The cutaneous (skin) porphyrias
The cutaneous (skin) porphyrias can all give sensitivity to sunlight, on exposed areas of skin. As with all porphyrias, the severity of the problem varies enormously.
Sunlight should be avoided as much as possible. When avoiding sunlight is not possible, special sunblocks which block visible light can help. (In mild cases, high-factor sun cream with high-UVA protection may be enough.) Hats and clothing which cover up the skin are also recommended.
As EPP is characterised by sensitivity mainly to visible light, conventional sunscreens that are formulated to protect against just ultraviolet (particularly UVB) are usually not effective. Reflectant sunscreens that are based on titanium dioxide or zinc oxide will be more effective as they cover both UVA, UVB, and visible light to a degree. In the UK, the SPF (sun protection factor) number tells you how effective the sunscreen is for UVB, and the star rating (usually found on the back of the bottle, with a maximum 4 stars) gives a measure of the UVA protection.
An example of a reflecant sun screen is the Dundee Reflectant Sunsreen and if you haven’t already used the Dundee Cream it’s worth a try. Ask your GP to prescribe it for you. Your GP/chemist will need to contact Dundee Pharmaceuticals, Ninewells Hospital, Dundee. DD1 9SY, telephone: 01382 632052. Dundee Cream comes in three colours: beige, white and coffee.
For further useful information check out British Association of Dermatologists website www.bad.org.uk and type in porphyria on the top left search bar.
Wearing clothing such as long-sleeved close-knit fabric tops and long trousers during the summer months is a must. Gloves are useful especially when driving. Wide-brimmed hats can help, also using an umbrella whilst shadow hopping is an idea.
Useful UV reflective umbrellas can be purchased from www.umbrellaheaven.com.
The light that protoporphyrin absorbs is different from that which causes ordinary sunburn. Usually sunburn is caused by the shorter wavelengths of ultraviolet light (UVB), but in EPP the skin is more sensitive to longer ultraviolet wavelengths (UVA) and to visible light, EPP pain is due to violet light at 400-420nm.
Some EPP sufferers have found that using film on car (and home) windows is useful to give them a bit more time exposed to sunlight before pain occurs. With regards to car windows the rules on cars from DVLA are:
“75% of visible light has to be able to come through front and back windows and 70% through the front side windows”. Therefore for cars there is a compromise between letting visible light through and stopping the violet light which causes the pain in EPP and still having a legal car. There are no restrictions on rear passenger windows or the rear windscreen
Our medical colleagues recommend the following filter films for cars or home: ARC UVCL (ARC Window Films) or Dermagard (Bonwyke) give about 50% protection to EPP patients and are legal on cars and completely clear and colourless. They block out everything up to 400nm, nothing past 420nm and since EPP pain is due to 400-420nm they therefore give 50% protection from the pain-inducing waveband
However, ARC UVTA (ARC Window Films) gives almost 100% protection for EPP Patients. It is Amber in colour and is suitable for almost any window. The only windows it cannot be installed to, are the driver and passenger windows of a car, as only clear films may be installed to these windows under DVLA Regulations.
If a film were developed which blocked out everything up to 430nm that would be more effective, not too yellow, and still OK for car windows but we are not aware of such a film, if anyone does have any information on this please do contact the BPA (email@example.com)
The two companies mentioned above can be contacted as below:
- Bonwyke Ltd, Unit 3 Salterns Lane Industrial Estate, Salterns Lane, Fareham, Hampshire, PO16 0SU Tel: 01329 289621 Fax: 01329 822768 Email: firstname.lastname@example.org www.bonwyke.co.uk
Other treatments for EPP in particular, include beta-carotene and/or UV light treatment, for more information on these please speak with your porphyria specialist as they will know if they would be suitable for your individual requirements.
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Skin problems and acute porphyrias
People with acute porphyria are as likely as anyone to also have other skin problems as well.
The comments below are from one of our advisors, who is a medical expert in both skin problems and porphyria. They are relevant for people with acute porphyrias.
ACNE: Topical treatments for mild to moderate acne are generally safe in patients with porphyria, though there is scant evidence in the literature. Unfortunately, none of the commonly-used antibiotics are safe. Similarly, Isotretinion, the most effective treatment for acne, is unsafe in the acute porphyrias and should be avoided.
ECZEMA: Eczema is often controlled with a combination of moisturisers and steroid creams or ointments. These treatments are generally safe. Moisturisers keep the skin in good condition, which may help to avoid minor injuries of the skin which may lead to scarring. Anti-histamine tablets are sometimes given to help reduce itching. Chlorpheniramine (Piriton) and Promethazine (Phenergan) are considered safe. Most other anti-histamines should be avoided. Considering systemic treatments for eczema, Prednisolone and Azathioprine are both safe in all forms of porphyria. Ciclosporin, another agent occasionally used to treat eczema, is not safe. For ultraviolet treatment of eczema, see below under psoriasis.
PSORIASIS: Treatment with moisturisers and steriod creams/ointments is safe. Topical treatments such as Calcipotriol, Dithranol and coal tar are not known to be harmful. Systemic treatments for psoriasis include Methotrexate which must be used with caution. Acitretin and Ciclosporin are both unsafe. Ultraviolet light treatments are often used together with psoralens tablets for both psoriasis and eczema, however it should be avoided by patients with photosensitive porphyrias. Psoralens tablets which are known to be unsafe should be avoided by those who have any of the acute porphyrias.
SKIN SURGERY: Sometimes the skin needs to be sampled either to help make the diagnosis or to remove benign or malignant tumours. This is usually done under local anaesthetic. Most local anaesthetic agents are safe in porphyria, but Bupivacaine (Marcaine) is preferred. WARTS: Preparations such as salicylic acid (cream or paint) are safe but care should be taken since the skin in porphyria is fragile. This is also the case for cryosurgery.
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