Bullous pemphigoid: Symptoms, causes and treatment

If you’re not already aware of the skin condition bullous pemphigoid, it’s probably because it’s quite a rare one. But if you have come across it you’re not likely to forget it, thanks to the fact it causes an itchy, raised rash that can develop into – sometimes very large – blisters.
 
Bullous pemphigoid can affect anyone, but it’s mostly found in older people (the NHS says it mainly affects people over 60 (i), while Patient UK suggests a slightly older age group – those over 70 – is the most likely to develop it (ii)). Every year in the UK, just four or five people in every 100,000 develop bullous pemphigoid, including children (though childhood bullous pemphigoid is very rare). So as you get older, your risk increases – 1.5 per 100,000 in people in their 50s develop bullous pemphigoid, rising to 46 per 100,000 in those aged 90 and older (iii). Some women also develop a related condition during pregnancy.      

 

What is bullous pemphigoid?

 
Bullous pemphigoid is an autoimmune disease (in fact it’s the most common autoimmune blistering disease (iii)). This means it’s one of many conditions where the immune system mistakenly attacks healthy tissue in the body – in the case of bullous pemphigoid, the tissue attacked is the membrane (called the basement membrane) between the top layer of skin (the epidermis) and its neighbouring layer (the dermis).
 
Nobody really knows why the immune system acts in this way in some people. According to the British Society for Immunology, those with autoimmune diseases have a defect that makes them unable to distinguish ‘self’ from ‘non-self’ or ‘foreign’ molecules – in other words their immune systems don’t just attack pathogens (disease-causing micro-organisms such as viruses and bacteria) but they also attack the cells and tissues they’re meant to protect (iv).
 
Read our guide to autoimmune conditions for more information about some of the other types, including rheumatoid arthritis, lupus, psoriasis, type 1 diabetes, coeliac disease and Hashimoto’s disease.
 

Who is at risk for bullous pemphigoid?      

 
If you’re affected by another autoimmune disease your risk of developing bullous pemphigoid may be higher than someone who doesn’t have a problem with autoimmunity – though since the risk is very small to begin with it doesn’t mean your risk is high, just higher than normal. Meanwhile bullous pemphigoid affects equal numbers of men and women as well as people of all races. Other things that may increase your risk include:
 

• Having a close relative who also has or had bullous pemphigoid (some experts believe some people may have a genetic predisposition to the disease (v) – though others claim it isn’t hereditary (vi))

• Having a neurological disorder such as dementia, Parkinson’s disease, stroke, epilepsy or multiple sclerosis

• Having the skin condition psoriasis (v)

 

What are the symptoms?

 
The first symptom of bullous pemphigoid is itchy skin. Later a rash can appear with patches of skin that look red or pink on white skin and dark red/brown on brown or black skin. At this stage the rash can resemble other skin conditions such as eczema and urticaria. Several weeks or sometimes even months after the rash appears blisters (bullae) may develop, mostly on the arms, legs, armpits and groin (though any area of skin can be affected, especially areas where there are skin folds).
 
These blisters – which are firm to the touch and dome shaped – can develop in just one area or all over the body. They can become very large and are filled with a clear fluid, though sometimes they may also contain blood. The blisters don’t burst easily, but when they rupture the underlying skin can be quite sore and painful. Once they’ve healed, the blisters don’t leave scars but some people may notice skin discolouration once the inflammation has settled down (post-inflammatory pigmentation).
 
Not everyone with bullous pemphigoid develops blisters, however. But if you do, the blisters can keep coming and going for months or even years (according to Patient UK bullous pemphigoid usually goes away after three to six years, though in some people the condition can persist for an even longer period (ii)). It can even be life-threatening, especially in elderly people who are in poor health and those who have a compromised immune system, as it can cause skin infections that may lead to sepsis (this is a potentially fatal reaction by the body to severe infection).
 
If you have bullous pemphigoid blisters it’s important not to try busting them yourself, as this can lead to skin infections. Your GP or practice nurse, however, will be able to safely drain any problematic blisters with a needle.
 

What conditions are similar to bullous pemphigoid?    

 
Bullous pemphigoid is one of a group of autoimmune blistering skin diseases, known collectively as pemphigoid. Other types of pemphigoid include:
 

Cicatricial pemphigoid  

 
Known more commonly these days as mucous membrane pemphigoid this causes blisters in the mucous membranes, which include the skin inside the mouth, nose, throat, genital areas and anus (the mouth is the most common area to be affected). Unlike in bullous pemphigoid, the blisters can leave scars that can cause longer-term damage (a hoarse voice when scarring happens in the throat, for instance).
 

Ocular mucous membrane pemphigoid  

 
As its name suggests, this is when the disease affects the eyes – specifically the conjunctiva, the thin mucous membrane that covers the eyeball and lines the inside of the eyelids. Both eyes are usually affected, though in most people one eye is affected more severely than the other. Symptoms include red, sticky and painful eyes, with scarring of the conjunctiva sometimes causing turned-in eyelids and eyelashes that scratch the surface of the eye (trichiasis).
 

Pemphigoid gestationis  

 
This can develop during the second and third trimesters of pregnancy and includes an itchy rash, usually on the arms – legs and abdomen – that develops into blisters. In most cases, the symptoms stop within days after childbirth, though some women may continue to experience itchy skin and blisters for up to six more weeks.
 

What causes bullous pemphigoid?

 
If you develop bullous pemphigoid, substances made by your immune system called IgG and IgE immunoglobins (antibodies) and activated T lymphocytes (white blood cells) are thought to attack your skin’s basement membrane. This sets off a series of actions that causes inflammation and eventually leads to the formation of blisters.
 
We don’t know why this happens in some people, but experts believe there are a few things that can sometimes trigger the autoimmune process that leads to bullous pemphigoid. These include:
 

• Some neurological disorders (as mentioned above)

• Medications including anti-inflammatory drugs (NSAIDs), some diabetes medicines, some antibiotics (eg amoxicillin, penicillin), diuretics (water pills) such as furosemide, and a medicine sometimes prescribed for high blood pressure called captopril (this belongs to a group of medicines called ACE inhibitors)

• Skin burns (including sunburn and electrical burns)

• Radiotherapy

• Ultraviolet light therapy

• Surgical procedures (including transplants)

• Chemical preparations

 
Most often, however, bullous pemphigoid develops at random, with no clear trigger involved. On the other hand it’s not contagious, so you can’t catch it from someone or pass it to another, and it isn’t thought to be affected by your diet or lifestyle.
 

Bullous pemphigoid treatment    

 
If your doctor thinks you may have bullous pemphigoid they will usually refer you to a skin specialist who can carry out tests – including a biopsy of the affected skin – to confirm it. There’s no cure for the condition, but there are treatments available that aim to help your skin heal, reduce the likelihood of infection and stop new patches or blisters forming. These include:
 

Steroid medicines

 
The main treatments for bullous pemphigoid are steroid tablets and high-strength steroid creams.  Steroid tablets are usually only prescribed for severe cases, where they are used alongside steroid creams. However steroid medication has a high risk of side effects, especially when taken in high doses for long periods of time. So if you need to use steroid treatment for longer than a month you may also have to take other medications to reduce your risk of side effects (for example you may need treatments to help prevent osteoporosis).
 
Indeed, side effects of medications are among the main complications associated with bullous pemphigoid alongside skin infections, sepsis and viral infections such as herpes simplex (the virus that causes genital and oral herpes, or cold sores) and varicella-zoster (the virus that causes chickenpox).
 
If steroid medicines aren’t suitable for you or if they don’t control your symptoms your specialist may prescribe alternative medicines, such as drugs that affect your immune system.
 

Antibiotics

 
Tetracycline antibiotics such as doxycycline are often used to treat bullous pemphigoid as they may help reduce inflammation. The advantage of these antibiotics is that while they are effective they are also significantly safer than steroid tablets, so they are often prescribed as a long-term treatment. Antibiotics may also be prescribed for you to help protect against secondary skin infections.
 
Meanwhile you will usually need specialist skin products such as creams to wash with and moisturisers to keep your skin as healthy as possible. If you are in pain, your doctor can also advise you about pain relief medication.
 

How can you help yourself?

 
As with any other health condition, making sure your lifestyle is as healthy as possible is a good idea if you have bullous pemphigoid. This means eating healthily, staying physically active, not smoking, limiting yourself to a moderate amount of alcohol if you drink, and making sure you take any medicines your doctor has prescribed for you correctly.
 
For instance, the British Association of Dermatologists says you shouldn’t change the dosage of any steroid tablets you’re taking without speaking to your doctor first. Your GP can also advise you about possibly changing your dose of steroid tablets when you feel unwell (these are often called sick-day rules) (vii).
 
Meanwhile the UK charity PEM Friends says there’s no real evidence that any foods contribute to pemphigoid (viii). However if your mouth has been affected and the pain is making eating difficult you may want to avoid certain foods such as hot, spicy, acidic or salty foods as well as sharp or crunchy foods that could irritate your mouth such as toast, crisps and crusty bread.
 

What is the best diet for bullous pemphigoid?

 
Some people with bullous pemphigoid have, however, suggested their symptoms improved when they stopped eating some foods. So if you think it may be helpful, try keeping a food diary to see if your symptoms become any worse whenever you eat a certain food or foods. Meanwhile if you don’t always eat as healthily as you should, you may want to consider taking a good-quality multivitamin and mineral supplement to make sure your body is getting all the essential nutrients it needs.
 
Some of the other things you can do to make yourself more comfortable include the following:
 

• Wear soft, loose-fitting clothes that allow your skin to breathe

• Keep your skin hydrated by using moisturising lotions and creams (ask your GP to suggest the best products for this)

• Use antibacterial soap and water to wash the affected skin as this may help prevent infections

• Use sunscreen whenever your skin is exposed to sunlight and avoid spending a lot of time in the sun

• If the skin on your feet is affected, try to avoid standing or walking for lengthy periods

 

Natural support from supplements

 
While there may not be any evidence that diet plays a part in the development of bullous pemphigoid, there are a few natural supplements that could offer some additional support to those who have the condition – though always ask your doctor for advice before taking supplements if you’re taking any prescription medication. These supplements include the following:
 
Vitamin D   This is probably best known for its role in boosting bone health. However some experts believe there may be a link between low levels of vitamin D and having a higher risk of developing autoimmune conditions, saying there may be benefits in giving supplements to those with autoimmune conditions who are deficient in it (ix).
 
Unfortunately vitamin D deficiency is thought to be common in many countries including the UK, as sunlight – our main source of vitamin D – is in short supply outside of the summer months. Indeed, the UK-based Scientific Advisory Committee on Nutrition advises that adults and children over the age of four should consider taking a daily supplement containing 10mcg of vitamin D during the autumn and winter as well as throughout the year if they avoid sun exposure or keep themselves covered up in the summer (x). Supplementing vitamin D may also be important for people with bullous pemphigoid who are advised to avoid sun exposure.
 
The recommended form of vitamin D is vitamin D3 or cholecalciferol, as it’s the natural form of vitamin D that the body makes when it’s exposed to sunlight. Vitamin D3 supplements are available in tablet form, and now you can also get them in veggie-friendly drops. Most vitamin D3 supplements are made from the fat of lamb’s wool, which means they’re unsuitable for vegans. However vegan vitamin D3 supplements sourced from lichen are now more widely available.
 
High-strength fish oils   The omega-3 fatty acids found in oily fish are widely thought to help with inflammation in the body, with one review of 38 studies suggesting omega-3 supplements may benefit people with inflammatory skin conditions (xi). You can add more omega-3 fatty acids to your diet by eating more oily fish such as mackerel, salmon, sardines and fresh (not tinned) tuna, or by taking a good-quality, high-strength fish oil supplement.
 
Vitamin E   This nutrient is often described as essential for healthy skin, with one of its benefits being its anti-inflammatory capability. It may not have been investigated for its effect specifically on bullous pemphigoid, but studies have suggested vitamin E levels can be low in people with some inflammatory skin diseases (xii). This potentially suggests vitamin E supplements might have a beneficial role in treating skin conditions in which inflammation plays a role.
 
Meanwhile several foods and supplements may be helpful in supporting your immune system – read our article How can you strengthen your immune system? for more details.
 

Need to find out more?

 
You can find out more details about a range of skin problems by visiting the skin care section of our pharmacy health library. There’s also lots of information and advice you may find helpful in our section about immunity. 

References:

1. Available online: https://www.nhs.uk/conditions/bullous-pemphigoid/

2. Available online: https://patient.info/skin-conditions/bullous-pemphigoid-leaflet

3. Available online: https://patient.info/doctor/bullous-pemphigoid-pro

4. Available online: https://www.immunology.org/public-information/what-immunology

5. Available online: https://dermnetnz.org/topics/bullous-pemphigoid

6. Available online: https://www.bad.org.uk/pils/pemphigoid/

7. Available online: https://www.bad.org.uk/pils/pemphigoid/

8. Available online: https://www.pemfriendsuk.co.uk/food-and-nutrition

9. Aranow C, Vitamin D and the Immune System. J Investig Med. 2011 Aug;59(6):881-886. Available online: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3166406/

10. Thomsen BJ, Chow E, Sapijaszko MJ, The Potential Uses of Omega-3 Fatty Acids in Dermatology: A Review. J Cutan Med Surg. 2020 sept/Oct;24(5):481-494. Available online: https://journals.sagepub.com/doi/10.1177/1203475420929925

11. Liu X et al., Serum vitamin E levels and chronic inflammatory skin diseases: A systematic review and meta-analysis. PLoS One 2021; 16(2): e0261259. Available online: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8670689/

12. Berardesca E, Camell N, Vitamin E supplementation in inflammatory skin diseases. Dermatol Ther 2021 Nov;34(6):e15160. Available online: https://onlinelibrary.wiley.com/doi/10.1111/dth.15160
    
    Berardesca E, Camell N, Vitamin E supplementation in inflammatory skin diseases. Dermatol Ther 2021 Nov;34(6):e15160. Available online: https://onlinelibrary.wiley.com/doi/10.1111/dth.15160

 

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Disclaimer: The information presented by Nature's Best is for informational purposes only. It is based on scientific studies (human, animal, or in vitro), clinical experience, or traditional usage as cited in each article. The results reported may not necessarily occur in all individuals. Self-treatment is not recommended for life-threatening conditions that require medical treatment under a doctor's care. For many of the conditions discussed, treatment with prescription or over the counter medication is also available. Consult your doctor, practitioner, and/or pharmacist for any health problem and before using any supplements or before making any changes in prescribed medications.

 

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