What is Paraneoplastic Pemphigus?

Paraneoplastic pemphigus (PNP) is an autoimmune disease that adversely affects the skin and mucous membranes. Symptoms typically include blisters in the oral cavity and esophagus as well as in the genital region and sometimes the lungs. Fever, chills and other flu-like symptoms may present as well. Individuals with certain types of cancer, such as lymphoma, are often diagnosed with this chronic condition. There is no cure for paraneoplastic pemphigus, therefore, treatment is generally centered on symptom management and is entirely dependent on the severity of symptom presentation.

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 differentiate between foreign and known substances, which triggers the development of unnecessary antibodies. The immune response of someone with paraneoplastic pemphigus involves the production of antibodies that are designed to eliminate proteins known as desmogleins, which play a pivotal role in the stabilization of skin cells. As the antibodies attack, skin cells are separated from one another, resulting in blister formation.

A diagnosis of pemphigus generally results from a process of elimination. Due to the ubiquitousness of blisters as a symptom, pemphigus signs may be mistaken for those associated with a different condition. Considering the development of pemphigus-induced blisters is usually confined to the oral cavity and genital region, a series of diagnostic tests may be administered to confirm a diagnosis. Symptomatic individuals will generally undergo a blood test and skin biopsy to verify the presence of pemphigus-associated antibodies known as anti-desmogleins.

Individuals with paraneoplastic pemphigus will generally develop blisters within and without the oral cavity and esophagus. The presence of blisters can cause extreme discomfort, making eating, drinking and swallowing difficult. It also is not uncommon for some individuals to develop blisters in their lungs that can compromise their ability to breathe. Additional signs of pemphigus can include fever and flu-like symptoms, such as widespread achiness and chills.

If left untreated, paraneoplastic pemphigus symptoms can progress, leading to the spread of blisters to other dermal regions, 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 infection. Other complications of paraneoplastic pemphigus may include a severe impairment of respiration and premature death.

Timely and appropriate treatment is essential to managing one’s symptoms and slowing disease progression. Mild presentations generally necessitate the administration of a corticosteroid medication to ease inflammation. In some cases, antibiotic and antiviral medications may be utilized to eliminate infection and prevent reinfection. Severe presentations of paraneoplastic pemphigus often necessitate a more intricate 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 one from eating or drinking, intravenous fluids and nutrients, including electrolytes and potassium, are administered to facilitate nutritional balance and prevent dehydration. In some cases, a process known as therapeutic plasmapheresis may be performed to cleanse the individual’s plasma of unnecessary antibodies that are contributing to a continued worsening of symptoms and accelerated disease progression.