Bullous Pemphigoid

1 What is Bullous Pemphigoid?

Bullous pemphigoid (BUL-us PEM-fih-goid) is a chronic inflammatory, but rare skin disorder that is characterized by large, fluid-filled blisters.

These blisters usually develop on the areas of skin that often flex such as the lower abdomen, upper thighs or armpits.

This condition occurs more commonly in older people above the age of 60. Bullous pemphigoid is an autoimmune disorder that occurs when your body's immune system attacks a thin layer of healthy tissue below the outer layer of your skin.

However, the cause for this abnormal immune response is not known, but it sometimes gets triggered by taking certain medications.

The treatment for bullous pemphigoid includes medications such as prednisone and immunosuppressive drugs.

If left untreated, this skin condition persists for several months to years during spontaneous renissions and excerbations occur.

This disorder can be life-threatening, especially in older people with poor general health.

2 Symptoms

The main symptom of bullous pemphigoid is the appearance of several large blisters called bullae that do not break open easily when touched.

The fluid that accumulates inside the blisters is usually clear but may sometimes contain little amount of blood. The skin surrounding the blisters may be normal, reddish or darker than usual.

In most cases, the blisters usually form on

  • the arms,
  • upper thighs,
  • lower abdomen,
  • groin,
  • upper thighs.

Blisters are often located along the skin creases or folds such as the skin on the inner side of a joint. The affected parts may be very itchy. Blisters or sores may also develop in your mouth.

In some people, an eczema or hive-like rash may develop rather than blisters. If the blisters are concentrated in the mucous membrane of your eyes and mouth, the condition is called mucous membrane pemphigoid. Scarring is more likely to occur if you develop blisters on your eyes.

This condition should be identified early and treated appropriately.

When to see a doctor

It is important to consult your doctor if you develop unexplained blistering — a condition not caused, for example, by a known skin allergy or contact with poison ivy.

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3 Causes

The cause of bullous pemphigoid is not known clearly. The blisters are thought to form as a result of an abnormality in your immune system.

Normally, your immune system produces antibodies to fight against invading bacteria, viruses or other potentially harmful substances. Sometimes, for unknown reasons, the body may start producing an antibody against a healthy tissue in your body.

In bullous pemphigoid, the immune system develops antibodies against the fibers connecting the outer layer of skin (epidermis) to the next layer of skin (dermis). These antibodies activate an inflammation that causes the blisters and itching of bullous pemphigoid.

Contributing factors

Bullous pemphigoid usually develops at random with no distinctive factors contributing to the onset of disease. However, in few cases, it may be triggered by certain medical treatments such as:

Medications

Prescription drugs such as

  • penicillin,
  • etanercept (Enbrel),
  • sulfasalazine (Azulfidine),
  • furosemide (Lasix)

may cause bullous pemphigoid.

Light and radiation

Ultraviolet light therapy used to treat certain skin conditions and radiation therapy to treat cancer may trigger bullous pemphigoid.

4 Making a Diagnosis

Making a diagnosis of bullous pemphigoid is done by performing several tests.

You may initially consult your primary care doctor. After evaluation, you will be referred to a doctor who specializes in skin disorders (dermatologist).

You may ask a friend or relative to accompany you to your appointment. In addition to offering support, the person who comes along can write down information given by your doctor or other clinic staff during the visit.

What you can do

Before going to your appointment, prepare a list of the following:

  • Symptoms you are experiencing and its duration
  • All your regular medications, vitamins, and supplements including their doses
  • Name and contact information of doctor you have consulted recently or see regularly

Questions to ask your doctor

For bullous pemphigoid, some basic questions you may ask your doctor include:

  • What is the most likely cause of my symptoms?
  • Are there any other possible causes?
  • What kind of tests do I need?
  • How long will the skin changes last?
  • What treatments are available, and which one do you recommend?
  • What side effects can I expect from the treatment you have suggested?

What to expect from your doctor

  • Your doctor may ask you a number of questions such as:
  • When were these symptoms first noticed?
  • What is the location of the blisters? Do they itch?
  • Is there any oozing, draining of pus or bleeding from the blister?
  • Have you recently started taking any new medication?
  • Have you had a fever?

Your doctor will confirm the diagnosis of bullous pemphigoid by doing following tests

  • Histopathologic analysis of a small sample of the affected skin taken from the edge of a blister
  • Direct immunofluorescence studies on normal-appearing perilesional skin.
  • Indirect immunofluorescence (IDIF): This test is performed using the patient's serum, if the result of DIF is positive.

5 Treatment

The treatment of bullous pemphigoid is focused towards healing of the skin as quickly as possible and providing relief from itching.

Your doctor will advise a combination of medications that suppress the activities of the immune system that cause inflammation. These drugs may include:

Corticosteroids

The most common drug is prednisone, which is taken orally. However, long-term use can increase your risk of

Corticosteroid ointment that can be applied on your affected skin causes fewer side effects.

Immunosuppressive drugs

These drugs inhibit the production of your body's defense cells- white blood cells. Examples include

  • azathioprine (Azasan, Imuran)
  • mycophenolate mofetil (CellCept)

Immunosuppressants help reduce the dosage of prednisone you need.

Other drugs that fight inflammation

Anti-inflammatory drugs may be used alone or in combination with corticosteroids. Examples include

6 Lifestyle and Coping

Lifestyle modifications are necessary in order to cope with bullous pemphigoid.

Following self-care strategies help you take care of your condition well.

Avoid injury

Your skin may be fragile due to the blisters of bullous pemphigoid and use of corticosteroid ointment.

Avoid getting injured, and if your blister breaks open, cover it with a dry, sterile dressing so that it heals properly without getting infected.

Avoid sun exposure

Avoid prolonged sun exposure, especially in the area of the skin affected by bullous pemphigoid.

Avoid eating certain type of foods

If there are blisters in your mouth, avoid eating hard foods, such as

  • chips,
  • raw fruits
  • vegetable

as these might aggravate your symptoms.

7 Risk and Complications

Bullous pemphigoid commonly occurs in older people above the age of 60, and the risk further increases with advancing age.

Complications

Sepsis

If the ruptured blisters get infected, it leads to sepsis, which is a potentially life-threatening blood infection that affects your entire body. Sepsis is more likely to occur in older adults with poor health condition.

Scarring

May occur in rare forms of bullous pemphigoid that involve the mucous membranes of the mouth or eye.

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