Bullous pemphigoid is a rare skin condition that typically affects people in middle age and beyond. It causes a range of skin findings, from itchy, hive-like welts to large, fluid-filled blisters which can get infected.
Bullous pemphigoid is an autoimmune disease, which means that the cells in the body that normally fight infection attack the body instead. The body’s immune system is confused and makes an antibody (type of protein used to fight infection) that targets a part of the skin that normally holds it together. The attack on the skin causes blisters (firm, fluid-filled bubbles on the skin) to form. This disease most often involves only the skin, but the eyes, mouth, and genitals also can be affected. In most cases, the disease develops on its own, but certain medications also can cause bullous pemphigoid to develop.
A nonspecific red, itchy rash may be present for several weeks or months before blisters appear. The rash may be thought to be due to dermatitis or urticaria. The early plaques may resolve temporarily or persist, with or without treatment. They may have a smooth or dry surface and are often ring-shaped (annular).
Bullous pemphigoid may affect a small area of the body or be widespread. The blisters usually occur on areas of the skin that are flexible, such as under the armpit or on the abdomen. One-third of people with bullous pemphigoid develop blisters in their mouth, throat, and esophagus in addition to their skin.
Bullous pemphigoid occurs most often in people over age 60, but it can appear in younger individuals as well. It is seen more in the Western world; it is not common in the Far East. Both men and women are affected equally by bullous pemphigoid.
Causes
Pemphigoid is an autoimmune disease. This means that your immune system mistakenly 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. It is not fully understood why the immune system reacts this way in patients with pemphigoid
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 are found to be at a higher risk for developing pemphigoid. It is more common in the elderly than in any other age group.
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
Bullous pemphigoid can present with itching, hive-like welts, and multiple blisters, called bullae.
The blisters have certain characteristics, regardless of where on the body they form:
- A red rash develops before the blisters
- The blisters are large and filled with fluid that is usually clear, but may contain some blood
- The blisters 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
These are most commonly seen on the –
- Arms
- Legs
- Abdomen
- Groin
- Mouth
The blisters may break open and become an open sore or ulcer. The fluid inside the may be clear, or contain some blood. The skin around the blisters can appear either normal or red. The blisters are usually located along creases in the skin
Treatment
As in other autoimmune bullous diseases, the goal of therapy is to decrease blister formation, to promote healing of blisters and erosions, and to determine the minimal dose of medication necessary to control the disease process.
Medications
Corticosteroids – The most common treatment is prednisone, which comes in pill form. But long-term use can increase your risk of weak bones, diabetes, high blood pressure, high cholesterol and cataracts. Corticosteroid ointment can be rubbed on your affected skin and causes fewer side effects.
Drugs that suppress the immune system – These drugs inhibit the production of your body’s disease-fighting white blood cells. Examples include azathioprine (Azasan, Imuran) and mycophenolate mofetil (CellCept). Immunosuppressants are often used to help reduce the dosage of prednisone you may need.
Other drugs that fight inflammation – Other drugs with anti-inflammatory properties may be used alone or with corticosteroids. Examples include methotrexate (Trexall), a rheumatoid arthritis drug; tetracycline, an antibiotic; and dapsone (Aczone), a leprosy treatment.
These other medications may include –
- Topical steroids (usually clobetasol propionate); these may be used as sole treatment in mild or localised disease
- Tetracycline antibiotics
- Nicotinamide
- Dapsone
- Azathioprine
- Methotrexate
- Mycophenolate
- High dose intravenous immunoglobulin
Alternative Treatment (Not Found)