Pemphigus or pemphigus vulgaris is a serious skin disorder characterized by blisters on the skin, inside of the mouth, nose, throat, and genitals. The blisters break easily and leave scars that are prone to infection.
Pemphigus is a rare disease, but can cause death if left untreated. Pemphigus is more common in people aged 50-60 years, although it can occur at any age. Keep in mind this skin disease is not contagious.
Causes of Pemphigus
In people with pemphigus, the immune system produces antibodies that turn against healthy cells in the skin and other layers of the body. This condition is called autoimmune. Normally, antibodies function to attack harmful organisms, such as viruses or bacteria.
It is not known exactly what causes it, but it is suspected that pemphigus is triggered by the use of drugs, such as:
- Rifampicin.
- Antibiotics, eg cephalosporins.
- Non-steroidal anti-inflammatory drugs (NSAIDs).
- High blood group ACEinhibitor, for example captopril.
Other factors that are thought to trigger pemphigus are:
- Stress.
- UV exposure.
- Burns.
- Infection.
- Age.
- Suffering from other autoimmune diseases, especially myasthenia gravis andthymoma.
Symptoms of Pemphigus
Symptoms of pemphigus are blisters on the skin that are prone to rupture, leaving crusty sores. The blisters can be painful, but not itchy. It could also be the other way around, itchy, but not painful. Blisters may appear in the following areas:
- Shoulder.
- Chest.
- Back.
- The inside of the eyes, nose, mouth, throat, lungs, and genitals.
The blisters may appear small, then enlarge gradually. Over time, the blisters will multiply and cover the face, scalp, and entire body.
The presence of blisters in the mouth can cause a burning sensation when eating, drinking, or brushing teeth. The patient's voice can also become hoarse due to blisters in the throat.
Pemphigus Diagnosis
Many conditions can cause blisters on the skin. Therefore, the doctor will carry out examinations in order to properly diagnose pemphigus, including:
- blood test. Blood tests are done to detect antibodies that cause pemphigus.
- Biopsy. The dermatologist will take a sample of skin tissue from the blister for examination under a microscope.
- Endoscopy.In patients with pemphigus, the doctor will perform an observation or endoscope to see the wound in the throat.
Pemphigus Treatment
Pemphigus treatment aims to relieve symptoms and prevent complications. To be more effective, treatment should be started as early as possible. Treatment can be done with drugs or special measures. In mild pemphigus caused by drug use, the blisters may heal on their own after the drug is discontinued.
To treat pemphigus, doctors can give several types of drugs. The drug can be given alone or in combination with other drugs, depending on the severity. Some types of drugs that are usually used in cases of pemphigus are:
- Cortosteroids.Your doctor will give you a corticosteroid cream to treat mild pemphigus. Meanwhile, for severe pemphigus, corticosteroid tablets will be given, such as: methylprednisolone. Initially, the doctor will give high doses of corticosteroids to prevent new blisters from forming. After confirming that new blisters are no longer forming, the dose will be gradually reduced to reduce the risk of side effects.
- Drugs that suppress the immune system (immunosuppressive drugs).Mycophenolate mofetil, azathioprine, and cyclophosphamide can help prevent the immune system from attacking healthy cells.
- Rituximab.Rituximab given by injection when other drugs are ineffective or cause serious side effects to the patient.
- Injectanimmunoglobulins. Immunoglobulin injection aims to reduce or prevent the severity of infection in patients with impaired immune systems. Immunoglobulin It can also neutralize antibodies that turn against healthy cells.
- Antiviral, antibiotic, and antifungal drugs. These drugs are given to prevent and treat infection caused by the blisters.
In severe pemphigus, the doctor will remove fluid in the patient's blood (blood plasma) and replace it with special fluids or healthy blood plasma from a donor. This action is called plasmapheresis. Plasmapheresis aims to remove antibodies that cause pemphigus from the patient's blood.
If the blisters on the skin have spread widely, the patient should be hospitalized. The treatment steps given are the same as in cases of serious burns, including:
- Provide nutritional intake through an IV, if there are severe blisters in the mouth.
- Provide replacement fluids through an IV, to replace lost body fluids.
- Clean the wound and cover it with a sterile bandage, to prevent infection.
To help the healing process, follow the doctor's instructions in treating the wound. Proper wound care can prevent infection and scar tissue formation. When cleaning the skin, do it slowly, and use a mild soap and moisturizer afterwards.
Avoid eating spicy foods that can aggravate blisters in the mouth. If the blisters in your mouth make it difficult for you to brush your teeth, ask your dentist about ways to keep your mouth clean and healthy.
In addition to the various ways above, also limit sun exposure to the skin, because ultraviolet rays can trigger the appearance of new blisters.
Pemphigus Complications
Open blisters, susceptible to bacterial infection. Infection can be characterized by pain and burning on the skin, discharge of greenish or yellowish pus on the blisters, and widespread redness around the blisters. The bacteria can spread into the bloodstream and cause a dangerous condition called sepsis.
Other complications can arise from long-term use of corticosteroids and immunosuppressive drugs, namely:
- Growth disorders in children.
- Hormone disorders.
- Osteoporosis.
- Cancer occurs, such as lymphoma.