Pemphigus is a rare disease in the United States that causes blistering inside the skin and inside the body’s parts including the mouth, throat, eyes, genitals, and nose. It is an autoimmune disease that attacks the body itself by accidentally—specifically the epidermis and the mucous membrane. This autoimmune disease manifests due to a production of antibodies that reduce proteins that help bind skin cells to one another, making the skin more fragile. As the skin becomes more fragile, fluid can often build up in between layers, resulting in blisters.
There are two main types of pemphigus: pemphigus vulgaris and pemphigus foliaceus. Pemphigus vulgaris appears on the mucous membrane while the pemphigus folicaceus only affects the skin. More rare forms of the disease include paraneoplastic pemphigus, IgA pemphigus, and drug-induced pemphigus. Paraneoplastic pemphigus include severe lung disease and sores in mucous membranes and on the skin. IgA pemphigus is caused by the IgA antibody which appears like rings on the skin. Drug-induced pemphigus occurs when some drugs cause the disease.
Though there isn’t a clear reason as to why someone may acquire the disease, there are certain factors that may make someone have a greater chance of having the disease. In terms of ethnic background, those of Jewish, Indian, Southeast European, or Middle Eastern ethnicities have a higher risk of developing the disease. Brazil and Tsunia have a greater geographic disposition to the disease. Women usually get the disease more often than men and the most common age to develop the disease is between 50 and 60. The decrease in immune system efficiency can be attributed to a genetic variant of the HLA genes which have been linked to an increase in autoimmune diseases. Additionally, certain medications and cancer tumors can trigger the disease.
Treatment plans should be prescribed by a certified dermatologist. A doctor may assess a patient and prescribe corticosteroids (anti-inflammatory medicines that may be an ointment or cream), bioresponse modifiers (which target immune responses and interrupt the signal), and antibiotics, antivirals, and antifungal medications. If these medications do not work, then immunosuppressants, plasmapheresis (which dilutes antibodies in the body), and intravenous immunoglobulin therapy (which also dilute negative antibodies in the body). Contacting a dermatologist is crucial to this process.
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