What Is Pemphigus Vulgaris?
Pemphigus vulgaris is a rare autoimmune disorder presented by painful blistering and erosions on the skin and mucous membranes like the mouth, nose, throat, eyes, and genitals. This is the most common type of pemphigus (a group of autoimmune disorders). The other two common subtypes of pemphigus are pemphigus foliaceus and paraneoplastic pemphigus. Each subtype of pemphigus is characterized by where the blisters form.
How Common Is Pemphigus Vulgaris?
Pemphigus vulgaris represents 70 % of all pemphigus cases worldwide. The condition can affect people of all ages, gender, ethnicity, or race, but it is more common in middle-aged or older people. It is a long-lasting (chronic) condition, and some types of pemphigus can be fatal without treatment.
What Causes Pemphigus Vulgaris?
Normally, the immune system produces proteins called antibodies. Antibodies help attack harmful foreign substances such as bacteria and viruses. But in pemphigus vulgaris, the immune system mistakenly produces antibodies against proteins in healthy skin and mucous membranes. As a result, the antibodies break down the bonds between the cells and fluid pools up between the layers of the skin. This leads to blisters and erosions. Rarely, certain medications can also trigger pemphigus vulgaris.
What Are the Symptoms of Pemphigus Vulgaris?
Most pemphigus vulgaris cases start in the mouth. Half of the people with this condition first develop painful blisters and sores in the mouth, followed by skin blisters. Skin sores may come and go.
Symptoms may include:
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Blisters on healthy skin.
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Blisters that are easy to burst.
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Affected skin that peels easily when rubbed.
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Painful blisters.
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The skin sores may be draining, oozing, crusting, peeling, or easily detached.
Who Is at More Risk for Pemphigus Vulgaris?
Pemphigus vulgaris is not contagious and cannot be passed from one person to another. However, an individual’s genes can increase the risk for the condition. In addition, if the parents or other family members had or have this condition, the child is more likely to develop it. However, pemphigus vulgaris can affect anyone irrespective of race, gender, and age.
However, the condition is more often seen in the following groups:
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Middle-eastern or Jews.
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People of Mediterranean descent.
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People who live in rainforests in Brazil.
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Middle-aged and older adults.
What Are the Complications of Pemphigus Vulgaris?
Pemphigus vulgaris complications can be life-threatening and severe. They may include:
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Skin infection.
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Infection that spreads to the bloodstream (sepsis)
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Painful mouth sores may cause difficulty eating, resulting in malnutrition.
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Adverse effects of medications, such as high blood pressure and infections.
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Dehydration.
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Death if some forms of pemphigus vulgaris are left untreated.
How Is Pemphigus Vulgaris Diagnosed?
Blisters also occur with many other common conditions, so pemphigus, which is rare, can sometimes be difficult to diagnose. During the visit, a physician diagnoses based on the medical history and physical examination of the skin and mouth. In addition, the doctor may undergo the following tests:
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Skin biopsy, in which a piece of tissue from a blister is removed and examined under a microscope.
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Blood tests help detect and identify antibodies in the blood that are known to be present with pemphigus vulgaris.
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An endoscopy may be done to look for sore throat by inserting a flexible tube (endoscope) down the throat.
How Is Pemphigus Vulgaris Treated?
Treatment usually involves medications that suppress blister formation and prevent complications, especially infections. It is more effective when treatment is started as early as possible.
Medications:
Depending on the type and severity of the pemphigus vulgaris, the following prescription medications may be used alone or in combination:
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Good oral hygiene is needed as blistering may affect the mouth's health.
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Antibiotics and antifungal medicines may help control or prevent infections.
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Fluids and electrolytes may be given intravenously if there are severe mouth ulcers.
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Numbing (anesthetic) mouth lozenges to ease mouth ulcer pain.
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Pain medications if local pain relief is not enough
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Systemic therapy may be needed to control pemphigus vulgaris and should be started as early as possible. Systemic therapy includes:
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An anti-inflammatory medicine called Dapsone.
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Corticosteroids.
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Immunosuppressant drugs such as Azathioprine, Methotrexate, Cyclosporine, Cyclophosphamide, Mycophenolate Mofetil, or Rituximab help keep the immune system from attacking healthy tissue.
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Intravenous immunoglobulin (IVIg) may be used in some cases.
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Plasmapheresis may be combined with systemic medicines to decrease the number of antibodies in the blood.
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Treatments for ulcers and blisters include soothing or drying lotions and wet dressings.
The blisters often heal slowly, especially the ones present in the mouth. On average, the blister formation stops in two to three weeks. Healing of the blisters may take six to eight weeks. However, full recovery can sometimes take years. Follow-up care is essential.
General Management:
The following tips can help manage life with pemphigus vulgaris:
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Avoid injuring the skin to prevent the formation of new blisters.
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Take precautions to avoid infections by keeping all wounds, including cuts and scrapes, clean.
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Take care of mouth sores by:
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Eating bland, soft foods.
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Drinking beverages that do not cause pain.
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Keeping the mouth clean.
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Seeing a dentist.
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Consult a medical professional if unsure how to care for the blisters and sores.
What Is the Outlook or Prognosis of Pemphigus Vulgaris?
Pemphigus vulgaris is a lifelong condition. The disease can be life-threatening without treatment. Severe secondary infection is the most frequent cause of death. The side effects of the treatment may be severe or disabling. Improvement is usually seen after a few days of starting the corticosteroid treatment.
When to See a Medical Professional?
See a medical professional if someone has already been treated for pemphigus vulgaris and may develop any of the following symptoms:
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Chills.
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Fever.
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Feeling ill.
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Joint aches.
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Muscle aches.
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New blisters or ulcers.
Conclusion
Pemphigus vulgaris is an autoimmune disease that causes blisters on the skin and mucous membranes throughout the body. Most people with pemphigus vulgaris feel better after receiving treatment and can live full and active lives. However, without treatment, the condition can be life-threatening. Call a healthcare provider if painful, soft blisters develop on the skin or mucous membranes. Treatment helps prevent the blisters from spreading and getting worse.
