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Paraneoplastic pemphigus is an autoimmune disease that causes blisters in the oral cavity, esophagus, genital region, and sometimes the lungs. It is often diagnosed in individuals with certain types of cancer. There is no cure, and treatment is focused on managing symptoms. The immune response involves the production of antibodies that attack skin cells, resulting in blisters. Timely treatment is essential to manage symptoms and prevent complications such as sepsis and impaired breathing. Mild cases may be treated with corticosteroids, while severe cases may require hospitalization and therapeutic plasmapheresis.
Paraneoplastic pemphigus (PNP) is an autoimmune disease that negatively affects the skin and mucous membranes. Symptoms typically include blisters in the oral cavity and esophagus as well as the genital region and sometimes the lungs. Fever, chills, and other flu-like symptoms may also occur. Individuals with certain types of cancer, such as lymphoma, are often diagnosed with this chronic condition. There is no cure for paraneoplastic pemphigus, so treatment is usually centered around symptom management and is entirely dependent on the severity of the presenting symptoms.
There is no known definitive cause for the development of paraneoplastic pemphigus. In the presence of an autoimmune condition, an individual’s immune system is unable to distinguish between foreign and known substances, which triggers the development of unnecessary antibodies. The immune response of a person with paraneoplastic pemphigus involves the production of antibodies designed to scavenge proteins known as desmogleins, which play a vital role in stabilizing skin cells. When the antibodies attack, the skin cells are separated from each other, resulting in blisters.
A diagnosis of pemphigus usually results from a process of elimination. Due to the ubiquity of blisters as a symptom, signs of pemphigus can be mistaken for those associated with a different condition. Considering that pemphigus-induced blistering development is usually limited to the oral cavity and genital region, a variety of diagnostic tests can be administered to confirm a diagnosis. Symptomatic individuals will usually have a blood test and a skin biopsy to check for pemphigus-associated antibodies known as anti-desmogleins.
Individuals with paraneoplastic pemphigus will generally develop blisters inside and outside the oral cavity and esophagus. Blisters can cause extreme discomfort, making it difficult to eat, drink, and swallow. It’s also not uncommon for some individuals to develop blisters in their lungs that can impair their ability to breathe. Additional signs of pemphigus may include fever and flu-like symptoms, such as widespread pain and chills.
If left untreated, the symptoms of paraneoplastic pemphigus can progress, leading to the spread of blisters to other regions of the skin, including the face and chest. Blisters that rupture and become infected can contribute to the development of a blood infection, known as sepsis, if appropriate treatment is delayed or absent. Considering that individuals with a malignant condition often possess compromised immunity, they may be more susceptible to infections. Other complications of paraneoplastic pemphigus can include severely impaired breathing and premature death.
Timely and appropriate treatment is essential to manage symptoms and slow the progression of the disease. Mild presentations generally require administration of a corticosteroid drug to relieve inflammation. In some cases, antibiotic and antiviral drugs may be used to clear the infection and prevent reinfection. Severe presentations of paraneoplastic pemphigus often require a more complex treatment approach.
Those with severe or widespread infection may require hospitalization to stabilize their condition. When the prominence and severity of blisters within the oral cavity prevent eating or drinking, intravenous fluids and nutrients, including electrolytes and potassium, are given to help maintain nutritional balance and prevent dehydration. In some cases, a process known as therapeutic plasmapheresis may be performed to purify the individual’s plasma of unnecessary antibodies that contribute to continued worsening symptoms and accelerated disease progression.
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