Pemphigus Vulgaris - A Rare Skin Disease and Treatment

What is Pemphigus Vulgaris?

Pemphigus Vulgaris is a disease that causes blisters and sores on the skin or mucous membranes, such as in the mouth or on the genitals Parts.

Pemphigus can occur at any age, but it's most often seen in people who are middle-aged or older. It tends to be a long-lasting (chronic) condition, and some types can be life-threatening without treatment. Treatment with medication usually controls it.

Causes

The exact cause of Pemphigus Vulgaris is unknown. Pemphigus is an autoimmune disorder. Normally, your immune system produces antibodies to fight off harmful invaders, such viruses and bacteria. 

But in Pemphigus, the body produces antibodies that damage cells of your own skin and mucous membranes. This attack on your healthy cells causes symptoms of blistering on your skin.


In rare cases, your body can negatively react to certain medicines that cause symptoms of pemphigus vulgaris.  

Symptoms

Symptoms of pemphigus vulgaris usually arise in your mouth, which causes painful blisters to form that can make eating and drinking difficult. After targeting the mucous membranes in your mouth, your symptoms can move to the skin on other parts of your body. 

These blisters are very thin and can easily break open. When your blisters break, they can leak fluid and cause your skin to peel, which turns the blisters into open sores.

Be careful not to injure or scratch your blisters, as they become open sores or wounds that can easily become infected.

Signs of an infection include:

• White or yellow pus leaks out of the blister. 

• The blister burns or you have severe pain.

• A yellow crust forms on or around the blister.

• The skin around your blister swells or gets bigger.

• Your blister doesn’t heal.

• Pain in and around the blisters.

• Fluid-filled bump (blister) that has a red halo around the outside and a light pink to white center.

The most common places where you’ll experience symptoms include:

• Mouth and throat.
• Nose.
• Eyes.
• Genitals.
• Chest.
• Back.
• Arms and legs.
• Scalp.

The signs and symptoms of two common types of pemphigus are as follows:

Pemphigus foliaceus

This type causes blisters on the chest, back and shoulders. The blisters tend to be more itchy than painful. Pemphigus foliaceus doesn't cause mouth blisters.

Pemphigus vulgaris 

This type usually begins with blisters in your mouth and then on your skin or genital mucous membranes. The blisters typically are painful but don't itch. Blisters in your mouth or throat may make it hard to swallow and eat.


Pemphigus is distinct from bullous pemphigoid, which is a blistering skin condition that affects older adults and may cause death.

Complications

Here are some common possible complication you can face, if you are suffering from Pempigus Vulagris

• Blisters and sore patches becoming infected, which can cause life-threatening.

• Weight loss and Malnutrition because painful mouth sores make it difficult to eat. 

• Tooth decay and gum disease because it can be painful to look after your teeth.

• Pain during sex and when peeing or pooing (if your genitals or anus are affected).

• Medication side effects, such as high blood pressure and infection.

• Death, if certain types of Pemphigus are left untreated.

• You'll have do regular check-ups to look for these problems and may be referred to other specialists if needed.

Risk factors

Pemphigus vulgaris is rare and can affect anyone, but it most often affects people over the age of 40. Pemphigus vulgaris rarely affects babies and children.

Things you can do if you have pemphigus vulgaris

• If you have Pemphigus Vulgaris, there are some things you can do to help ease the symptoms and reduce the risk of further problems.

• Clean your teeth using a soft toothbrush and mint-free toothpaste.

• Use antiseptic or painkilling (anaesthetic) mouthwash (try to avoid mouthwashes that contain alcohol).

• Keep any sore patches clean to reduce the risk of infection.

• Have regular dental check-ups

Things you should avoid if you have pemphigus vulgaris

• Do not eat spicy, acidic or hard foods if you have blisters in your mouth.

• Do not do activities that could damage your skin (such as contact sports) if you have blisters on your skin

DIAGNOSIS AND TESTS

How is Pemphigus Vulgaris diagnosed?

Your healthcare provider will diagnose pemphigus vulgaris after a physical exam to learn more about your symptoms and will ask questions about your health and medical history. They’ll also offer tests to diagnose your condition.

What tests diagnose pemphigus vulgaris?

To confirm a diagnosis, your healthcare provider might offer a skin biopsy. During a skin biopsy, your healthcare provider will remove a small sample of your tissue from an affected area of your skin to examine it under a microscope. This may include an immunofluorescence test.

Another test is a blood test to check for immune system antibodies that cause your symptoms.

TREATMENT

How is Pemphigus vulgaris treated?

Treatment for Pemphigus Vulgaris focuses on managing symptoms and preventing complications like infections. Treatment is unique to each person and could include:

• Taking medicines to treat infections.

• Stopping any medicines that cause symptoms.

• Using medicines, creams or ointments to treat, soothe and heal sores.

• Caring for sores and broken blisters as you would a burn or wound.

• Eating a bland diet or receiving nutrients through an IV to avoid malnutrition if sores in your mouth prevent you from eating.

• Using numbing anesthetic medicine to minimize pain from blisters in your mouth.

What medications treat pemphigus vulgaris?

Your healthcare provider might recommend taking medicines to reduce your symptoms, prevent flares of symptoms or treat infections. 

Medicines used to treat pemphigus include:

Corticosteroids: Medication to reduce inflammation (swelling), delivered by mouth, by injection (a shot) or topically (ointments or creams).

Rituximab: A monoclonal antibody that targets problematic B cells.

Immunosuppressive drugs: Drugs that manage your body’s autoimmune response, which is what happens when your body attacks healthy cells.

Antibiotics: In some cases, infections can develop in pemphigus blisters. If this happens, your doctor may prescribe antibiotic medicine to treat the infection.

Intravenous immunoglobulin: Healthy antibodies (proteins made by your immune system to attack foreign substances), given through a needle into your vein, to help reduce the antibodies that cause your diagnosis.

Are there side effects of the treatment?

Each treatment is different and there could be side effects depending on what medicine your healthcare provider prescribes. Before starting a new medicine, tell your healthcare provider about any medicines or supplements you actively take and about any allergies you have. 

They’ll monitor the progress of your condition and treatment by offering routine blood or urine tests to make sure you don’t have any side effects.

Side effects may vary based on the type of treatment and could include:

• Chest pain.
• Dizziness.
• Headache.
• Joint pain.
• Nausea.
• Upset stomach.
• Weight gain.

Your healthcare provider will monitor your condition regularly and order blood and/or urine tests to verify that your treatment is working. Tests also check for negative reactions to medications that treat pemphigus.

Frequently Asked Question

Is pemphigus vulgaris contagious?

No, Pemphigus vulgaris isn’t contagious. You can’t spread the condition to other peopl. In most cases, it's unknown what triggers the disease.

What can I eat or drink with pemphigus vulgaris?

Eating and drinking can be challenging with pemphigus vulgaris, especially if you have blisters and sores in your mouth. Choose foods that are soft, liquid and bland.

These will cause the least amount of irritation to your sores. Avoid foods that are acidic, spicy and crunchy, like citrus fruits, hot wings or potato chips.

If you can’t eat or drink, contact your healthcare provider.

How soon after treatment will I feel better?

The length of time you need to heal varies for each person. On average, it could take several weeks for new blisters to stop forming after you start treatment. After that, it could take several months for your skin to heal.

You should also be aware of that Pemphigus vulgaris is a chronic condition, which means that your symptoms can return after you receive treatment or you could experience symptoms throughout your life. Treatment can help reduce your symptoms.

How can I prevent pemphigus vulgaris?

You can’t prevent pemphigus vulgaris because the cause is unknown. Treatment is effective to reduce your symptoms.

What can I eat or drink with pemphigus?

Eating and drinking can be difficult if you develop blisters in your mouth and throat. Choose foods that are soft and bland. Avoid foods that are crunchy, acidic and spicy, which could irritate your blisters and cause pain.

If you have trouble eating, contact your healthcare provider. They may recommend taking nutritional supplements to avoid malnutrition.