What Causes Pemphigus and How Is It Treated?

Understanding Pemphigus: Causes, Symptoms & Healing Options

Pemphigus is an autoimmune disorder in which the immune system attacks skin cells and mucous membranes. Symptoms of pemphigus arise as blisters or sores on the skin. Pemphigus is caused by autoantibodies that mistakenly attack proteins called desmogleins, which are essential for holding skin cells together. This leads to blister formation. While the trigger for this autoimmune response is often unknown, it can be induced by certain medications, such as some blood pressure drugs and penicillamine. 

Who Gets Mostly Affected by Pemphigus?

Pemphigus affects a certain section of people, such as:

• Individuals aged 40 years and above
• Of Jewish ancestry
• Of Middle Eastern, Indian, and Southeastern European descent
• Individuals with autoimmune disease, especially myasthenia gravis
   

Is Pemphigus Contagious?

Pemphigus is not contagious. This disease does not spread from one individual to another through direct contact. It is an autoimmune disease, where the immune system of the body mistakenly attacks the healthy tissues.

What Foods Trigger Pemphigus?

Research suggests that there are no specific foods triggering pemphigus. However, stress and consuming certain acidic, spicy, and citrus foods make pemphigus even worse. This can also be true under treatment. Make sure to focus on what makes you feel better and prevents symptoms from worsening.

How is pemphigus diagnosed?

The diagnosis of pemphigus is complicated, as several conditions cause blisters all over your body. To find out the root cause, your healthcare consultant will prescribe you several tests, including:

• A Skin Examination: The doctor may perform a test for Nikolsky's sign. This involves applying gentle lateral pressure with a finger on normal-appearing skin near a blister. If the top layer of skin separates easily from the layer below (a positive sign), it is a strong indicator for pemphigus.
• Medical History: Your doctor might ask for your medication history, as there are some specific drugs that cause pemphigus.
• Skin Biopsy: To conduct this, the doctor takes out a single piece of tissue from any one of your blisters and examines it under a microscope.
• Endoscopy: If there are blisters in your mouth, the doctors consider inserting a thin, flexible tube known as an endoscope down your throat.
• Blood Tests: Your healthcare consultant will examine your blood for specific antibodies commonly referred to as desmogleins. Antibodies are proteins your body prepares to detect bad germs and then destroy them before they cause harm to your body.
   

If there is pemphigus, there will be the presence of more of these antibodies in your blood than usual. With gradual improvement in symptoms, these antibodies in your blood reduce.

How Can Pemphigus Be Treated?

To treat pemphigus, doctors prescribe medications to reduce symptoms and make you feel comfortable. The medicine prescribed depends on the kind of pemphigus that exists, along with the severity of the pemphigus. Treatment includes:

• Immunosuppressants: This drug protects your immune system and prevents attacks on healthy tissues.
• Corticosteroids: This is the primary treatment of pemphigus and is effective for relieving symptoms, usually within a couple of weeks. High-dose oral corticosteroids (e.g., prednisone) are often used initially to control the disease. Topical corticosteroids are important for managing localized blisters and oral lesions. In severe cases, corticosteroids may be administered intravenously.
• Antibiotics, Antivirals, and Antifungal Medications: These medications help fight and prevent infections. Keeping pemphigus untreated can cause life-threatening conditions. At times, doctors might also need to admit you to a hospital if you get better.
• Biological Therapies: If other medications fail, the doctor might provide you with a medication, namely rituximab (Rituxan). You will also need an injection to lower the number of antibodies causing harm to your body.
• Wound Care: Wound dressings and topical antibiotics are essential for healing the blisters.
• Plasmapheresis: If pemphigus does not respond well to other treatments, doctors take the help of a special machine to extract plasma from your blood. Further, they are replaced with plasma from a blood donor.
   

Complications of Pemphigus

Here are the possible complications of pemphigus. Let’s explore them in detail:

• Changes in your skin colour and scarring occur after the healing of the skin in the affected area. When the skin becomes darker, people commonly refer to it as post-inflammatory hyperpigmentation. Whereas, if the skin loses its original colour, people refer to it as post-inflammatory hypopigmentation.
• Individuals with black or brown skin are prone to a high risk of changes in their long-term skin colour.
• Infections spreading rapidly to your blood are known as sepsis. This kind of infection can be particularly life-threatening.
• Side effects from medicines prescribed by your doctor to treat pemphigus. Examples include infection and high blood pressure.
• Death, though rare, can happen if pemphigus is left untreated for a prolonged period.
   

Treating pemphigus takes adequate time before you can see visible results. With continuous treatment, new blisters stop forming within several weeks, and your skin also starts to heal. However, your blisters and sores might take months to heal completely. An effective treatment helps in easily managing these symptoms. If you notice any unusual blisters, consult a doctor right away.