About bullous pemphigoid
What is bullous pemphigoid?
Bullous pemphigoid is an uncommon skin disease characterized by tense blisters on the surface of the skin. Occasionally, the inner lining tissue of the mouth, nasal passages, or conjunctivae of the eyes (mucous membrane tissue) can be involved. The condition is caused by antibodies and inflammation abnormally accumulating in a particular layer of the skin or mucous membranes. This layer of tissue is called the "basement membrane." These antibodies (immunoglobulins) bind to proteins in the basement membrane called hemidesmosomal BP antigens and this attracts cells of inflammation. The mucous membrane disease is also referred to separately as mucous membrane pemphigoid.
A majority of those affected by bullous pemphigoid are 50 years of age or older. While the cause is unknown, it is felt by some that an aging immune system may become activated in certain individuals with a genetic predisposition to develop bullous pemphigoid.
What are the symptoms for bullous pemphigoid?
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, reddish or darker than usual. Some people with bullous pemphigoid develop an Eczema or hive-like Rash rather than blisters.
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. If the mucous membranes of your eyes and mouth are primarily where your Blisters are concentrated, this type of condition is called mucous membrane pemphigoid. If you develop Blisters on your eyes, you're more likely to have scarring. This condition requires prompt diagnosis and treatment.
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.
What are the causes for bullous pemphigoid?
The cause of bullous pemphigoid is not well-understood. The blisters occur because of a malfunction in your 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 fibers that connect the outer layer of skin (epidermis) and the next layer of skin (dermis). These antibodies trigger inflammation 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.
What are the treatments for bullous pemphigoid?
The goals of bullous pemphigoid treatment are to help the skin heal as quickly as possible and relieve itching. Your doctor will likely prescribe a combination of drugs that inhibit immune system activities that cause inflammation. These drugs may include:
- 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.
What are the risk factors for bullous pemphigoid?
Bullous pemphigoid most commonly occurs in people older than age 60, and the risk increases with age.
Is there a cure/medications for bullous pemphigoid?
Bullous pemphigoid is a rare skin condition that causes large, fluid-filled blisters. They develop on areas of skin that often flex - such as the lower abdomen, upper thighs or armpits. Bullous pemphigoid is most common in older adults.
Treatment
- For moderate-to-severe cases of bullous pemphigoid, your healthcare provider may prescribe an oral corticosteroid, like prednisone in addition to a steroid-sparing immunomodulatory agent such as dapsone mycophenolate mofetil, azathioprine, methotrexate or chlorambucil.
- If you can't take corticosteroids or other immunomodulatory agents, your healthcare provider may prescribe oral tetracycline
- Treatment is focused on healing the skin and relieving itching while minimizing adverse side effects of medications.
Your doctor will likely prescribe one or a combination of the drugs: - 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 infection. Corticosteroid ointment can be rubbed on your affected skin and causes fewer side effects.
- Steroid-sparing drugs. These drugs affect the immune system by inhibiting the production of your body's disease-fighting white blood cells. Examples include azathioprine (Azasan, Imuran) and mycophenolate mofetil (CellCept). If your signs and symptoms involve the eyes or upper digestive tract, the drug rituximab (Rituxan) may be used if other approaches haven't helped.
- Other drugs that fight inflammation. An example is methotrexate (Trexall).
Symptoms
Itching skin,Large blisters that don't easily rupture when touched,Skin around the blisters that is normal, reddish or darker than normal,Eczema or a hive-like rash,Small blisters or sores in the mouth or other mucous membranes,In extreme cases- unexplained blistering, blisters on your eyes, signs of infection
Conditions
A rare skin condition that causes large, fluid-filled blisters
Drugs
Corticosteroid medications, such as prednisone,Steroid-sparing drugs,Other drugs that fight inflammation such as methotrexate (Trexall)
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