Bullous pemphigoid

DEFINITION

Bullous pemphigoid (BUL-us PEM-fih-goid) is a rare skin condition that causes large, fluid-filled blisters on areas of skin that often flex — such as the lower abdomen, upper thighs or armpits. Bullous pemphigoid is most common in people older than age 60.

 

Bullous pemphigoid occurs when your immune system attacks a thin layer of tissue below your outer layer of skin. The reason for this abnormal immune response is unknown, although it sometimes can be triggered by taking certain medications.

Treatment usually includes corticosteroids, such as prednisone, and other drugs that suppress the immune system. Bullous pemphigoid can be life-threatening, especially for older people who are already in poor health.

CAUSES

The cause of bullous pemphigoid is not well understood. The blisters occur because of a malfunction in the immune system.

 

Your body’s immune system normally produces antibodies to fight bacteria, viruses or other potentially harmful foreign substances. For reasons that are not clear, the body may develop an antibody to a particular tissue in your body.

In bullous pemphigoid, the immune system produces antibodies to the skin’s basement membrane, a thin layer of fibers connecting the outer layer of skin (epidermis) and the next layer of skin (dermis). These antibodies trigger inflammatory activity that produces the blisters and itching of bullous pemphigoid.

Contributing factors

Bullous pemphigoid usually appears randomly with no clear factors contributing to the onset of disease. A small percentage of cases may be triggered by certain medical treatments, such as:

  • Medications. Prescription drugs that may cause bullous pemphigoid include penicillin, etanercept (Enbrel), sulfasalazine (Azulfidine) and furosemide (Lasix).
  • Light and radiation. Ultraviolet light therapy to treat certain skin conditions may trigger bullous pemphigoid, as can radiation therapy to treat cancer.

SYMPTOMS

The primary feature of bullous pemphigoid is the appearance of large blisters that don’t easily rupture when touched. The fluid inside the blisters is usually clear but may contain some blood. The skin around the blisters may appear normal or red.

 

In most cases, the blisters appear on the lower abdomen, groin, upper thighs and arms. Blisters are often located along creases or folds in the skin, such as the skin on the inner side of a joint. The affected areas of skin can be very itchy. You might also develop blisters or sores in your mouth. Rarely, the mucous membranes of the eyes can be involved, creating redness, soreness and discomfort.

When to see a doctor

If you develop unexplained blistering — a condition not caused, for example, by a known skin allergy or contact with poison ivy — see your doctor.