Overview Of Pemphigus Vulgaris (PV)
Pemphigus vulgaris (PV) is an autoimmune disorder of the skin. It involves blistering and sores (erosions) of the skin and mucous membranes.
Causes Of Pemphigus Vulgaris (PV)
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.
In rare cases, pemphigus vulgaris 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 Of Pemphigus Vulgaris (PV)
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 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.
A skin biopsy and blood tests are often done to confirm the diagnosis.
Treatment Of Pemphigus Vulgaris (PV)
Severe cases of pemphigus vulgaris 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.
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 are given through a vein (IV) if there are severe mouth ulcers
- 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 vulgaris 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.
Possible Complications
Complications of PV include:
- Secondary skin infections
- Severe dehydration
- Side effects of medicines
- Spread of infection through the bloodstream (sepsis)
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 ulcers
