Learn About Pemphigus Vulgaris
Pemphigus vulgaris (PV) is an autoimmune disorder of the skin. It involves blistering and sores (erosions) of the skin and mucous membranes.
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, PV 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)
PV is uncommon. It most often occurs in middle-aged or older people.
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
Severe cases of PV 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 PV 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.
Penn Dermatology Perelman
Victoria Werth is a Dermatologist in Philadelphia, Pennsylvania. Dr. Werth is rated as an Elite provider by MediFind in the treatment of Pemphigus Vulgaris. Her top areas of expertise are Cutaneous Lupus Erythematosus (CLE), Dermatomyositis, Discoid Lupus Erythematosus (DLE), and Pemphigus. Dr. Werth is currently accepting new patients.
Emory Clinic At 1525 Clifton Road
Ron Feldman is a Dermatologist in Atlanta, Georgia. Dr. Feldman has been practicing medicine for over 20 years and is rated as an Elite provider by MediFind in the treatment of Pemphigus Vulgaris. His top areas of expertise are Pemphigus Foliaceus, Pemphigus, Bullous Pemphigoid, and Pemphigus Vulgaris.
Penn Dermatology Perelman
Christoph Ellebrecht is a Dermatologist in Philadelphia, Pennsylvania. Dr. Ellebrecht is rated as an Elite provider by MediFind in the treatment of Pemphigus Vulgaris. His top areas of expertise are Pemphigus, Pemphigus Vulgaris, Pemphigus Foliaceus, and Bullous Pemphigoid. Dr. Ellebrecht is currently accepting new patients.
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.
Complications of PV include:
- Secondary skin infections
- Severe dehydration
- Side effects of medicines
- Spread of infection through the bloodstream (sepsis)
Your health care provider should examine any unexplained blisters.
Contact 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
Summary: ONO-4059-10:Multicenter, placebo-controlled, randomized, double-blind, Phase 3 study in patients with steroid-resistant pemphigus
Summary: A phase 1/2, open-label, safety and dosing study of autologous CART cells (desmoglein 3 chimeric autoantibody receptor T cells \[DSG3-CAART\] or CD19-specific Chimeric Antigen Receptor T cells \[CABA-201\]) in subjects with active, pemphigus vulgaris
Published Date: May 28, 2024
Published By: Ramin Fathi, MD, FAAD, Director, Phoenix Surgical Dermatology Group, Phoenix, AZ. Also reviewed by David C. Dugdale, MD, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team.
Amagai M. Pemphigus. In: Bolognia JL, Schaffer JV, Cerroni L, eds. Dermatology. 5th ed. Philadelphia, PA: Elsevier; 2025: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.