Pemphigus Vulgaris: Causes, Symptoms, and Treatment
Medically reviewed by Harsha Moole, MD, Internal Medicine. Last reviewed: October 23, 2026.
What kind of doctor treats Pemphigus Vulgaris?
A dermatologist, a doctor who treats skin, hair, and nails, usually leads care for pemphigus vulgaris. A dentist or an ear, nose, and throat doctor may also help. A direct primary care or concierge doctor can be a first stop for an evaluation and can refer you to a specialist when needed.
What is pemphigus vulgaris?
Pemphigus vulgaris is an autoimmune disorder of the skin. In an autoimmune disorder, the body's defense system attacks its own healthy parts. This causes blisters and sores, called erosions, on the skin and on the mucous membranes. Mucous membranes are the moist linings inside the body, such as the mouth and throat.
The condition is uncommon. It most often happens in middle-aged or older people. Pemphigus vulgaris is the most common type of pemphigus.
Symptoms
The main sign is a soft blister filled with clear fluid. It can appear on healthy or irritated skin. Common signs include:
- Painful blisters and sores in the mouth, which are the first sign for about 50% to 70% of people.
- Skin blisters that follow the mouth sores and may come and go.
- Skin sores that drain, ooze, crust, or peel.
- Sores in the mouth and down the throat, on the face or in the eyes, and on the scalp, trunk, or other skin areas.
- Mouth blisters that make it hard to eat.
- Painful skin blisters that break open and limit daily activities.
Problems can include skin infections, severe dehydration, and infection that spreads through the blood. Doctors call blood infection sepsis. The damage from the blisters can also cause the body to lose fluids and protein.
Causes and who gets it
The immune system makes antibodies against specific proteins in the skin and mucous membranes. Antibodies are proteins that normally fight germs. These antibodies break the bonds between skin cells, and that leads to a blister. The exact cause is unknown. In rare cases, some medicines cause it, including penicillamine, blood pressure medicines called ACE inhibitors, and NSAIDs. NSAIDs are nonsteroidal anti-inflammatory drugs.
How doctors diagnose it
A doctor may rub the skin next to a blister with a cotton swab or finger. If the surface of unaffected skin separates easily, it is called a positive Nikolsky sign. A skin biopsy and blood tests are often done to confirm the diagnosis. For a biopsy, the dermatologist removes part of a new blister and some normal-looking skin next to it. The samples are looked at under a microscope in a lab. The blood test can show whether you have proteins in your blood that cause pemphigus. Your dermatologist will also ask about your health and the medicines you take.
Treatment
Treatment aims to reduce symptoms, including pain, and to prevent problems, especially infections. Treatment for the whole body is needed to control the condition and should start as early as possible. It often begins with medicines that quiet the immune system, such as rituximab, azathioprine, methotrexate, cyclosporine, cyclophosphamide, or mycophenolate mofetil. Corticosteroids are also used. For pemphigus in general, using a corticosteroid together with an immune-quieting medicine may give better results. The medicines can cause serious side effects.
Other care can help with symptoms. Doctors may use antibiotics and antifungal medicines to control or prevent infections. Numbing mouth lozenges can ease mouth ulcer pain. Soothing or drying lotions and wet dressings can treat blisters and ulcers. Plasmapheresis may be added to reduce the antibodies in the blood. In this process, plasma that holds antibodies is removed from the blood and replaced.
Severe cases may need wound care like the care for severe burns. Some people stay in a hospital burn unit or intensive care unit. With treatment, the disorder tends to be chronic, which means long lasting. The side effects of treatment may be severe or disabling. If pemphigus affects your eyes, you also need an eye doctor. For most people with pemphigus, treatment can keep the disease under control.
When to see a doctor
A health care provider should examine any unexplained blisters. Without treatment, this condition can be life threatening. If you have been treated for this condition, contact your provider if you develop certain symptoms. They are chills, fever, a general ill feeling, joint aches, muscle aches, and new blisters or ulcers. Call 911 or go to the emergency room if you have trouble breathing, faint, or have a sudden change in mental status, such as confusion.
How a direct primary care or concierge doctor fits in
A direct primary care doctor charges a flat monthly fee instead of billing insurance, while a concierge doctor usually charges more and often works alongside insurance. Such a doctor can look at unexplained blisters or mouth sores and review the medicines you take. They can send you to a dermatologist and help you keep up with follow-up visits during long-term treatment.
Common questions
Is pemphigus vulgaris the most common type of pemphigus?
Yes, the American Academy of Dermatology calls it the most common type.
Can pemphigus vulgaris be life threatening?
Yes, without treatment it can be life threatening. Severe infection is the most frequent cause of death. Few people die of pemphigus today because of medicines and other treatments.
Does pemphigus vulgaris start in the mouth?
Often, yes. About 50% to 70% of people first develop painful blisters and sores in the mouth, and skin blisters follow.
Can medicines cause pemphigus vulgaris?
Yes, but this is rare. Some medicines can also make pemphigus worse, so give your dermatologist a list of everything you take, including medicines you buy without a prescription.
Sources
- MedlinePlus - Pemphigus vulgaris
- NIH Genetic and Rare Diseases Information Center - Pemphigus vulgaris
- American Academy of Dermatology - Pemphigus: Diagnosis and treatment
Medical Disclaimer
This content is for general educational and informational purposes only and is not medical advice. It is not a substitute for professional medical advice, diagnosis, or treatment, and reading it does not create a doctor-patient relationship. Always seek the advice of your physician or another qualified health provider with any questions about a medical condition, and never disregard or delay seeking professional advice because of something you read here. If you think you may have a medical emergency, call 911 (or your local emergency number) immediately.
