Return to Homepage

So it turns out I've got a thing - it's called EPP and it's a proper 'thing.' All my life I've had to stay out of the sun, as regulars will know if you recall my blog from early July when I had to report that my 'skin had flared up' during the Rochester Castle fundraising bodyblade wobble in Kent. What happens is that if my skin is over-exposed it feels like it's on fire, even though nothing shows on the outside. Then it starts to itch as well, and then it swells up and the skin gets super tight. It takes around two days to go down.


When I was on a skiing hol once using a balaclava, the skin in between my eyes swelled up and my eyes were like slits. When I stupidly used a sunbed aged around 22 I had the most horrendous reaction my rings needed to be cut off my fingers. And apart from one occasion, I've never been able to do normal things in the sun. When I was a kid I had to shelter away from the sun, I felt like my needs were never made a priority as I just had to make the best of it and bought comics and books and sweets and sat away from everyone else and rarely went in the sea. (the pic above is when I was about 9, that's me behind the deck chairs in the shade. Poor Debbie.) My family know this, and just take it for granted, and so did I, until this year.

I'd been genning up on how gut bacteria affects everything and remembered that the only time I felt like I could go in the sun was, yes, with my regular triple whammy of high once a day spf, plus L-tyrosine tablets (they work on the melanin from the inside out) and tan accelerator (it works on the melanin from the outside in) BUT I'd also been vegan for three months and was eating clean for another seven before I went to Cyprus for my brother's wedding. It was the first and so far only time I've been able to be in the pool with my kids on a summer holiday. So maybe there's a connection with nutrition. So I went to see a specialist.

The dermatologist called Dr Flohr, at Guildford Nuffield, asked me a very pertinent question. Why was this the first time I'd ever come to an expert? I was stumped by that. Have you ever had it where you just accept that's the way life is and have stopped questioning it? I guess that was me. It had been part of me, my reality, for so long, it didn't really occur to me to challenge it, it was just sensitive photosensitive skin, right? Till the gut thing. So here I was. And he looked me in the eye and said 'I think you've got this.'

It was EPP.

Erythropoietic protoporphyria (EPP) is one of a group of genetic diseases called the porphyrias. EPP is due to an inherited deficiency of the enzyme ferrochelatase. Reduced activity of this enzyme causes a build-up of the chemical protoporphyrin in the skin; resulting in photosensitivity i.e. the skin is damaged by light. There is no known cure, but you can reduce the photosensitivity with certain supplementation of enzymes etc. More here.

He swung the monitor round and showed me the page and I read words that resonated so strongly they of course could be writing about me. I was shocked. “First symptoms usually appear in infancy or early childhood and present as an uncomfortable or painful burning sensation of the skin after sun exposure. It occurs most often on the tops of the hands and feet, face and ears. In most cases visible changes to the skin are mild. The affected skin may become red and swollen and blistered.”

But at least I don’t now have the worst version of the condition – “Later there are pitted scars and sometimes crusty thickened skin, particularly over the cheeks, nose and knuckles of the hands.” Although I used to get a nose scab every summer when I was young. Plus my knuckles are not attractive – nor are my hands – I always said I have man’s hands, my dad’s hands, and now I know why. Hence you’ll often see me on jewellery shows using whole hand shots rather than close ups of my hands with the jewellery on. Don’t suppose you’ve ever noticed but that’s why. When the condition flares, after the swelling starts going down again and the burning dissipates, I’m left with a rough, sensation-deprived surface on my knuckles in particular. God, now I know why.

If this is indeed what I have, and it seems as though it is, then I’m rare.

The treatment possibilities include –

Lifelong photosensitivity is the major problem for EPP uncomplicated by liver disease, which about 10% may go on to get due to the protoporphyrin build up.

  • The obvious stuff I already do, eg avoid unnecessary exposure to sunlight and wear protective clothing and wide-brimmed hats. Sunscreens, especially formulations containing zinc oxide or titanium dioxide that reflect visible light. (I told the doctor about tan accelerators which he hadn’t heard about, I don’t think, and when I said Ultrasun he nodded his head.)
  • Consider tinting windows.
  • Other strong sources of light may also cause symptoms, including fluorescent and halogen lights. (I’ve had this before and thought I must be imagining things.)

But I’ll be looking into taking some of these for sure – maybe my vegan time included lots of carrots!:-

  • Oral beta-carotene (a food precursor of vitamin A found naturally in tomatoes and carrots) is thought to help reduce photosensitivity in some people. The dose for children is 30 to 150mg per day (1 to 5, 30-mg capsules) and for adults it is 30 to 300mg (1 to 10, 30-mg capsules).
  • Cysteine 500mg twice daily reduces photosensitivity
  • Colestyramine reduces photosensitivity and hepatic protoporphyrin content.
  • Narrowband UVB phototherapy increases melanin content and induces skin thickening so may reduce sun sensitivity.
  • Afamelanotide, an α-melanocyte stimulating hormone given by subcutaneous implantation, has been reported to provide good clinical effectiveness and safety in EPP. It is approved under exceptional circumstances by the European Medicines Agency for treatment of EPP (October 2014).

So there you go. And there I go. So now I am being referred to the country’s foremost expert , a colleague of Dr Flohr, who apparently did his doctorate in THIS SUBJECT!

I feel a bit weird since it’s like suddenly I’m not so strange, there are others like me out there. I spoke with a mate who has IIH and who felt exactly as I felt when she was first diagnosed. It’s not something to be proud of  cos everyone wishes they were ‘normal.’ BUT it feels relief to know there’s a course of action. I will keep you informed.

Best wishes



 ps coming soon - the latest update from my Till the Fat Lady Slims group - some super before and afters, stories and NSVs (non scales victories!)




Published in Back to You