Learn About Pemphigus Vulgaris

What is the definition of Pemphigus Vulgaris?

Pemphigus vulgaris (PV) is an autoimmune disorder of the skin. It involves blistering and sores (erosions) of the skin and mucous membranes.

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What are the causes of Pemphigus Vulgaris?

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 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.

What are the symptoms of Pemphigus Vulgaris?

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
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What are the current treatments for Pemphigus Vulgaris?

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.

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
  • 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.

Who are the top Pemphigus Vulgaris Local Doctors?
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Dermatology

UCI Health Gottschalk Medical Plaza

Irvine, CA 

Sergei Grando is a Dermatologist in Irvine, California. Dr. Grando has been practicing medicine for over 42 years and is rated as an Elite doctor by MediFind in the treatment of Pemphigus Vulgaris. He is also highly rated in 38 other conditions, according to our data. His top areas of expertise are Pemphigus, Pemphigus Vulgaris, Pemphigus Foliaceus, and Bullous Pemphigoid. He is board certified in Dermatology and licensed to treat patients in California. Dr. Grando is currently accepting new patients.

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Highly rated in
10
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Dermatology

Perelman Center For Advanced Medicine

Philadelphia, PA 

A Payne is a Dermatologist in Philadelphia, Pennsylvania. Dr. Payne has been practicing medicine for over 21 years and is rated as an Elite doctor by MediFind in the treatment of Pemphigus Vulgaris. She is also highly rated in 10 other conditions, according to our data. Her top areas of expertise are Pemphigus, Pemphigus Vulgaris, Pemphigus Foliaceus, and Bullous Pemphigoid. She is board certified in Dermatology and licensed to treat patients in Pennsylvania. Dr. Payne is currently accepting new patients.

 
 
 
 
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Elite
Highly rated in
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Rouen University Hospital

Rouen, FR 

Pascal Joly is in Rouen, France. Joly is rated as an Elite expert by MediFind in the treatment of Pemphigus Vulgaris. He is also highly rated in 19 other conditions, according to our data. His top areas of expertise are Pemphigus Vulgaris, Pemphigus, Pemphigus Foliaceus, and Bullous Pemphigoid.

What is the outlook (prognosis) for Pemphigus Vulgaris?

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.

What are the possible complications of Pemphigus Vulgaris?

Complications of PV include:

  • Secondary skin infections
  • Severe dehydration
  • Side effects of medicines
  • Spread of infection through the bloodstream (sepsis)
When should I contact a medical professional for Pemphigus Vulgaris?

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
Pemphigus vulgaris on the back
Pemphigus vulgaris - lesions in the mouth
What are the latest Pemphigus Vulgaris Clinical Trials?
A Randomized, Double-Blinded, Placebo-Controlled Trial to Investigate the Efficacy, Safety, and Tolerability of Efgartigimod PH20 SC in Adult Patients With Pemphigus (Vulgaris or Foliaceus)
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Phenotypic and Functional Characterisation of Human B-cell Response in Pemphigus and Application to Other Auto-immune Diseases
What are the Latest Advances for Pemphigus Vulgaris?
Regional Differences in the Permeability Barrier of the Skin-Implications in Acantholytic Skin Diseases.
Anti-Desmocollin Autoantibodies in Autoimmune Blistering Diseases.
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Diagnostic Concordance between Optical Coherence Tomography and Histological Investigations for Immune-Mediated Desquamative Gingivitis: Observational Study.
What are our references for Pemphigus Vulgaris?

Amagai 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.