Last reviewed by Dr. Raj MD on January 12th, 2022.
What is Pemphigus Vulgaris?
This medical condition is an autoimmune disorder which means this is a condition when your immune system mistakenly attacks and destroys your healthy body tissue. At this time there are more than eighty different types of autoimmune disorders. With this pemphigus vulgaris, this type of autoimmune disorder is a chronic skin blistering disease that results in skin lesions. These skin lesions are often times painful but are rarely pruritic, which means they do not itch and cause you to want to scratch the area. It usually affects your mouth and skin. This is considered an ultra-rare autoimmune disease/disorder and many times as many as eighty percent of the cases are misdiagnosed for an average of six months.
It does affect women and men equally and on average the symptoms first appear in people from fifty to sixty years of age but can affect younger people. It can affect any ethnic group but will usually appear more often in people who are Ashkenazi Jews and people of Mediterranean ancestry. It can also appear in some subtypes of people from Columbia and Brazil. In the United States there are approximately ten to thirty thousand people affected with this disease.
Pemphigus Vulgaris Symptoms
In approximately eighty percent of the cases, the first symptoms will appear in the mouth’s mucus membranes with erosions or flaccid (soft) blisters. Many times these first symptoms are mistaken for other mouth conditions so this is why it takes awhile for this disease to be properly identified. Eventually approximately two-thirds of these people will also experience lesions not only in the mouth but also on the skin. You may find these lesions not only in the mouth and on your skin but also on your scalp.
If you have skin sores they could be described as:
- Easily detached or peeling
These skin sores may also come and go. Generally this autoimmune disorder is characterized by blisters or lesions that do not heal. They will also recur and spread to bigger portions of your body. On average these ulcers will be there for weeks or could be months.
When a person has these blisters they could be accompanied by:
- Severe pain
- Sometimes itching but usually do not itch.
- Difficulty swallowing or eating if they are in your throat or mouth.
Pemphigus Vulgaris Causes
What exactly causes pemphigus vulgaris is not known but researchers believe it is an autoimmune disease. Research has shown that it is sometimes caused by certain medications although this is a rare cause. Some of the medications that they feel may cause pemphigus vulgaris are:
- Penicillamine which is a medication that is used to remove certain materials from your blood. It is considered a chelating agent.
- Medications that are used to treat your high blood pressure called ACE inhibitors.
If medication is the cause it will usually go away after stopping the medication.
The reason that researchers feel that is an autoimmune disease is that when a person has this medical condition their auto-antibodies will attack the molecules or proteins referred to as desmogleins that hold your skin cells together. When this happens it causes them to separate, resulting in blisters.
In order to confirm the diagnosis of pemphigus vulgaris the physician will usually need to do a skin biopsy; which is when a piece of the skin where the lesions are present is removed and sent to the laboratory to be tested. It can also be taken from the oral mucosa, which is the inner lining of your mouth. The test that they have done is called the direct immunofluorescence test.
Once the physician has the correct diagnosis they will start treatment to help reduce the symptoms along with helping to prevent any complications. In order for the treatment to be the most effective it needs to be started as early as possible because it is easier to control if it is not widespread. What treatments are used will depend on how severe the case is. If it is a severe case hospitalization may be needed and the care and treatment will be done in an intensive care or burn unit treating it as a severe burn. If pemphigus vulgaris is not treated it can begin to spread and cause complications such as metabolic disturbances, loss of fluid, and severe infections.
Mild pemphigus vulgaris treatment
- The main treatment to use is corticosteroids such as prednisone but these can have serious side effects if you use them in high doses or for a long period of time such as water retention, bone loss, and more. It can also lead to having a round face known as a moon face.
- Immunosuppressant – this type medication will help keep your immune system from attacking your healthy tissues but they can also have serious side effects such as a high risk of infection.
- Antibiotics, antifungal, and antiviral medications – this will be used to help control or prevent infections such as herpes viruses or staphylococcus bacteria.
Severe pemphigus vulgaris treatment
When you are required to be treated in a hospital because the sores are open and making you “at a high risk” for infection, these are the treatments that may be used along with the medications used to treat a mild case of pemphigus vulgaris.
- Fluids – when you are hospitalized with pemphigus vulgaris the sores are usually oozing and causing loss of fluids so normally you will be given fluids intravenously such as fluids containing calcium, proteins, sodium, electrolytes, and potassium.
- Intravenous feedings – this is usually done because of sores in your mouth
- Therapeutic plasmapheresis – using this process the plasma is removed from other parts of your blood using a device that is called a cell separator. The reason that this treatment would be done is to remove the plasma of any of the antibodies that are attacking your skin and then replacing it with either intravenous fluids or donated plasma.
If you have a severe case of pemphigus vulgaris and do not get it treated it can spread to your blood system and be deadly. For most people the treatments will have all the sores or blisters gone in a few months but there are some who have to remain on a low dose of the medication for an indefinite period of time so the symptoms do not return.
Pemphigus Vulgaris Pictures
Images, Photos and Pictures of Pemphigus Vulgaris…