What is pemphigus vulgaris?
Pemphigus Vulgaris is a rare and serious autoimmune disorder in which blisters of various sizes form on the skin and the lining of the mouth and other mucous membranes. It affects the mouth, nose, throat, eyes, and genitals. Pemphigus Vulgaris is the most usual type of pemphigus.
Who gets Pemphigus Vulgaris?
Pemphigus Vulgaris affects people of all races, ages, and genders. It usually appears between the ages of 30 and 60 and is more common in Jews and Indians than other races, probably due to genetic factors.
Drug-induced pemphigus has also been recognized and is most usually generated by penicillamine, angiotensin-converting enzyme inhibitors, angiotensin receptor blockers, and cephalosporins.
Pemphigus is sometimes caused by cancer (paraneoplastic pemphigus), infection, or injury.
Causes of Pemphigus Vulgaris
Pemphigus Vulgaris is not fully understood. Experts believe that a person with a genetic predisposition to this condition is induced when they come into contact with an environmental trigger, such as a chemical or drug. In some cases, Pemphigus Vulgaris goes away after the trigger is removed.
This condition causes the immune system to fight against the body’s cells and attack germs. With Pemphigus Vulgaris, the immune system looks for proteins that bind to skin cells. This causes fluid to form between skin cells, causing blisters. Experts hope that this condition will combat healthy proteins.
Symptoms of Pemphigus Vulgaris
About 50% of people with this disease first develop painful blisters and sores in the mouth. This is followed by blisters on the skin. Dermatitis may not occur.
Dermatitis can be described as:
- Easily peeled or peeled off
They may be:
- In the mouth and under the throat
- On the skin, trunk, or other areas of the skin
Diagnosis of Pemphigus Vulgaris
The diagnosis may not be clear at first. There are other causes of mouth ulcers and skin blisters. So if your doctor suspects you have this disease, they will refer you to a dermatologist. Tests are usually done to confirm the diagnosis. Besides these:
- A small sample (biopsy) of the skin may be taken. It is viewed under a microscope and examined to confirm that the blisters are caused by Pemphigus Vulgaris.
- Blood test for antibodies. It uses methods called immunofluorescence or Enzyme-linked immunosorbent assay (ELISA) to measure the level of antibodies in the bloodstream that causes Pemphigus Vulgaris. In addition to helping to diagnose the disease, the level of autoantibodies in the blood gives a good indication of how active the disease is. Measured from time to time to monitor how well the treatment is working. When the level of antibodies decreases, the disease becomes less active.
Treatment for Pemphigus Vulgaris
People with moderate to severe illnesses are hospitalized. In the hospital, raw skin surfaces require extraordinary care, similar to that given to people with severe burns. Antibiotics may be needed to treat infections in broken blisters. The dressing protects the raw soluble areas.
- Corticosteroids (oral or intravenous)
- Antibiotics and protective dressings for ruptured blisters
- Often immunosuppressants or immune globulin to decrease corticosteroid use
High doses of corticosteroids are essential for treatment. They can be taken orally (orally) or intravenously (intravenously) if the person is hospitalized. If the disease is controlled, the dose of corticosteroids is gradually reduced (damaged). It is sometimes given with corticosteroids called rituximab, which suppress the immune system (immunosuppressant).
Immunosuppressive medications such as azathioprine, cyclophosphamide, methotrexate, mycophenolate mofetil, cyclosporine, or rituximab (if not already given) may also be given if the person does not respond to treatment or if the disease worsens when the dose is damaged. Immunosuppressive medications may also be given to reduce the need for corticosteroids in people who need corticosteroids for a long time or in high doses.
Intravenous immune globulin is another treatment used for acute pemphigus Vulgaris. Some respond well to discontinuation of drug treatment, while others may need to continue taking higher doses of the drug. People with severe Pemphigus Vulgaris also undergo a plasma transplant, in which antibodies are filtered from the blood.
Potential pemphigus complications:
- Infection of your skin
- An infection that spreads to the bloodstream (sepsis)
- Malnutrition, because painful sores in the mouth make it difficult to eat
- Side effects like high blood pressure and infections
- Death, if certain types of pemphigus are not treated
Without systemic corticosteroid treatment, It is usually fatal, usually within 5 years of disease onset. Systemic corticosteroids and immunosuppressive therapies have a better prognosis, but complications from treatment can lead to death.