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Bullous Pemphigoid

Definition


Disease: Bullous Pemphigoid Bullous Pemphigoid
Category: Dermatological diseases
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Disease Definition:

When fluid-filled blisters (bullae) erupt on the surface of the skin, usually on the arms, legs or trunk, a rare condition called bullous pemphigoid occurs. Although this condition is thought to be related to a disorder of the immune system, its exact cause is still not known.

Treatment for this condition focuses on suppressing the immune response and inflammation over time. Without treatment, this condition could persist, with periods of remission and flare-ups for many years.
Bullous pemphigoid usually occurs in older adults.

Work Group:


Prepared by: Scientific Section

Symptoms, Causes

Symptoms:

The signs and symptoms of this condition could be either mild or severe. Although some people may experience only a slight redness and irritation on their skin, others may experience multiple blisters (bullae), which normally develop on the arms, legs or trunks, but could also develop in the mouth or other mucous membranes.

Sometimes, the blisters could form painful and open sores on the surface of the skin. Usually they appear as firm and fluid-filled sacs, which could either weep or appear crusty.

Some of the signs and symptoms of this condition include:

 

  • Rashes
  • Itching
  • Irritation
  • Mouth sores
  • Multiple blisters
  • Bleeding gums
  • Hive-like lesions
  • redness

Causes:

In this condition, antibodies that are produced by the immune system attack the basement membrane, which is a thin layer of connective tissue in the skin. Fluid-filled blisters form when the delicate basement membrane becomes inflamed.
What causes these antibodies to attack healthy tissue in the body is still not clear.

Complications

Complications:

One of the common complications of this condition is infection of the skin.
However, the medications used to treat this condition could cause some other complications.
Bullous pemphigoid could be treated with corticosteroids and immunosuppressants. However, these medications also depress the immune system functions, leading to an increased risk of developing certain cancers and infections. A person's risk of developing osteoporosis and bone fractures could also increase if they use corticosteroids for a long time.

The quality of the patient's life would also be affected because of this condition, for instance, when blisters appear in the mouth, they would make it harder for the patient to eat. Doing daily activities could also be difficult, especially if someone has ruptured blisters or blisters on the palms of the hands or soles of the feet. This condition could also affect the vision because it can lead to scarring of the eye.

Treatments:

Suppressing the autoimmune response in the skin and reducing inflammation, thereby easing the symptoms, is what treatment for this condition focuses on.

Immunosuppressants:
These drugs reduce inflammation by helping calm the autoimmune response in the skin. They include mycophenolate mofetil  and azathioprine.

Corticosteroids:
These drugs could be used orally (pill), which most people with this condition need to take at least in the beginning of therapy, or they could be used as a topical cream that is spread on the surface of the skin.

Corticosteroid-sparing agents:
These drugs help reduce the dosage or the need for continued use of corticosteroids, such as tetracycline, dapsone and in some severe cases, immunosuppressants.

If this condition is left untreated, it could disappear completely on its own, but usually within six to ten years. However, when treated, it only takes one and a half to five years to disappear completely.

Prognosis:

Not Available

Expert's opinion

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