Pemphigoid: Types, Causes, Symptoms and Outcome

picture of bullous-pemphigoid


Pemphigoid is a rare autoimmune disorder that can develop at any age, but that most often affects the elderly. Pemphigoid is caused by a malfunction of the immune system and results in skin rashes and blistering on the legs, arms, and abdomen. Pemphigoid can also cause blistering on the mucous membranes of the eyes, nose, mouth, and vagina, and can occur during pregnancy in some women. There is no cure for pemphigoid, but there are many treatment options.

Types of Pemphigoid

All types of pemphigoid are caused by an immune system malfunction. They create rashes and fluid-filled blisters. The types of pemphigoid differ in terms of where on the body the blistering occurs and when it occurs.

  • Bullous Pemphigoid – n cases of bullous pemphigoid, the skin blistering occurs most commonly on the arms and legs where movement occurs, that is, around the joints and on the lower abdomen.
  • Cicatricial Pemphigoid – Cicatricial pemphigoid refers to blisters that form on the mucous membranes. This includes the mouth, eyes, nose, throat, and genitals. The rash and blistering may begin in one of these areas and spread to the others if left untreated.
  • Pemphigoid Gestationis – When blistering occurs during or shortly after pregnancy, it is called pemphigoid gestationis. It is also called herpes gestationis, although it is not related to the herpes virus. The blistering typically develops during the second or third trimester, but may occur at any time during pregnancy or up to six weeks after delivery. Blisters tend to form on the arms, legs, and abdomen.

Causes and Risk Factors

Pemphigoid is an autoimmune disease. This means that your immune system, which normally protects you from infection, begins to attack your healthy tissues. In the case of pemphigoid, your immune system creates antibodies to attack the tissue just below your outer layer of skin. This causes the layers of skin to separate and results in painful blistering. Why the immune system reacts this way in patients with pemphigoid is not fully understood.

In many cases, there is no specific trigger for pemphigoid. In some instances, however, it may be caused by certain medications, radiation therapy, or ultraviolet light therapy. People with other autoimmune disorders may be at a higher risk for developing pemphigoid. It is much more common in the elderly than in any other group.

Symptoms of Pemphigoid

The most common symptom of pemphigoid is blistering that occurs on the arms, legs, abdomen, and mucous membranes. Hives and itching are also common. The blisters have certain characteristics, regardless of where on the body they form:

  • they are often preceded by a red rash
  • they are large and filled with fluid that is usually clear, but may contain some blood they are thick and do not rupture easily
  • the skin around the blisters may appear normal or slightly red or dark
  • ruptured blisters are usually sensitive and painful

Diagnosing Pemphigoid

Your doctor will be able to make a fairly firm diagnosis simply by examining your blisters. However, to know for sure and to prescribe the right treatment, further tests will be required.

Your doctor may want to perform a biopsy, which involves removing small samples of skin from the affected areas. Lab technicians will test these samples for the immune system antibodies characteristic of pemphigoid. These antibodies can also be detected in your blood, so you may need to have a small sample of blood drawn.

Treatments for Pemphigoid

Pemphigoid cannot be cured, but treatments are usually very successful at relieving symptoms. Corticosteroids, either in pill or topical form, will likely be the first treatment your doctor prescribes. These medications reduce inflammation and can help to heal the blisters and relieve itching. However, they can also cause serious side effects, especially from long-term use, so your doctor will take you off of the corticosteroids after the blistering clears up.

Another treatment option is to take medication that suppresses your immune system, often in conjunction with the corticosteroids. Immunosuppressants help, but they also put you at risk for other infections. Certain antibiotics, such as tetracycline, may also be prescribed to reduce inflammation and infection.

Long-Term Outlook

With comprehensive treatment, the outlook for pemphigoid is good. Most people respond well to medication. Often, the disease will go away after a few years of treatment. Even with proper treatment, however, pemphigoid may return at any time.