Bullous Pemphigoid

Overview

Bullous pemphigoid is a rare skin condition where the body's immune system, which normally fights infection, mistakenly attacks a thin layer just under the surface of the skin. This causes large, firm, itchy blisters. It is not an infection, it is not contagious, and you cannot catch it from someone else or pass it on. It is a long-term condition that tends to flare up and settle down, and it can usually be controlled well with treatment.

It is uncommon. It is the most common blistering skin disease in older adults, but it is still rare overall, affecting roughly a small number of people per million each year.

It mostly affects people over 70, and becomes more likely as people get older. It can affect men and women of all backgrounds. It is rare in children and younger adults, but it can happen. Some medicines and some long-term health conditions, such as dementia or Parkinson's disease, are linked to a higher chance of developing it.

Symptoms

Common symptoms

• Intense itching, which can be very uncomfortable and may keep you awake at night
• Raised, red or hive-like patches that may come before any blisters
• Large, firm blisters filled with clear or slightly cloudy fluid
• Blisters that often appear on the arms, legs, armpits, groin, lower belly and chest
• The skin under and around blisters can look red and inflamed
• Blisters may burst and leave raw, sore areas that can take time to heal
• Sometimes the mouth or other moist areas are affected, causing soreness

Symptoms in children

• Bullous pemphigoid is rare in children, but when it happens it can look similar to the adult form
• Itchy red patches and blisters are the usual signs, often on the arms and legs
• Because blistering rashes in children can have many causes, a doctor should always check them
• A child with a blistering rash should be seen by a healthcare professional promptly

Symptoms in older adults

• Older adults are the group most often affected
• The first sign may be an very itchy rash without blisters, which can be mistaken for eczema or a reaction
• Blisters may be fewer in number but the itch can be severe
• Skin may tear or blister more easily, especially after rubbing, pressure or a fall
• Because older skin heals more slowly, sores need good care to avoid infection
• Some people feel generally tired or unwell during a flare

Causes

Main causes

• Bullous pemphigoid is an autoimmune condition. The immune system makes antibodies (defence proteins) that mistakenly target two proteins that help hold the top layer of skin to the layer beneath. When these attachments are damaged, the layers separate and fluid collects, forming a blister.
• We do not fully understand why the immune system starts doing this in most people.
• In some cases, a medicine may trigger it, or it may start after an illness or injury. Often no clear trigger is found.

Risk factors

• Getting older — it is much more common over the age of 70
• Having certain long-term nerve conditions, such as dementia or Parkinson's disease
• Some prescription medicines have been linked to it; never stop a medicine on your own, always ask your doctor or pharmacist first
• Skin damage from sunburn, friction, burns, surgery or radiotherapy
• Having another autoimmune condition
• It is not caused by diet, stress, poor hygiene or anything you did wrong

When to see a doctor

See a doctor urgently if:

• A new blistering rash that appears quickly or spreads fast
• Blisters or sores in the mouth, eyes or genitals
• Signs of infection in a sore: increasing pain, redness, warmth, pus or fever
• Difficulty eating or drinking because of mouth blisters
• Feeling very unwell, confused or dizzy alongside the skin problem

Book a routine appointment if:

• An itchy, persistent rash that does not settle, especially if you are over 70
• Any new blisters, however few
• A skin problem that has not improved after usual treatment from your pharmacist or doctor
• If you have been diagnosed and a new flare starts, so treatment can be adjusted
• If you are worried or unsure, it is always reasonable to ask for a check-up

Diagnosis

A doctor will usually start by asking about your symptoms, your health history and any medicines you take, then look at your skin. Because blistering conditions can look alike, they will often refer you to a skin specialist (dermatologist). The specialist may take a small sample of skin and a sample of fluid from a blister to confirm the diagnosis.

Tests that may be done

• A skin biopsy: a small sample of skin is numbed and taken for testing in a laboratory
• A special skin test called immunofluorescence, which looks for the antibodies causing the problem
• A blood test to check for the antibodies linked to bullous pemphigoid
• General blood tests to check your overall health and see how your liver and kidneys are working before treatment starts
• Sometimes tests to rule out other blistering conditions or to look for a trigger

What to expect at your appointment

You may be seen in a dermatology clinic, and results can take a week or more to come back. You may be given treatment to ease the itch and calm the rash while you wait. Diagnosis is based on your skin, your story and the test results together. It can feel frustrating to wait, but getting the right diagnosis helps make sure you get the right treatment.

Treatment

The aim of treatment is to calm the immune system, stop new blisters forming, heal the skin and control the itch. Treatment is tailored to you, taking into account your age, other health conditions and how severe the flare is. Most people need treatment for many months, and sometimes longer, with the dose reduced gradually as the skin settles.

Self-care at home

• Handle your skin gently — pat rather than rub when drying
• Do not burst blisters; if one bursts on its own, cover it with a clean, non-stick dressing
• Use a gentle, fragrance-free moisturiser several times a day to protect the skin
• Take cool baths or use cool compresses to calm itching
• Wear loose, soft clothing and avoid tight waistbands or rough fabrics
• Avoid sunburn, hot water, friction and injuries that can trigger new blisters
• Keep fingernails short and consider cotton gloves at night to reduce scratching
• Ask your pharmacist or nurse about dressings if you have broken skin
• Keep taking any prescribed treatment exactly as directed, and do not stop suddenly
• Ask your healthcare team about vitamin D and bone health if you are on long-term treatment

Medical treatments

Doctors usually prescribe strong anti-inflammatory medicines, often called steroids, which may be applied as a cream or taken as tablets. In many people, a very strong steroid cream used on the skin works well and avoids the need for tablets. If the condition is widespread or not settling, tablets may be needed. Other medicines that calm the immune system may be used to help reduce the amount of steroid needed or to control difficult cases. Your doctor will also treat itching and may recommend treatments to protect your bones and stomach if you need steroids for a long time. Treatment is adjusted over time, and you will have regular check-ups and blood tests. Never start, stop or change any medicine without talking to your doctor or pharmacist first.

When is surgery considered?

Surgery is not a treatment for bullous pemphigoid itself. Occasionally, a small procedure may be needed to care for a badly damaged area of skin, or a skin sample may be taken as part of diagnosis. Your skin team will explain if any procedure is being considered and why.

Living with this condition

Bullous pemphigoid is a long-term condition that tends to flare and then settle. With treatment, most people find the itch and blisters become much more manageable. Day to day, the focus is on gentle skin care, taking treatment as prescribed, keeping up with check-ups, and knowing the signs of infection so you can get help quickly. It can take time for sores to heal, so be patient with yourself.

Lifestyle tips

• Keep your skin protected from the sun with clothing and shade
• Avoid very hot baths, harsh soaps and perfumed products
• Choose soft, breathable fabrics and loose fits
• Keep your home comfortably cool, as heat can make itching worse
• Stay well hydrated, especially if you have broken skin or are unwell
• Ask for help with heavy lifting or tasks that rub the skin if you are prone to blistering
• Keep a simple diary of flares and possible triggers to discuss with your doctor
• Make sure any other health professionals you see know about your diagnosis and treatment

Diet and exercise

There is no special diet that treats bullous pemphigoid. Eating a balanced diet with enough protein, fruit and vegetables helps your skin heal and supports your general health. Gentle regular movement, such as walking or stretching, is good for your overall wellbeing and helps keep you mobile. If blisters or sores make movement uncomfortable, tell your healthcare team, who can suggest ways to stay active safely. Always check with your doctor before starting a new exercise plan if you have other health conditions.

Mental health and emotional wellbeing

Living with a visible, itchy skin condition can be hard. Itching can disturb sleep, and blisters can affect confidence, mood and daily life. Feeling worried, low or frustrated is understandable. These feelings are common and they are not a sign of weakness. Talk to your doctor or nurse — support such as counselling, relaxation techniques and sleep advice can help. If you ever feel you cannot cope or have thoughts of harming yourself, please reach out for crisis support straight away: contact your local emergency number or a crisis line in your country, or tell someone you trust and ask them to stay with you.

Prevention

Bullous pemphigoid cannot usually be prevented, because it starts when the immune system mistakenly attacks the skin. What you can do is reduce the chance of flares and complications: protect your skin from sunburn, friction and injury, take your treatment as prescribed, keep up with check-ups, and report new symptoms early. If a medicine is thought to be a trigger, your doctor will review it carefully — never stop a medicine yourself.

Screening programmes

There is no routine screening test for bullous pemphigoid. If you have been diagnosed, your team will monitor you with check-ups and blood tests to make sure treatment is working and to catch any side effects early. Tell your doctor about any new blisters, sores or unusual skin changes.

Complications

If left untreated

• Blisters can burst and leave raw areas that are open to infection
• Skin infection can become serious, especially in older adults
• Fluid loss and poor healing from widespread raw skin can make you feel unwell
• Severe itching can disturb sleep, mood and daily life
• Mouth sores can make eating and drinking difficult, leading to dehydration
• Long-term inflammation can cause scarring or changes in skin colour in some people
• Some treatments, especially long-term steroids, can have side effects if not monitored

Long-term outlook

The outlook is generally good. With treatment, most people see their skin clear or improve a great deal, and many go into remission (a period with no symptoms). Some people need treatment for months or a few years, and flare-ups can happen, but they can usually be brought under control again. Your healthcare team will work with you to find the safest and most effective plan, and will keep checking your progress. Many people live full, normal lives with this condition.

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