Subacute cutaneous lupus erythematosus

What is subacute cutaneous lupus erythematosus (SCLE)?

Cutaneous lupus erythematosus (LE) is a diverse group of autoimmune connective tissue disorders localised to the skin that can be associated with systemic lupus erythematosus (involving other organs within the body) to varying degree.

Subacute cutaneous lupus erythematosus (SCLE) is type of cutaneous lupus erythematosus characterised by scaly, raised and/or ring-shaped skin changes usually affecting areas of sun-exposed skin such as the ‘V’ of the neckline, scalp, arms, and upper back; the face is often unaffected. 

What does SCLE look and feel like?

• Most people feel well; however, some people may notice symptoms such as fatigue or joint aches. Approximately 10% of cases go on to develop systemic lupus erythematosus (SLE) with evidence of lupus in other body organs (such as the kidneys and lungs)

• The main affected areas are sun-exposed sites such as the neck, trunk and outer arms; the face is often spared

• SCLE typically causes red, scaly, raised and/or ring-shaped skin changes. In skin of colour the affected area may be hyperpigmented (darker than the normal skin), or hypopigmented (lighter than the normal skin). Unlike in discoid lupus erythematosus, there is no scarring 

• Other features can include hair loss (non-scarring), a network-like appearance of the skin (livedo-reticularis), Raynaud’s phenomenon (pain and colour changes in the fingers and/or toes in the cold), and mouth ulcers 

What causes SCLE?

SCLE is an autoimmune disease, in which the body’s natural defence system can’t tell the difference between your own cells and foreign cells, causing the body to mistakenly attack normal cells.

It is thought that a combination of genetic and environmental factors most likely contribute to the development of SCLE.

Patients can be of any age, sex, or ethnicity. However, SCLE is most common in middle-aged women, except in cases caused by medications, when it often presents in older people.

Genetically, SCLE is associated with the human leukocyte antigen (HLA); there are several other genetic associations. Sometimes lupus erythematosus and related conditions run in families, but this is rare. 

Ultraviolet radiation (UVR), from sunlight and sunbeds, is an important trigger for SCLE; an association with smoking has been proposed but is not certain.

Approximately one-third of cases are caused by medications; including some blood pressure and antacid medications such as proton pump inhibitors (PPIs). Drug-induced lupus is more common in older people, and may cause more widespread skin changes. It is important that you should not stop any medication without first discussing it with your doctor. Most cases of drug-induced SCLE are reversible on stopping the offending drug, although resolution can take up to 12 months (most improve within 3 months).

Things you can do to help yourself

The two most importing things are related to smoking and UV-protection.

Smoking

If you smoke, we strongly recommend that you stop. In some people smoking can make SCLE worse and may result in a poorer response to treatment.

UV-protection

The most important UV protective measures are:

• Avoid sitting or lying directly in the sun, and remember you also get UV exposure when walking, playing sports, gardening, and even driving 
• Protect your skin with clothing. Ensure that you wear a hat that protects your face, neck and ears, and a pair of UV protective sunglasses
• When outside, even on a cloudy day or when under a sunshade, use a ‘high protection’ sunscreen of at least SPF 30 which also has high UVA protection (4 or 5 star). Apply sunscreen generously 15 to 30 minutes before going out in the sun, or go swimming, and make sure you reapply frequently when in the sun. 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. The sunscreen should be prescribed by your GP as it is being used for medical reasons
• Avoid sunbeds
• For more detailed information on UV-protection and advice on increasing your vitamin D intake (your vitamin D levels will drop if you reduce your UV exposure significantly) please refer to the patient information leaflet on Skin Cancer Prevention

Referral to a specialist

In the majority of cases you will need to be referred to a specialist, who could be a dermatologist or a GPwER/GPwSI (a GP who has been trained in relevant areas of dermatology).

What will happen when you are referred?

In most cases it is necessary to take a small sample of skin (a biopsy) to be examined under a microscope in order to confirm the diagnosis. Other tests may be performed including blood and urine tests.

Treatment

• Skin (cutaneous) treatments - these may include steroid creams, ointments, gels, or lotions. Other topical treatments which may be offered in addition or as an alternative to topical steroids, are the topical calcineurin inhibitors, pimecrolimus and tacrolimus. In some patients with localised skin involvement, injections of steroids into the affected skin may be effective

• Oral medications (tablets) - if your skin/scalp changes are more severe and/or do not respond to cutaneous treatments, then systemics medications may be required. The most commonly used medications are the anti-malarial drugs hydroxychloroquine and mepacrine. Occasionally, some patients may need additional medications, known as immunosuppressive therapy (i.e. drugs that supress your immune system)

Your mental health

SCLE can have a significant psychological impact and may affect many areas of daily life including work and personal relationships. If you require support with your mental health, then discuss this with a healthcare professional.

SCLE and pregnancy

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.

Can SCLE be cured?

If a particular medication has caused the condition, it make take many months after the drug is stopped before the skin improves. In the majority of other cases, there is no cure but there are many treatments that can help. 

Other resources

• The patient support group, Lupus UK - www.lupusuk.org.uk

Helping with other skin conditions

If you, a family member, or friend have an undiagnosed skin condition; or you want to learn more about how to treat skin conditions, please view our short video on how to get the best from this website.