Pemphigus or pemphigus vulgaris is a serious skin disorder characterized by blisters on the skin or inside of the mouth, nose, throat and genitals. These blisters break easily and leave scars that are prone to infection.

Pemphigus is a skin disease that rarely occurs, but can cause death if not treated immediately. Pemphigus can occur in all age groups, but is more common in people aged 50–60 years. Even so, pemphigus is not a contagious skin disease.

Causes of Pemphigus

Pemphigus occurs when the body's immune system produces antibodies that turn on healthy cells in the skin and other body layers. In other words, this condition is included in an autoimmune disease .

It's not known exactly what causes pemphigus, but it's thought to be triggered by taking the following medications:

  • Rifampin or rifampin
  • Antibiotics, such as the cephalosporin class
  • Non-steroidal anti-inflammatory drugs ( NSAIDs )
  • ACE inhibitors for high blood pressure , such as captopril

In addition, there are several factors that are thought to increase the risk of developing pemphigus, namely:

  • stress
  • Excessive exposure to ultraviolet light
  • Age 50–60 years
  • Sores on the skin from scratching, burning, or insect bites
  • Infection
  • Other autoimmune diseases, such as myasthenia gravis and thymoma

Symptoms of Pemphigus

Sores on the oral or genital mucosa are early symptoms of pemphigus. After a few weeks or months, these sores can appear on other parts of the skin, for example on the shoulder, chest or back. These sores are usually painful, but not accompanied by itching.

Pemphigus which attacks the throat can make it difficult for sufferers to swallow. Meanwhile, if the wound appears in the voice box or larynx, the patient can experience hoarseness.

When to see a doctor

Check with your doctor if you have sores in your mouth or skin that don't heal. Examination and treatment need to be done immediately to prevent a more severe condition.

Diagnosis Pemfigus

The doctor will conduct a question and answer regarding the patient's symptoms and medical history, then proceed with a physical examination. Next, the doctor will carry out further examinations, such as:

  • Blood test , to detect antibodies that cause pemphigus
  • Biopsy, to examine a sample of skin tissue from the blister under a microscope
  • Endoscopy, to detect sores in the throat using a tool in the form of a small tube equipped with a camera at the end

Pemphigus Treatment

Pemphigus treatment aims to reduce the formation of blisters, prevent infection, and heal wounds. The doctor will adjust the treatment method to the severity of the condition and the patient's medical history.

Pemphigus treatment can be done as an emergency or as long-term treatment. The following is an explanation of each treatment method and its explanation:

Emergency handling

Emergency treatment aims to treat blisters and prevent new blisters from forming. Some actions that can be taken are:

1. Corticosteroids

In severe cases of pemphigus, the doctor will give high doses of corticosteroids to prevent new blisters from forming. After it is confirmed that new blisters are not forming again, the dose will be gradually reduced to reduce the risk of side effects from corticosteroids.

2. Plasmaferesis

Patients can also be advised to undergo plasmapheresis procedures. This method is carried out by removing plasma from the patient's body, then replacing it with other plasma from the donor. Plasmapheresis aims to remove antibodies that attack the skin.

3. Immunoglobulin

Immunoglobulin injections aim to reduce or prevent the severity of infection in patients with compromised immune systems, including pemphigus. Immunoglobulins can also neutralize antibodies that turn against healthy cells.

4. Wound care

Treatment of a wound in pemphigus is similar to that for a severe burn. Patients can be given intravenous fluids or electrolytes if they lose a lot of fluids.

If the patient has sores in the mouth, the doctor can add treatment in the form of administering nutrients and fluids through an IV. If needed, antibiotics can also be given to patients.

Long term care

Pemphigus is a condition that cannot be completely cured. Some of the treatments above can heal blisters, but it does not rule out the sores will reappear. Therefore, the doctor will provide medication for long-term care, among other things

  • Medications that suppress the immune system ( immunosuppressants ), such as rituximab in injection form, mycophenolate mofetil , azathioprine, and cyclophosphamide
  • Topical therapy , such as topical steroids and emollients

To help the healing process, there are several ways that pemphigus sufferers can do, namely:

  • Follow the doctor's instructions in caring for the wound, to prevent infection and scar tissue formation.
  • Gently cleanse the skin using a mild soap and apply moisturizer afterwards.
  • Avoid activities that can cause skin injury.
  • Avoid consuming spicy or hot foods to prevent mouth irritation.
  • Limit excessive sun exposure by wearing long clothes and wearing sunscreen when doing activities outside the home.
  • Manage stress by doing meditation or yoga.

Pemphigus complications

Pemphigus that is prolonged and not treated immediately can cause several complications in sufferers, namely:

  • Malnutrition
  • Candida yeast infection ( candidiasis )
  • Herpes simplex virus infection
  • Secondary infection
  • Sepsis
  • Osteoporosis
  • Anxiety disorder
  • Depression

Pemphigus prevention

Pemphigus is a condition that cannot be prevented. However, pemphigus sufferers can prevent recurrence of pemphigus symptoms by diligently undergoing long-term treatment and carrying out routine controls to the doctor.

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