Pemphigus vulgaris is a rare autoimmune disorder that causes painful blisters or sores in the mouth, nose, throat, genitals, or esophagus, often accompanied by a burning or stinging sensation.
Though more common in adults and older individuals, pemphigus vulgaris can occur at any age. It is the most common type of pemphigus, characterized primarily by where the blisters form on the body.
Treatment of pemphigus vulgaris is managed by a dermatologist with the use of medications such as corticosteroids or immunosuppressants to control symptoms, reduce the formation of blisters and sores, and prevent complications like malnutrition or generalized infections.
Common symptoms
The main symptoms of pemphigus vulgaris include:
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Blisters in the mouth or throat;
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Intense pain, burning, or stinging in areas with blisters;
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Painful sores that form when blisters rupture;
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Blisters that appear with minimal pressure or trauma;
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Skin discoloration as the sores heal.
Typically, pemphigus vulgaris symptoms begin in the mouth or throat and are sometimes mistaken for canker sores. However, these blisters can also develop on the skin and mucous membranes, including the nose, eyes, genitals, anus, or esophagus.
The blisters are extremely painful and may form crusts, but they don't itch. When they occur in the mouth or throat, they can make it difficult to eat, which can lead to malnutrition.
There is also a variant of pemphigus vulgaris known as vegetative pemphigus, which causes thick, wart-like blisters or sores in areas of the body with folds, such as the groin or armpits.
Confirming a diagnosis
A diagnosis of pemphigus vulgaris is made by a primary care provider or dermatologist based on an evaluation of the blisters or sores, medical history, medication use, and blood tests.
To confirm the diagnosis, a doctor may also request a biopsy of the blister or sore. This will help rule out other conditions with similar symptoms, such as bullous pemphigoid, lupus, or Hailey-Hailey disease.
In some cases, a doctor may recommend an endoscopy to check for blisters or sores in the throat or esophagus caused by pemphigus vulgaris.
Possible causes
The exact cause of pemphigus vulgaris is not fully understood, but it is known to result from the immune system producing antibodies that attack and destroy skin and mucosal cells as if they were foreign invaders.
Several factors may increase the risk of developing pemphigus vulgaris, including:
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Age, with a higher incidence between 50 and 60 years old;
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Genetic factors;
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Medications for high blood pressure, such as enalapril;
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Antibiotics, like penicillins;
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Radiotherapy;
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Viral infections.
In addition, stress or diet may also contribute to immune system dysregulation, triggering flare-ups of pemphigus vulgaris.
Is it contagious?
Pemphigus vulgaris is not contagious because it is not caused by a virus or bacteria, but rather by immune system malfunction.
However, the blisters or sores may become infected. If this happens, the bacteria can be transmitted to others who come into direct contact with the infected area, potentially causing skin irritation.
Treatment options
Treatment for pemphigus vulgaris should be guided by a dermatologist to control symptoms, reduce flare-ups, and prevent complications such as malnutrition or widespread infections.
The main treatments for pemphigus vulgaris are:
1. Medications
A dermatologist may prescribe medications to help manage pemphigus vulgaris, including:
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Corticosteroids (e.g., prednisone, prednisolone) to reduce inflammation and suppress immune system activity;
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Immunosuppressants (e.g., azathioprine, mycophenolate mofetil, methotrexate, cyclophosphamide) to prevent the immune system from attacking skin or mucosal cells;
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Monoclonal antibodies (e.g., rituximab, ofatumumab) to regulate immunity and decrease immune system damage to the body.
A doctor may also prescribe pain relievers, antibiotics, antivirals, antifungals, or anesthetic lozenges to manage discomfort in the mouth.
If a medication is identified as the cause of the blisters, the doctor may recommend stopping the medication, which could be enough to resolve the pemphigus vulgaris.
2. Parenteral nutrition
In cases where malnutrition results from difficulty eating due to mouth or throat blisters and sores, hospitalization and parenteral nutrition (administered through an IV) may be necessary until recovery is achieved.
3. Plasmapheresis
In more severe cases, the doctor may recommend plasmapheresis, a blood filtration procedure designed to remove the antibodies attacking the skin or mucous membranes.
Care during treatment
Proper care during treatment can help speed recovery and alleviate symptoms. Important care guidelines include:
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Following the doctor’s instructions for wound care;
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Using mild soap to gently cleanse the body;
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Avoiding sun exposure, as ultraviolet radiation can cause new blisters;
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Avoiding spicy or acidic foods that can irritate the blisters in the mouth;
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Refraining from physical activities that might harm the skin, such as contact sports.
If pemphigus vulgaris causes mouth blisters that make brushing or flossing difficult, special care may be needed to prevent gum disease or cavities. It’s crucial to consult with a dentist for guidance on maintaining oral hygiene based on the severity of symptoms.
Potential complications
The most significant complications of pemphigus vulgaris include infections of the blisters or sores, which may spread to the bloodstream, causing sepsis.
Other complications include dehydration, malnutrition, or side effects from corticosteroid treatment, such as swelling, cataracts, diabetes, or osteoporosis.