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Pemphigus Vulgaris: Causes Symptoms & Treatment

Pemphigus Vulgaris: Causes Symptoms & Treatment

Pemphigus Vulgaris Treatment in Delhi, India


What Is Pemphigus Vulgaris?

Pemphigus vulgaris is a not so common autoimmune disease. This rare disease causes painful blistering on the skin and mucous membranes. If you are suffering from an autoimmune disease, your immune system unintentionally attacks your healthy tissues.

Pemphigus vulgaris is the most common kind of a group of autoimmune diseases known as pemphigus. Each type of pemphigus is represented by the area where the blisters occur.

Pemphigus vulgaris affects the mucous membranes, which are found in areas including the:

  • mouth
  • throat
  • genitals
  • lungs
  • nose
  • eyes

This disease generally begins with blisters in the mouth and then spreading on the skin. The blisters at times also have an effect on the membranes of the genitals.

Pemphigus vulgaris disease can be menacing. Treatment is necessary, and it principally involves the use of corticosteroids to subdue the immune system. The skin disorder can cause serious issues if it is not treated properly and on time. Some of these complications can be deadly.

The reports of death rate from this fatal disease has averaged 75% before corticosteroids were introduced in the 1950s. This rate has shown drastic decline with current time’s treatments.

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What Are the Symptoms of Pemphigus Vulgaris?

The most common symptoms of pemphigus vulgaris include:

  • painful blisters that begins forming in the mouth or then on other locations
  • crusting, peeling or seeping at the blister site
  • skin blisters around the surface of the skin that come and go



What Causes Pemphigus Vulgaris?

The immune system forms proteins referred to as antibodies. Antibodies ordinarily attack dangerous foreign compounds like bacteria, bacterial enzymes, viruses and germs. Pemphigus vulgaris happens when the immune system fallaciously makes antibodies against proteins in healthy skin and mucous membranes.

The antibodies break down the connection between the cells, and fluid accumulates in between the layers of the skin. This results in blisters and abrasion on the skin.

The exact cause of the attack by the immune system is still not known.

Very seldom, certain medications can cause pemphigus vulgaris. These drugs include:

  • Penicillamine, which is a chelating agent that eliminates certain substances from the blood

ACE inhibitors, which are a kind of blood pressure medication



Types of Pemphigus

The several types of pemphigus are diagnosed based on the site of the blisters. They include:


Pemphigus Vulgaris

This is the most common type of pemphigus in the Unites States. Blisters typically first show up in the mouth. The blisters usually do not itch but can be painful. Blisters may then occur on the skin and occasionally on the genitals.


Paraneoplastic Pemphigus

It is a very rare type of pemphigus that appears in individuals with some cancers which is called paraneoplastic pemphigus. The ulcers and blisters may show up in the mouth, on the lips, and then on the skin. This type is also reported to cause scars on the eyes and eyelids. It can also precipitate lung issues.


Pemphigus Foliaceus

This type of Pemphigus do not form blisters in the mouth. The blisters initially show up on the face and scalp and then occur on the chest and back. The blisters are generally itchy but painless.


Pemphigus Vegetans

It causes blisters that occurs on the crotch area, on the feet and under the arms.



How Is Pemphigus Vulgaris Diagnosed?

A dermatologist or a skin specialist doctor will perform a physical examination of your skin blisters. They will search for the signal of the condition termed as Nikolsky’s sign. A positive indicator of Nikolsky’s is when your skin snip away easily,  the surface is wiped edgewise with a sponge, cotton swab or a gentle finger.

Your doctor may then conduct a biopsy of the blister, which involves eliminating a portion of tissue for assessment and check it under a microscope to assert the diagnosis. The biopsy may be undertaken in the laboratory with chemical compounds that help your doctor identify the peculiar antibodies. Your doctor can use this detail to discern the type of pemphigus.



How Is Pemphigus Vulgaris Treated?

Treatment is targeted at decreasing pain, itchiness and other symptoms and impeding risk factors such as infection. It includes one or more drugs and other medications and methods. This may involve any of the following:


Corticosteroids and Immune-Suppressing Drugs

A high dose of corticosteroids is the “key” treatment for the Pemphigus Vulgaris condition. The most frequently prescribed corticosteroids include prednisolone, predenisone. A high dose is generally required to keep the condition under control at first.

These drugs have many some complications, including:

  • an increased chance of getting infected
  • increased blood sugar
  • diabetes
  • cataracts
  • glaucoma
  • a loss of muscle mass
  • stomach ulcers
  • water retention
  • osteoporosis (decrease in bone mass density)

You may require consuming supplements such as vitamin D and calcium, having a low sugar diet, or simultaneously using other medications to treat these complications. Once the blisters are put under control, the dose can be lowered to the lowest level necessitated to halt new blisters from appearing and to suppress the side effects in a best possible way. A corticosteroid ointment can be applied directly on the blisters as well.

To enable keeping the low doses of corticosteroids, your health care provider may prescribe extra medications that repress the immune system. These include:

  • mycophenolate mofetil
  • azathioprine
  • cyclophosphamide
  • methotrexate
  • rituximab


Intravenous (IV) Feeding

If you have some serious mouth sores and ulcers, you would not be able to eat, drink or even speak without pain. You may be required to be fed in through your veins. This includes using an intravenous (IV) connection.



In utterly severe cases, an individual may undergo a procedure called plasmapheresis. This procedure is deliberated to eradicate the antibodies attacking the skin from the blood. During this plasmapheresis procedure, the plasma, or fluid part of the blood, is discarded by an instrument and replaced with donated plasma. This treatment can be costly.


Antibiotics, Antifungals and Antivirals

Any of these may be given by the doctor to prevent other infections.

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Wound Management

If the blisters are severe, you may be required to stay in the hospital to undergo a wound treatment. This treatment is going to be similar to what’s provided for severe burns. You may require receiving electrolytes and IV fluids in case you have lost excessive fluid by the seeping of the blisters.

The treatment for the blisters may also include:

  • numbing lozenges for mouth blisters, sores or ulcers
  • soothing ointments
  • pain medications
  • wet dressings
  • avoidance of acidic or spicy foods that may agitate the blisters
  • avoidance of excessive sun exposure
  • soft or pulpy food diets and liquids

If the blisters in your mouth prevent you from brushing or flossing your teeth, you may also require having a special oral health care treatment plan to curb tooth decay and gum disease. Go to your dentist to discuss them about oral care.


Biologic injections and drugs

Biologic injections have proven to be of huge advantage for diseases where there is no indicated treatment, like for immunobullous diseases. Conventional treatment for pemphigus vulgaris is systemic steroids and immunosuppressive agents like azathioprine and cyclosporine and/or methotrexate.



“A relatively rare condition “Pemphigus vulgaris” with rituximab (Rituxan, Genentech) has been a considerable advance, although is not a officially approved indication for the drug,” “In the past decade, rituximab has been used by dermatologists in pemphigus vulgaris, an autoimmune blistering disorder impacting the skin and mucous membranes with very substantial responses reported.

Recently a Clinical Immunology assessed data on 153 patients for pemphigus vulgaris kept on rituximab. They deduced that the mechanism of action is B-cell modulation and that its use has escalated clinical suspension degree to 65 from 30 % with traditional therapies.

The clininical Medicos suggested that rituximab can be used to treat patients with pemphigus vulgaris if they fail to respond well to traditional therapy or have developed severe side effects due to its usage. They counseled that the likeliness of extended B-cell exhaustion supports scanning of patients for peripheral B-cell counts

Pemphigus vulgaris has also been treated with a mix of 2 medications that are: rituximab and intravenous immunoglobulin (IVIG). In a small study conducted, patients were first  treated with rituximab followed by IVIG. Researchers noted that 9 out of 11 patients had sped up clinical remission and resolution of lesions.


Etanercept (Enbrel, Amgen/Pfizer)

Etanercept (Enbrel, Amgen/Pfizer) is approved and indicated for the treatment of psoriasis in pediatric patients in Europe, but not in the United States. Medicos also seek out for other tumor necrosis factor-alpha (TNF-alpha) blockers biologic agents if children, youngsters and adolescents do not respond well to etanercept.

“Adalimumab (Humira, Abbott) and infliximab (Remicade, Janssen Biotech) are also not approved (to treat psoriasis) for adolescents under 18, but we do use both drugs in recalcitrant cases in kids and adolescents. As with using drugs or therapies that are indicated for particular conditions, doctors must watch out closely at adverse the side-effect profile of a biologic if they decide to use it in an off-label trend, and measure the adversities and advantages of using these drugs cautiously.


Risk Factors

Who Is at Risk for Pemphigus Vulgaris?

Pemphigus vulgaris isn’t communicable and cannot be transferrable from one individual to another. It also does not seem to be transferable from parent to child. However, an individual’s genes can place them at a greater risk for the condition. If either of your parent or other close family members (siblings, grandparents) had or have the condition, you have got more chances to develop it.

Pemphigus vulgaris can affect people of all races, ages and genders. However, the condition is more often seen in the following groups:

  • middle-aged and older adults
  • people of eastern European Jews
  • people of Mediterranean descent
  • people living in the rainforests in Brazil



What Are the Complications of Pemphigus Vulgaris?

The complications of pemphigus vulgaris can be severe and sometimes life threatening

They may include:

  • skin infections and allergies
  • sepsis, or the outspread of infection by way of the bloodstream
  • dehydration
  • serious side effects of medication



What Is the Long-Term Outlook for People with Pemphigus Vulgaris?

If left untreated, pemphigus vulgaris can become lethal. Often the most typical cause of death is a serious secondary infection.

Pemphigus vulgaris is a long-standing condition for life. It cannot be easily cured. Many people have also been announced to go into suspension after taking corticosteroids. Improvement is generally visible within days of beginning the corticosteroids.

The blisters will heal gradually, particularly the ones in the mouth. On an average, the blisters are prevented from formation in 15 to 20 days time. Healing of the blisters takes 6 to 8 weeks on an average. However, full healing may at times take years. Some people may require to rest on a low dose of the medication for their entire life.

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