Bullous Pemphigoid – Causes, Symptoms and Treatment

Definition

Bullous pemphigoid is a rare, chronic condition in which fluid-filled blisters (bullae) erupt on the surface of your skin, usually on your arms and legs. A majority of cases occur in persons 80 years of age or older.

Yet another type of pemphigoid (pemphigoid gestationis) occurs during pregnancy. This leaflet will not discuss cicatricial pemphigoid or pemphigoid gestationis further.;

Bullous pemphigoid (BP) is rare – about seven people in a million develop it each year in the UK.

Causes

In people with BP, antibodies are made against the membrane between the top layer of skin (the epidermis) and the next layer (the dermis). Chemical reactions and inflammation in the skin due to this antibody attack causes fluid to build up as blisters between these two layers of skin.

Sometimes an outside event, such as sunburn or a reaction to a drug, seems to trigger the rash but usually it comes out of the blue. It is not infectious, due to allergies, or affected by diet or lifestyle.

Symptoms

Symptoms of bullous pemphigoid include hard, tight blisters on the skin. The skin around the blisters may appear normal, but is often red and swollen. Itching is common.

Some people might only notice a slight redness and irritation on their skin, while others may experience multiple blisters.

The blisters appear as firm, fluid-filled sacs at least 1 centimeter in diameter. They may weep or appear crusty. In some cases, the blisters may form painful, open sores on the surface of your skin.

Common symptoms are-

  • Usually located on the arms, legs, or trunk
  • May also occur in the mouth
  • May weep, crust over
  • May appear deep below the surface of the skin
  • May erode the skin, form ulcers or open sores
  • Itching

Treatment

Treatment of bullous pemphigoid can resolve with topical cortisone creams, but sometimes requires high doses of cortisone (“steroids”) taken internally. Recent research has indicated that large quantities of high-potency topical corticosteroids applied to the entire body surface were safer and more effective in controlling extensive bullous pemphigoid than oral corticosteroids.

Treatment is usually needed for several years, but generally after a few months it is possible to reduce the dose of medications to reasonably low levels. BP also often has a pattern of remissions and flare-ups. It may be without symptoms for 5 or 6 years then suddenly flare up.

Treatment for more severe blistering is usually with high doses of steroid tablets to get the pemphigoid under control quickly. This needs careful monitoring, especially in elderly patients or those taking other medicines. Treatment often starts in hospital to ensure good control and so that proper dressings can be put on the blisters and raw areas.

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