A tale of bullous pemphigoid

My name is Ingrid and in April of 2015 I was diagnosed with a rare autoimmune blistering skin disease called bullous pemphigoid. Why 467 you may ask? That is the number of blisters on my body when I was hospitalised on April 8th, 2015.  

Blisters or "bullae" appeared

Most of these “bullae” appeared whilst I was on holiday in Madeira. If I had known what was going to happen, I wouldn’t have gone on holiday, but the blisters emerged so rapidly. I’d had a bad rash for some time but was misdiagnosed with possible scabies. This happens quite frequently with my disease.

Ingrid's initial rash

Dr Adil Sheraz, Consultant Dermatologist & British Skin Foundation spokesperson explains:

Bullous pemphigoid is the most common of the various autoimmune blistering diseases.  There are between 2.4 and 21.7 new cases per million population in different populations worldwide. The condition tends to affect those between 60-80 years of age. In the initial stages patients may see red, itchy patches that can look like hives.  Blisters (also known as bullae) will then often form overlying the red patches. The blisters tend to be relatively tense and, in some cases, can grow to the size of a small apple! It is not unusual to see patients with dozens of blisters with multiple new ones appearing daily.

The causes of the condition are unfortunately not fully understood however we do know that certain auto-antibodies attack the skin resulting in the rash and blister formation. Occasionally bullous pemphigoid can be caused by medications.

The treatments of choice initially (depending on the severity) are either topical or oral steroids, prednisolone in particular tends to work very well.  The disease varies in its course and some patients may just have a single episode while for others it may be a chronic problem lasting for years requiring longer term immunosuppressant medication.

Many GPs know little or nothing about the disease, let alone having seen someone with it. Luckily for me one doctor at my practice had suggested tentatively that I may have bullous pemphigoid (despite the fact I am young for it as it tends to be a disease for the over 70’s and I was 55).

Lots of random internet searching led me to two ladies – who kept me going that lonely week in Madeira – from PEM Friends, a closed Facebook group. As there is pemphigus and pemphigoid, the term PEM is used to cover both.

Trying to get dressings for the open wounds in Madeira was impossible. I didn’t really want to show anyone in a pharmacy the open sores. I was already fairly sure that it was some sort of PEM I had “acquired” but try explaining that in Portuguese! It’s hard enough to explain in English! The other difficult thing to get across in any language is that you’re not infectious. In fact, being seen by people who think you are infectious can become quite stressful.

The stress of the blisters

The flight home was horrendous (people staring etc.) and I waited three days for the doctor as it was Easter Saturday when we flew back. I went to my GP on the Tuesday and he was quite shocked, sending me to an emergency walk in for dressings. I still remember the look of shock on the face of the clinician who was to dress my blisters.

Ingrid's skin after her holiday

The blisters were very severe. On Wednesday 8th April, after an emergency dermatology appointment, I went into hospital with a staggering 467 blisters. I was immediately put on a wheelbarrow full of drugs, including steroids which helped the blisters to disappear quickly.

Ingrid's skin improved after one month of treatment

One year on with my medicines reduced, my hands were still scarred, but blister and rash free. The medicines themselves can be challenging to deal with, including steroid tapering which all needs to be done very gradually under the supervision of your dermatologist.

Some medicines are rare and need to be ordered specially by the pharmacy.

The scarring remains on Ingrid's skin

Finding support with PEM Friends

It’s been a bit of a roller coaster ride since I developed bullous pemphigoid. Five and a half years on and I still live with the consequences of this condition. The disease has had a significant impact on my lifestyle, including early retirement. It’s very hard to explain to some people (especially when you don’t have blisters) that it’s a bit more than ‘just’ a skin complaint.

During the Covid-19 pandemic I have been shielding due to immunosuppressant drugs and the side effects of my medication have led to high blood pressure and diabetes.  Although bullous pemphigoid can go into remission, the current pandemic stress seems to have caused a lot of people to flare.

Despite all the challenges I have discussed, I found that connecting with others who have one of the PEM conditions has helped me a great deal. Just knowing that you aren’t alone out there can be a great comfort. This is why we (myself and some others with PEM conditions) have recently launched a new website called www.pemfriendsuk.co.uk to support and inform patients and carers of people with autoimmune blistering skin diseases. There are a few of us out there who know what you are going through, and we can help.

Ingrid Thompson

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