While what causes pemphigus vulgaris is unknown, the autoimmune disease can be triggered by environmental factors, medications, emotional stress, and infections.
The cause of pemphigus vulgaris is not fully understood. It is an autoimmune disease, which is a disorder where the immune system attacks its own body cells and is mostly found in people aged between 50 to 60 years old.
Experts believe that the condition is inherited from the parents, and it is triggered by some environmental factors, such as a chemical or drug.
Other possible triggers of pemphigus vulgaris include:
- Angiotensin-converting-enzyme (ACE) inhibitors (blood pressure medications)
- Penicillamine (medication used in the treatment of rheumatoid arthritis and Wilson’s disease)
- Cephalosporin (antibiotic)
- Nonsteroidal anti-inflammatory drugs (NSAIDs)
- Rifampin (an antibiotic used most commonly for treating tuberculosis)
- Emotional stress
- Exposure to sunlight (particularly the ultraviolet rays)
What are the signs and symptoms of pemphigus vulgaris?
The signs and symptoms of pemphigus vulgaris vary between people, but may include:
- Fluid-filled blisters on the skin and mucous membrane
- Blisters in the mouth
- Bursting of blisters (which may be painful or itchy)
- Scarring of the skin
What are the diagnostic tests for pemphigus vulgaris?
For diagnosing pemphigus vulgaris, the patient will need to see a skin specialist (dermatologist) who will take their medical history, ask them about their symptoms and perform a physical examination.
The dermatologist may also order tests that include:
- Blood test: A test to detect antibodies in the body helps check if the condition is autoimmune.
- Clinical examination: The dermatologist will check for signs that identify blistering of the skin.
- Biopsy: The doctors remove a small part of the tissue of the affected skin and send it to the lab to analyze it under a microscope.
- Endoscopy: This involves inserting a thin, flexible tube fitted with a lighted camera that helps the doctor look inside of the throat and esophagus for blisters.
What is the treatment of pemphigus vulgaris?
There is no specific treatment available for pemphigus vulgaris because it is an autoimmune disease, which means avoiding trigger factors is vital.
Treatment aims to decrease the severity of the disease or pain and avoid further complications. Treatment options may include:
- Medicines: Medicines are used as the first line of treatment.
- Steroids: Corticosteroids help reduce inflammation and inhibit disease-causing cytokine (chemicals that trigger inflammation in the body) production.
- Other immunosuppressants: These are used with steroids to reduce chances of relapse.
- Adjuvant therapy: This therapy is used to reduce the risk of relapse and lower the dosage of steroids required. Drugs used for adjuvant therapy are rituximab and azathioprine.
- Intravenous immunoglobulin therapy: Immunoglobulin is administered in the vein through the needle to destroy disease-causing antibodies.
- Medical procedure: Medical procedures, such as plasmapheresis, could be used in some cases. This procedure involves the removal of disease-causing antibodies from the body by replacing the plasma of the blood with some other solution.
- Other medications: To avoid secondary infection, the doctor may initiate antibiotics, antifungal or antiviral treatment.
- Wound care: This includes proper wound care, such as regular wound dressings, which help heal blisters.
- Hospitalization: In severe cases (such as severe blistering in the mouth and throat), intravenous fluids and electrolytes are administered.