Pemphigus vulgaris

Pemphigus vulgaris

Pemphigus Vulgaris is a skin disease that is classified as an autoimmune disease. It is caused by the body's immune system's mistake and turns to destroy the "biological glue" that holds skin cells together, causing the cells to separate and form blisters and abrasions.

Disease mechanism (simple to understand)

In normal conditions, skin cells are held together by a protein called Desmoglein, which acts like a "zipper" between cells. However, in people with Pemphigus Vulgaris, the body creates antibodies (IgG) that destroy this protein, causing the outer layer of skin to separate, resulting in blisters and sores. In particular, in vulgaris, the antibodies destroy Desmoglein-3 (found in the mouth and mucous membranes) and some people may also have antibodies to Desmoglein-1 (found in the skin).

Symptoms and manifestations of the disease
1. Initial symptoms Patients often start to have sores in the mouth first, without blisters. The sores are often painful and do not heal easily like canker sores.
2. Skin blisters, often on the chest, back, scalp, or face. The blisters are thin and easily broken. When the blisters break, they become abrasions (erosion) and can easily become infected.
3. Characteristics When the skin is lightly rubbed, it may peel off (positive Nikolsky sign). Pressing on the blisters causes the fluid in the blisters to spread to the sides (positive Asboe-Hansen sign).

Who is at risk?
•Age 40–60 years, common in people of Jewish, Mediterranean, and Middle Eastern descent.
•Not contagious and usually not directly passed on genetically.
•Triggers may include:
•Using certain medications (eg, Penicillamine, ACE inhibitors)
•Stress, strong sunlight, or viral infections

Diagnosis
1. Physical examination The doctor will look for specific lesions. And Nikolsky sign test
2.Skin biopsy will show the separation of the upper skin cells (suprabasal acantholysis)
3.Immunological examination Direct Immunofluorescence (DIF): Examine directly from the skin, find the accumulation of IgG and C3 in a "net" pattern Indirect Immunofluorescence (IIF) or ELISA: Test the blood for antibodies to Desmoglein

Treatment guidelines
1.First aid Corticosteroids (eg Prednisolone): reduce inflammation and suppress the immune system
2.Other immunosuppressive drugs (Steroid-sparing agents) to reduce the side effects of steroids, such as: Azathioprine, Mycophenolate mofetil, Methotrexate, Cyclophosphamide
3.Specialized treatment Rituximab: A new drug that is very effective. It is a monoclonal antibody that eliminates B cells, which are the source of abnormal antibodies. IVIG and Plasmapheresis: Used in severe cases or those who do not respond well to other drugs

Long-term care
•Monitor blood test results and check your health every 1-3 months during treatment.
•Keep the wound clean, avoid strong sunlight, and reduce stress.
•Patients may need to take long-term immunosuppressive drugs for a year or several years.

Prognosis
•If treated early and continuously, most patients can control the disease.
•The mortality rate is significantly reduced after Rituximab.
•However, the disease may recur, so it is important to follow up with your doctor regularly.



Summary
Pemphigus vulgaris is a disease that does not seem serious at first. However, if left untreated, it may develop secondary infections and be life-threatening. Early diagnosis and care by a specialist will greatly improve the quality of life of patients.