Bullous pemphigoid is a chronic blistering of the skin. It is an autoimmune disorder, meaning it is caused when the body's immune system malfunctions. The immune system is meant to defend the body against bacteria, viruses, and disease, but instead produces antibodies against healthy tissue, cells and organs. The cause is not known, but may be related to immune system disorders. Bullous pemphigoid typically occurs in elderly persons and is rare in young people. It is characterized by a pattern of exacerbations and remissions. In most patients, the disease spontaneously clears within 6 years. It may be localized to one area but is more often widespread, often favouring body folds. In severe cases, there may be blisters over the entire skin surface as well as inside the mouth. Bullous pemphigoid is usually evaluated by a doctor who has special training in diagnosing and treating skin conditions.
The skin over the blisters is quite strong and may remain for several days before the blister bursts. Some blisters do not burst but the fluid is absorbed into the body and the roof of the blister settles back down on the skin. Most blisters eventually rupture and form shallow erosions. The most common complications of bullous pemphigoid arise from the medications used to treat the condition. Drugs called immunosuppressants help treat bullous pemphigoid, but they also subdue your immune system, which may increase your likelihood of developing certain infections and cancers. Tetracycline and Minocycline antibiotics are very useful for mild to moderate disease. They do not work on bacteria, but act directly on the immune system. They can be used in combination with potent topical steroid creams for more rapid relief. Severe cases are best treated in the hospital to allow expert dressing of the wounds, and intravenous injections of the most potent treatments.
Causes of Bullous pemphigoid
Bullous pemphigoid is an autoimmune disease. This means that the cells in the body that normally fight infection or germs attack the skin cells, causing blisters. The cause of bullous pemphigoid is an unknown autoimmune mechanism wherein autoantibodies attack glycoproteins in the dermal basement membrane. In rare cases, certain drugs may precipitate true disease or may cause a blistering drug eruption, which is a temporary condition that, unlike true pemphigoid, eventually resolves once the offending agent is discontinued.
Common causes and risk factors of Bullous pemphigoid:
- Immune system disorders.
- Certine viruses.
- Sunlight, or other factors.
- Autoimmune (the body's immune system mistakenly causes a reaction).
- Chemical reactions.
Signs and Symptoms of Bullous pemphigoid
Bullous pemphigoid is a disease characterized by tense blistering eruptions of the skin. Small patches of itchy skin are typical at first. Blisters develop a week or more later. The blisters are quite firm and dome-shaped. The blister fluid is usually clear, but may be blood-stained. Any area of skin can be affected but the arms, legs, armpits, and groin are the most common sites. The body may be covered in blisters but sometimes the blisters occur just in one area - often on the lower leg. Some blisters do not burst but the fluid is absorbed into the body and the roof of the blister settles back down on the skin.
Sign and symptoms may include the following :
- Bleeding gums.
- Mouth sores.
- General ill feeling.
- Hemorrhagic blisters.
Treatment for Bullous pemphigoid
Treatment is focused on relief of symptoms and prevention of infection. Most patients with bullous pemphigoid are treated with steroid tablets, usually prednisone. The dose is adjusted until the blisters have stopped appearing, which usually takes several weeks. The dose of prednisone is then slowly reduced over many months or years. Immunosuppresants work by suppressing the immune system. Also, the dose of steroid needed may be less if you take an immunosuppressant. This means that any side-effects from steroids may be less severe. Antibiotics may be required for secondary bacterial infection.
Treatment may include:
- Tetracycline and Minocycline antibiotics are very useful for mild to moderate disease.
- They can be used in combination with potent topical steroid creams for more rapid relief.
- Oral steroids are the treatment of choice for severe cases.
- Often, immunosuppressive agents ( Immuran, Cellcept, Methotrexate, cyclophosphamide and Neoral ) are used in combination with the oral steroids to allow a lower dose.
- The condition can be controlled with strong medications. Bullous pemphigoid sometimes gets better on its own and can even disappear completely after a few years.