Dermatology - Subacute Cutaneous Lupus Erythematosus

This leaflet has been adapted from the patient information leaflet written by the British Association of Dermatologists (www.bad.org.uk/pils/subacute-lupus-erythematosus/).

What are the aims of this leaflet?

This leaflet has been written to help you understand more about subacute cutaneous lupus erythematosus (SCLE). It explains what it is, how it can be treated, and where you can find more information.

What is subacute cutaneous lupus erythematosus?

Subacute cutaneous lupus erythematosus is an auto-immune disease. In this condition, the body’s immune system, which is meant to protect against infection, mistakenly attacks the skin. It is part of a group of auto-immune skin disorders known as ‘cutaneous lupus erythematosus’ that share related clinical and laboratory test findings.

What does subacute cutaneous lupus erythematosus look like?

SCLE typically causes a red, scaly, raised and/or ring-shaped rash and usually affects areas of the skin exposed to sunlight, such as the ‘V’ of the neckline, scalp, arms and upper back. The face is usually unaffected.

Are there any other symptoms of subacute cutaneous lupus erythematosus?

Most people feel well; however, some people may notice symptoms such as fatigue or joint aches. In a small proportion of people with SCLE, there is evidence of lupus in other body organs (such as the kidneys and lungs) leading to a diagnosis of systemic lupus erythematosus (SLE). Your dermatologist will assess whether you have evidence of SLE by asking you detailed medical questions, examining your skin and taking blood and urine samples. This will allow them to determine whether you should see other specialists such as a rheumatologist to help diagnose and treat your condition.

What causes subacute cutaneous lupus erythematosus?

SCLE is an autoimmune condition. This means that the body’s own immune system attacks the skin causing inflammation. Environmental factors are also important. For example, sunlight (or other sources of ultraviolet light) typically causes a flare up of the rash and SCLE tends to be more active in people who smoke. SCLE is more common in women than men.

The condition can sometimes be caused by medications, including some blood pressure and antacid medications such as proton pump inhibitors (PPIs). It is important that you should not stop any medication without first discussing it with your doctor.

Is subacute cutaneous lupus erythematosus hereditary?

Sometimes lupus erythematosus and related conditions run in families, but this is rare. If you are affected by SCLE and become pregnant, certain antibodies called anti-Ro/SSA and/or anti-La/SSB from your blood can cross the placenta and, very rarely, affect your baby causing a rash and/or a slow heartbeat. If you are considering pregnancy, please discuss this with the doctor.

How will subacute cutaneous lupus erythematosus be diagnosed?

Your doctor may be able to make a diagnosis of SCLE based on your history and the appearance of your rash. In some cases, it is necessary to take a small sample of skin (a biopsy) to be examined under a microscope to confirm the diagnosis. The doctor will probably also take a sample of blood to test for certain specific antibodies (known as “extractable nuclear antigens (ENA)” or “anti-Ro/SSA and/or anti-La/SSB”) which appear to be important in the condition.

Can subacute cutaneous lupus erythematosus be cured?

There is no cure for SCLE. If a particular medication is suspected to have caused the condition, then once that medication is stopped the rash may settle. It is important to note that this may take a long time, more than 6 months in some cases. In the majority of cases of SLCE however, there is no cure but there are many treatments that can be used successfully to control it.

How can subacute cutaneous lupus erythematosus be treated?

Avoiding ultraviolet light is essential as this can trigger flare-ups of skin disease. 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 (high UVB protection) and also has high UVA protection. Remember to re-apply the sunscreen evenly and often
• Learn how much sunlight your skin can tolerate and keep within that limit

Local treatment:

Strong or very strong corticosteroid creams and ointments are commonly offered as a first line treatment and can be used safely, even on the face, under the direction of your dermatologist. Other topical treatments (treatments applied to the skin), which may be offered in addition or as alternatives to topical corticosteroids include topical calcineurin inhibitors such as tacrolimus.

Systemic treatment:

If the skin rash is more severe, widespread, or if topical treatment is ineffective, then oral medications may be used. The most commonly used treatments are the anti-malarial drugs hydroxychloroquine and mepacrine, which are sometimes used together. As these medications may take some time to work, some people may be prescribed oral corticosteroids for a short period of time. Occasionally, some people may need other medications as additional or alternative treatments, and these include methotrexate or mycophenolate mofetil.

Self-care (What can I do?)

Sun protection

The most important thing you can do is to protect your skin from sunlight. This does not just mean avoiding sunbathing, but also exposure to the sun when out in the garden, walking, shopping, playing sports, or even driving a car. It is important to wear a broad spectrum sunscreen on all skin that is exposed to light, even on cloudy days.

Remember, the sun is most intense at mid-day. It can be quite powerful even on a hazy or cloudy day. Lying under a sunshade does not protect the skin totally and ultraviolet rays from the sun can be reflected from water and snow.
 
No sunscreen can offer you 100% protection. They should be used to provide additional protection from the sun, not as an alternative to clothing and shade.

Pregnancy

If you become pregnant, your baby may be born with a rash or a slow heartbeat as the ENA antibodies are able to cross the placenta. For this reason, it is important to let your obstetrician or midwife know that you have subacute lupus erythematosus. If you are considering pregnancy, please discuss this with the doctor.

Smoking

Evidence suggests that people who smoke cigarettes may have a more severe condition. If you smoke, speak to your dermatologist or general practitioner if you wish to be directed to the relevant services to help you stop smoking.

Vitamin D advice

In order for people to make enough Vitamin D themselves, they need a certain amount of sun exposure. People who are avoiding (or need to avoid) sun exposure may be at risk of vitamin D deficiency and should consider having their serum vitamin D levels checked. If the levels are low, they may need to consider:

• Taking vitamin D supplements of 10-25 micrograms per day
• Increasing intake of food rich in vitamin D such as oily fish, eggs, meat, fortified margarine and cereals

Where can I get more information?

Contact us

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

Links to patient support groups:

The patient self-help group Lupus UK supports people with all forms of lupus; it has local branches around the country and arranges regular meetings, as well as supporting research into the condition. For further information, contact:

LUPUS UK
St James House Essex, RM1 3NH
Tel: 01708 731251
Fax: 01708 731252
Email: headoffice@lupusuk.org.uk
Web: www.lupusuk.org.uk