Paraneoplastic pemphigus is typically a side effect of a cancerous tumor and can cause mouth pain and oral sores. It can be aggressive and should be diagnosed and treated as soon as possible.

Pemphigus is an autoimmune condition that causes blisters on the skin as well as inside areas such as the nose, throat, mouth, eyes, and genitals.

There are four types of pemphigus. The rarest type is called paraneoplastic pemphigus. It’s often caused by a cancerous tumor and can result in blistering that affects the lungs and other organs.

Keep reading to learn more about this rare autoimmune disorder and how to treat it.

Paraneoplastic pemphigus is almost always caused by a cancerous tumor. Cancer types commonly linked to paraneoplastic pemphigus include non-Hodgkin’s lymphoma and chronic lymphocytic leukemia.

Sometimes tumors formed by cancers such as sarcomas, squamous cell carcinomas, and carcinomas of the stomach, lung, or colon can cause paraneoplastic pemphigus. A rare condition called Castleman disease is also linked to paraneoplastic pemphigus.

People diagnosed with paraneoplastic pemphigus are typically between the ages of 45 and 70. When children are diagnosed, they often have underlying Castleman disease. Some research has linked genetic factors mostly likely to be found in people of Chinese and European ethnic backgrounds to a higher risk of paraneoplastic pemphigus.

How common is paraneoplastic pemphigus?

Paraneoplastic pemphigus is very rare. All types of pemphigus are uncommon in the United States. It’s estimated that less than 50,000 people nationwide have pemphigus.

Paraneoplastic pemphigus is the rarest type of pemphigus.

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Skin blisters and lesions are the primary symptoms of paraneoplastic pemphigus. These blisters can look similar to the blisters caused by other skin conditions or autoimmune conditions.

Additional symptoms can include:

Paraneoplastic pemphigus can also lead to a complication called bronchiolitis obliterans. This serious lung condition affects the small airways in the lungs. The condition is irreversible and progressive. It can be fatal without treatment.

Paraneoplastic pemphigus is rare. Treatment depends on the individual and their symptoms. However, treating the underlying cancer is almost always the first step. Often, removing a tumor caused by the cancer can resolve paraneoplastic pemphigus.

Additional treatment might be needed to minimize symptoms and to reverse any damage caused by paraneoplastic pemphigus. This can include:

  • IV-immunoglobulin: Receiving a high dose of immunoglobulin treatment through an IV can help stop antibodies from causing lung damage. You might receive this before and after cancer or tumor-removal surgery.
  • Corticosteroids: Corticosteroids can reduce inflammation and suppress the immune system’s response.
  • Wound care: Treatment to address blisters and prevent infection is often a priority. This can include the use of sterile dressings and topical pain creams.
  • Immunosuppressants: Immunosuppressant medications can sometimes help slow the progression of paraneoplastic pemphigus.
  • Antibody medications: Monoclonal antibody medications, such as rituximab (Rituxan), are used to treat certain autoimmune conditions. They’re sometimes used in the treatment of paraneoplastic pemphigus.
  • Pain-reliving mouthwashes: Pain-relieving mouthwashes can help manage mouth sores.
  • Protein supplements: It can be hard to get the protein intake your body needs for wound healing while also managing cancer. Protein supplements can help.

Paraneoplastic pemphigus is linked to low survival rates. In some studies, the mortality rate is as high as 90%. However, most people with paraneoplastic pemphigus already have a cancer diagnosis, and that affects survival rates.

Additionally, paraneoplastic pemphigus is rare, making statistics harder to draw conclusions from.

It’s always a good idea to discuss your specific diagnosis with your doctor. They can help you understand the factors that affect you, your course of treatment, and your outlook.

You’re not alone

There are resources you can turn to when you’ve been diagnosed with a rare condition such as paraneoplastic pemphigus. They can help you find the support you need. For instance, you can look into:

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Paraneoplastic pemphigus is the rarest form of the autoimmune condition pemphigus. It’s almost always caused by a cancerous tumor.

Paraneoplastic pemphigus causes blisters and lesions that can spread to the eyes and lungs, causing serious complications. Treatment includes removal of a tumor, wound care, pain management, and medications to control inflammation.

If you notice any symptoms of paraneoplastic pemphigus, it’s best to meet with a healthcare professional for diagnosis. Early diagnosis and treatment are key to positive outcomes.