Multiple crusted erosions intermixed with erythematous patches over the hand. Dr. Rametrika Saha
Medicine

Dermatologist Explains: Is Pemphigus Vulgaris a Cancer or an Autoimmune Disease?

Pemphigus vulgaris (PV) is an autoimmune disorder of the skin. It involves blistering and sores (erosions) of the skin and mucous membranes

Dr. Rametrika Saha

No, Pemphigus Vulgaris is not cancer, but it is the most common and severe form of pemphigus, typically affecting individuals between the ages of 40 and 60 years. Because it involves long-term treatment and may cause widespread lesions, many patients fear it could be cancerous.

To address this concern, MedBound Times connected with Dr. Siddharth Munot, MD Dermatologist (DVL) & Co-Founder of Clinderma. He explained that while pemphigus vulgaris can mimic the severity and long-term management challenges of cancers, it is not a malignancy. Dr. Munot emphasized the importance of early diagnosis, appropriate treatment, and patient education in dispelling myths, reducing fear, and improving quality of life for those living with the condition.

Dr. Siddharth Munot, MD Dermatologist (DVL) & Co-Founder of Clinderma

What is Pemphigus?

Dr. Siddharth Munot: Pemphigus is a rare set of autoimmune diseases that lead to the formation of blisters on the skin and mucous membranes throughout the body. This condition can impact areas such as the mouth, nose, throat and eyes.

In pemphigus, the body produces IgG autoantibodies that attack proteins in the cell membranes of skin cells, causing them to lose their ability to adhere to each other. These autoantibodies typically target desmogleins, which are part of a family of proteins that help cells stick together.

What causes it?

Dr. Siddharth Munot: Pemphigus occurs when the immune system produces antibodies against proteins that hold skin cells together. The primary pathogenic antibodies are directed against desmogleins 1 and 3, members of the cadherin family of calcium dependent cell–cell adhesion molecules that contribute to the structure of the desmosome. This leads to loss of adhesion between skin cells, causing blisters.

What are the early signs and symptoms of Pemphigus Vulgaris?

Dr. Siddharth Munot: Pemphigus vulgaris typically begins in the mouth. Other common places where you can experience symptoms are- nose, eyes, genitals, chest, back, arms & legs, scalp.

The symptoms include:

  • Painful mouth ulcers and erosions are often the first signs.

  • Difficulty eating food (especially spicy food), difficulty drinking water or other liquids.

  • Fragile blisters appear on the skin, which break easily to form raw erosions.

  • Gentle rubbing causes the skin to peel.

Can I scrub these lesions off?

Dr. Siddharth Munot: In pemphigus vulgaris, new blisters can sometimes appear in areas where the skin has been irritated or injured, a response called the Koebner phenomenon. So, scratching it might make it worse. This phenomenon is also seen in other diseases such as lichen planus and psoriasis.

How is pemphigus vulgaris diagnosed?

Dr. Siddharth Munot:

  • Clinical signs: fragile blisters, oral erosions, positive Nikolsky sign.

  • Skin biopsy: shows loss of adhesion (acantholysis).

  • Direct immunofluorescence: shows antibody deposits in the skin.

  • Blood test (ELISA): detects antibodies against desmogleins, useful for monitoring disease activity.

Is pemphigus vulgaris curable?

Dr. Siddharth Munot: It is not curable in the absolute sense, but it is very treatable. With modern medicines, patients can achieve remission and live a normal life.

What is the treatment?

Dr. Siddharth Munot: The treatment is mainly dependent on counselling for lifestyle modifications and medical line of management. Certain foodstuffs, such as garlic, ginger, tomatoes, and bananas, as well as beverages like tea and coffee, are to be avoided. The treatment must be strictly undertaken under the supervision of a dermatologist

  • First line: Corticosteroids (prednisone) and Rituximab (an antibody therapy).

  • Other medicines: Azathioprine, Mycophenolate, Cyclophosphamide, Methotrexate (to reduce steroid use).

  • Supportive care: wound care, pain control, infection prevention, nutrition.

The blisters have burst, leaving painful sores. About 70% of people who get pemphigus have this type.

Additional FAQs:

Is Pemphigus Vulgaris contagious?

Dr. Siddharth munot: No. It is an autoimmune condition, not an infection. It does not spread by touch, food, or close contact.

What’s the difference between Pemphigus Vulgaris and Bullous Pemphigoid?

Dr. Siddharth Munot: Both, pemphigus and bullous pemphigoid belong to the bigger category of autoimmune mechanobullous disorders. Pemphigus is marked by shallow ulcers or delicate blisters that burst easily. In contrast, pemphigoid features more robust or "tense" blisters that do not break open easily. 

Is pemphigus vulgaris hereditary?

Dr. Siddharth Munot: No. It is not passed from parents to children in a simple way. Some people may be genetically more prone, but it is not directly inherited.

If you have itchy or painful blisters on your body, it's crucial not to attempt a self-diagnosis. Instead, see your doctor, as they are the only ones qualified to accurately determine if you have pemphigus or another condition.

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