Supporting Lives Affected by Pemphigus and Pemphigoid Across Africa

BULLOUS PEMPHIGOID

This page covers the following:

• Introduction
• How is bullous pemphigoid diagnosed?
• What are the symptoms of bullous pemphigoid?
• Can bullous pemphigoid be cured?
• How is bullous pemphigoid treated?
• Where can I find out more information about bullous pemphigoid?

INTRODUCTION

Bullous pemphigoid (BUL-us PEM-fih-goid) is a rare skin condition that causes large fluid-filled blisters. They often appear on the skin near creases, such as the upper thighs and armpits. Sometimes, people get a rash instead of blisters. The affected areas may be painful and are usually very itchy. Blisters or sores also might form in the mouth, but this is rare.

Bullous pemphigoid occurs when the immune system attacks a layer of tissue in the skin. The reason for this immune system response is not well understood. In some people, the condition is brought on by certain medicines.

How is bullous pemphigoid diagnosed

The look of the rash, especially if blisters have formed, may be enough for your GP to make the diagnosis, but further investigations are usually performed including a blood test and skin biopsies (under local anaesthetic). This will determine which type of PEM (if any) is present. If BP is suspected, a skin biopsy is performed for histology and direct immunofluorescence testing. Samples from within and around the lesion are often used for histology, but samples of uninvolved skin (often about 3 mm from the edge of a lesion) are used for direct immunofluorescence. Biopsies reveal the layers of skin that are affected by the blisters and direct immunofluorescence shows where the pemphigoid antibodies are binding in the skin. A blood test usually shows the circulating autoantibodies specific to BP.

What are the symptoms of bullous pemphigoid?

The symptoms of bullous pemphigoid may include:

1. Itching, which can start weeks or months before blisters form.
2. Large blisters that don't break easily, often found along skin folds. On brown and Black skin the blisters may be dark pink, brown or black. On white skin they may be yellow, pink or red.
3. Pain.
4. A rash.
5. Small blisters or sores in the mouth or other mucous membranes. This is a symptom of a rare type of the disease called mucous membrane pemphigoid.

Bullous Pemphigoid blisters

Blisters are firm and filled with a fluid. Most commonly, the fluid is clear but, in some people, it can be bloodstained. The blisters will gradually grow as they fill with fluid and then burst, often leaving raw skin which is very sore. When the skin blisters heal, they do not leave permanent scarring but skin colour changes might be observed after the inflammation has settled down.

Can bullous pemphigoid be cured?

BP cannot be cured but with the correct diagnosis, management and treatment it can be controlled. However, sometimes it does slowly resolve itself after one to five years.

More information about bullous pemphigoid?

For more detailed information on BP, see the British Association of Dermatologists Patient Leaflet. The New Zealand Dermatology service, DermNet NZ, also produces very good literature on BP.

The UK Centre of Evidence Based Dermatology investigates aspects of BP. You can find videos and information about their findings on their website.