicines
-
Spread of infection through the bloodstream (
sepsis
)
Sepsis
Sepsis is an illness in which the body has a severe, inflammatory response to bacteria or other germs.
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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
General ill feeling
Malaise is a general feeling of discomfort, illness, or lack of well-being.
Read Article Now Book Mark Article - Joint aches
-
Muscle aches
Muscle aches
Muscle aches and pains are common and can involve more than 1 muscle. Muscle pain also can involve ligaments, tendons, and fascia. Fascia are the s...
ImageRead Article Now Book Mark Article - New blisters or ulcers
References
Ferri FF. Pemphigus vulgaris. In: Ferri FF, ed. Ferri's Clinical Advisor 2016 . Philadelphia, PA: Elsevier; 2016:941-942.
Habif TP. Vesicular and bullous diseases. In: Habif TP, ed. Clinical Dermatology . 6th ed. Philadelphia, PA: Elsevier; 2016:chap 16.
James WD, Berger TD, Elston DM. Chronic blistering dermatoses. In: James WD, Berger TG, Elston DM, eds. Andrew's Diseases of the Skin . 12th ed. Philadelphia, PA: Elsevier; 2016:chap 21.
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Pemphigus, 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 back
illustration
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Pemphigus, 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 mouth
illustration
-
Pemphigus, 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 back
illustration
-
Pemphigus, 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 mouth
illustration
Review Date: 4/29/2016
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, Isla Ogilvie, PhD, and the A.D.A.M. Editorial team.
