Paraneoplastic Pemphigus (PNP)

Paraneoplastic pemphigus (PNP) is a life-threatening autoimmune blistering skin disease. It is characterized with the uniform distribution of painful sores on the lips, mouth, and the esophagus as well as in the genital region and sometimes the lungs. 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. Individuals with certain types of cancer, such as lymphoma, are often diagnosed with this chronic condition.

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 the disease 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.

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. Although, the skin disease can be completely cured by the complete surgical removal of the tumor, the disorder generally causes an irreparable damage to the lungs.

Mild forms of the disease are treated with 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 forms may require hospitalization to stabilize the condition.

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