Dermatology - Hydroa Vacciniforme

This guide has been written to help answer some of your questions about hydroa vacciniforme. You may have been given this guide because you have been diagnosed with hydroa vacciniforme or because we think this might be the cause of your symptoms.

What is hydroa vacciniforme?

Hydroa vacciniforme is a rare skin condition caused by sunlight. The hydroa part of the name describes the fluid filled blisters and the vacciniforme part describes the chicken pox-like scars.  Patients can be affected throughout the year, but it is usually worse in spring and summer months.

Who develops hydroa vacciniforme?

Hydroa vacciniforme mostly affects children, though can occasionally persist and affect adults too. It can affect both males and females.

What are the symptoms of hydroa vacciniforme?

After a short time in the sun, a tingling discomfort develops followed by raised bumps and blisters of varying size on a red swollen background. The blisters are filled with a clear fluid and can then become scabbed and crusted. As these heal they can leave chickenpox-like scars. The rash normally affects sun exposed sites, usually the face, ears and backs of hands. Covered areas of skin can also sometimes be affected. Occasionally people can also develop some soreness of the eyes or feel generally unwell during a flare-up.

Is hydroa vacciniforme serious?

Classical or typical hydroa vacciniforme can restrict your lifestyle, particularly during the summer months and on holidays. Once it occurs patients need to cover up, stay out of the sun and wait for it to settle. Over time, most patients can judge how long they can stay out in the sun without developing their rash.

What causes hydroa vacciniforme?

Hydroa vacciniforme is due to sensitivity to sunlight, usually ultraviolet-A light. It is not known what causes it to occur in some people but not in others. Hydroa vacciniforme can be associated with Epstein Barr virus infection (the virus which can cause glandular fever), and it is thought that this alters the way the skin reacts to sunlight.

Occasionally, this type of infection can cause more serious illness and more severe skin problems. This is more of a problem in certain countries and ethnic groups. However, if this is suspected it will be discussed with you, and you may need further investigations.

Is hydroa vacciniforme infectious?

No. There is no risk of other people catching the condition from you.

Is hydroa vacciniforme hereditary?

It does not seem to be passed on to children, but there have been some rare cases where several family members have been affected.

Can hydroa vacciniforme be cured?

No treatment will completely get rid of hydroa vacciniforme but by careful sun avoidance and use of sunscreens the rash can be kept under control. Most children grow out of hydroa vacciniforme and the rash goes away, although it can persist and occasionally affects adults.

How will having hydroa vacciniforme affect me?

If you have been diagnosed as having hydroa vacciniforme you may have to make changes to your lifestyle which can restrict it to some extent. Steroid creams or ointments can be applied if the rash appears, but prevention is better than cure. You should try to:

• Avoid the sun during conditions that provoke your photosensitivity and particularly when sunlight is intense. In the UK this is mainly between 11 and 3pm, although other times of day can be troublesome
• Wear protective clothing, with close/tightly woven fabrics, long sleeves and trousers, hats with broad brims. Glasses and sunglasses with wrap around lenses can also be helpful
• Use a sunscreen that is SPF 30 or above (UVB protection) and also has high UVA protection. Remember to reapply the sunscreen evenly and often. Learn how much sunlight your skin can tolerate and keep within that limit
• Make sure you introduce your skin to sunlight gradually in the spring

What about vitamin D?

Importantly, for people to make enough Vitamin D themselves, they need a certain amount of sun exposure. The sun protection measures needed for people with hydroa vacciniforme mean Vitamin D deficiency is a real risk and this does require treatment with supplements if it occurs. This can be monitored through blood tests.

How can hydroa vacciniforme be treated?

Sunscreens

You can be provided with sunscreens on prescription, and these will help prevent the rash developing. Sunscreen should be applied as thickly and evenly as possible and re-applied often.

Topical creams

Your doctor may prescribe steroid creams or ointments to put on your skin if the rash appears. Steroid creams can be strong and should only be applied as directed and while the rash is there.  You should stop applying steroid cream when the rash has gone. More information about steroid creams and how they should be used can be accessed through the British Association of Dermatologists website at https://www.bad.org.uk/pils/topical- corticosteroids/

Desensitisation or UV Treatment

Sometimes the resistance of your skin to the effects of the sun can be increased by ultraviolet treatment. This is done in a hospital department and will involve visiting the hospital three times a week for about 5 weeks in the spring. It involves gradually exposing your skin to a little more ultraviolet light every visit to try to build up your skin’s resistance. The effects of this treatment are lost in the winter so you will have to build up your resistance again in the next spring.

Hardening or toughening

Some people with hydroa vacciniforme may be able to increase the resistance of their skin at home. This involves going outside for short periods in the spring to build up your resistance. You might find the time is as short as a few minutes at first, but you may be able to gradually build this up to longer times. You will have to be careful not to overdo it, but as you have learnt how much light triggers your rash you will be able to judge how long to stay out. The effects of hardening are lost in the winter, so you will have to build up your resistance again in the next spring. We have included an example of how you might try to toughen your skin in the spring.

Example

Jane knows her rash usually appears in the evening if she has been out in the sun during the day. Jane has been toughening her skin in the spring and this is her diary.

Monday - I went outside in the sun for 5 minutes. I went back indoors and waited until the evening to see if my rash appeared - thankfully it didn't!

Wednesday - Today I went out in the sub for 10 minutes. I covered myself up after that, so I didn't catch any extra sun and waited until the evening. My rash still didn't appear.

Friday - I decided to go outside for 20 minutes and then I went back inside. Later I noticed my rash started and I felt itchy. I put some steroid cream on very thinly and stayed out of the sun.

Sunday - The rash has started to go down, but it still hasn't fully gone. I will wait until next Wednesday before I go back out in the sun.

Wednesday - I went outside in the sun for 10 minutes. The rash did not come back.

Friday - Went out in the sun for 20 minutes ... no rash!

Tablets

Occasionally tablet treatments have been used in hydroa vacciniforme. Some of these aim to reduce the photosensitivity, and some are tablets which can affect the immune system. There is the potential for side effects with some of these tablets, so they are not suitable for everyone.

Contact us

The Photobiology Unit can be contacted on 0161 206 4081 or by email: photobiology.salford@nca.nhs.uk